{
 "meta": {
  "title": "Epilepsy Board Review",
  "note": "Original teaching notes written for NeuroLogic from primary guidelines and position papers. Not a summary of any single textbook.",
  "parts": 9,
  "sections": 47,
  "questions": 127,
  "cards": 337,
  "bank": 440
 },
 "parts": [
  {
   "id": "basics",
   "n": "1",
   "title": "Mechanisms and the EEG signal",
   "short": "EEG basics",
   "sections": [
    {
     "id": "seizure-mechanisms",
     "n": "1",
     "title": "How seizures start and stop",
     "tags": [
      "eeg-fundamentals",
      "seizure-management"
     ],
     "updated": false,
     "goals": [
      "Relate excitation and inhibition to the drugs that act on them",
      "explain why absence seizures behave differently from focal seizures",
      "describe why status epilepticus becomes harder to stop with time"
     ],
     "blocks": [
      {
       "type": "p",
       "text": "A seizure is a transient burst of abnormally excessive or synchronous neuronal activity that produces signs or symptoms. Two things have to go wrong at once: neurons become too easy to fire, and the network lets that firing recruit its neighbors in lockstep. Almost every antiseizure medication (ASM) acts on one side of that balance."
      },
      {
       "type": "h3",
       "text": "Excitation and inhibition"
      },
      {
       "type": "li",
       "text": "**Glutamate** drives excitation. AMPA receptors give fast depolarization; NMDA receptors, held shut by magnesium until the membrane depolarizes, let calcium in and sustain firing; kainate receptors contribute in the hippocampus."
      },
      {
       "type": "li",
       "text": "**GABA** drives inhibition. GABA-A receptors are chloride channels: benzodiazepines increase how often they open, barbiturates how long they stay open. GABA-B receptors are G-protein coupled and act slowly through potassium channels."
      },
      {
       "type": "li",
       "text": "**In the newborn** high intracellular chloride (the importer NKCC1 outweighs the exporter KCC2) can make GABA-A depolarizing. This is one reason neonatal seizures respond less reliably to GABAergic drugs; adding bumetanide to block NKCC1 failed in the NEMO trial."
      },
      {
       "type": "li",
       "text": "**Intrinsic excitability** is set by ion channels: sodium channels start action potentials, potassium channels (the KCNQ2/3 M-current, KCNT1) end them and set the resting state, and T-type calcium channels in the thalamus let relay neurons fire in bursts."
      },
      {
       "type": "h3",
       "text": "From one cell to a seizure"
      },
      {
       "type": "p",
       "text": "The **paroxysmal depolarizing shift** is the intracellular signature of an interictal spike: a large, prolonged depolarization crowned by a burst of action potentials and followed by hyperpolarization. The hyperpolarization and the ring of inhibition around the focus (surround inhibition) normally contain it. A seizure starts when that restraint fails and recurrent excitatory connections recruit neighboring cortex. Extracellular potassium rises and calcium falls as it goes, which makes spread easier still."
      },
      {
       "type": "p",
       "text": "Seizures end actively, not by exhaustion. Inhibitory interneurons, adenosine release, acidosis and potassium currents all push the network back down. The same processes explain postictal slowing and Todd paralysis."
      },
      {
       "type": "h3",
       "text": "Two circuits worth knowing"
      },
      {
       "type": "li",
       "text": "**Absence seizures** come from a thalamocortical loop between the cortex, thalamic relay neurons and the GABAergic reticular nucleus. When relay neurons are hyperpolarized, T-type calcium channels let them fire in bursts, and the loop locks into roughly 3-Hz spike-and-wave. Ethosuximide blocks T-type channels. Drugs that deepen that hyperpolarization (vigabatrin, tiagabine) or that act mainly on sodium channels (carbamazepine, oxcarbazepine, phenytoin) can make absences worse."
      },
      {
       "type": "li",
       "text": "**Mesial temporal lobe epilepsy** with hippocampal sclerosis shows neuronal loss (most marked in CA1 and CA4), loss of hilar interneurons and **mossy fiber sprouting**: dentate granule cell axons turn back and synapse on other granule cells, building a recurrent excitatory circuit that was not there before."
      },
      {
       "type": "h3",
       "text": "Epileptogenesis"
      },
      {
       "type": "p",
       "text": "After an injury such as trauma, stroke, infection or prolonged status there is often a latent period before epilepsy declares itself. Cell loss, axonal sprouting, inflammation (interleukin-1β, HMGB1) and blood–brain barrier leak (albumin activating TGF-β signaling in astrocytes) all contribute. No ASM has been shown to prevent this process in people. Phenytoin after head trauma cut seizures in the first week but did not reduce later epilepsy. Kindling, where repeated subthreshold stimulation eventually produces spontaneous seizures, is the classic laboratory model."
      },
      {
       "type": "h3",
       "text": "Why status becomes self-sustaining"
      },
      {
       "type": "p",
       "text": "Within minutes of continuous seizure activity, synaptic GABA-A receptors are pulled into the cell while NMDA and AMPA receptors move to the membrane. Inhibition falls and excitation rises as time passes. That is the biological case for giving an adequate benzodiazepine dose early, and for adding an NMDA antagonist such as ketamine in refractory status."
      },
      {
       "type": "table",
       "head": [
        "Target",
        "What it does",
        "Examples"
       ],
       "rows": [
        [
         "Sodium channels, fast inactivation",
         "Stops sustained repetitive firing",
         "Phenytoin, carbamazepine, oxcarbazepine, eslicarbazepine, lamotrigine"
        ],
        [
         "Sodium channels, slow inactivation",
         "Dampens prolonged depolarization",
         "Lacosamide"
        ],
        [
         "T-type calcium channels",
         "Breaks thalamic burst firing",
         "Ethosuximide (also valproate, zonisamide)"
        ],
        [
         "α2δ subunit of calcium channels",
         "Reduces transmitter release",
         "Gabapentin, pregabalin"
        ],
        [
         "Synaptic vesicle protein SV2A",
         "Modulates vesicle release",
         "Levetiracetam, brivaracetam"
        ],
        [
         "GABA-A receptor",
         "Enhances inhibition",
         "Benzodiazepines, phenobarbital, ganaxolone, stiripentol, cenobamate (with a sodium-current effect)"
        ],
        [
         "GABA metabolism and reuptake",
         "Raises synaptic GABA",
         "Vigabatrin (GABA transaminase), tiagabine (GAT-1)"
        ],
        [
         "AMPA receptor",
         "Blocks fast excitation",
         "Perampanel"
        ],
        [
         "Several mechanisms",
         "Broad spectrum",
         "Valproate, topiramate, zonisamide, felbamate, cannabidiol, fenfluramine"
        ]
       ]
      },
      {
       "type": "note",
       "label": "Board pearl",
       "text": "If a question asks which drug made absences or myoclonus worse, look first for carbamazepine, oxcarbazepine, phenytoin, gabapentin, pregabalin, vigabatrin or tiagabine."
      },
      {
       "type": "mcq",
       "id": "q-8z9g53",
       "stem": "A 7-year-old with childhood absence epilepsy has daily absences. A new medication is started, and within two weeks the absences are more frequent and she has an episode of absence status. Which medication was most likely started?",
       "options": [
        "Ethosuximide",
        "Valproate",
        "Lamotrigine",
        "Carbamazepine",
        "Levetiracetam"
       ],
       "answer": 3,
       "explain": "Carbamazepine, oxcarbazepine and phenytoin, and GABAergic drugs that deepen thalamic hyperpolarization (vigabatrin, tiagabine), can aggravate absence and myoclonic seizures. Ethosuximide, valproate and lamotrigine all treat absence; levetiracetam is not first-line for absence but does not typically worsen it."
      },
      {
       "type": "mcq",
       "id": "q-1cpk30l",
       "stem": "Why does the chance that lorazepam stops convulsive status fall the longer the seizure has lasted?",
       "options": [
        "Hepatic enzymes are induced within minutes of seizure onset",
        "Synaptic GABA-A receptors are internalized while NMDA and AMPA receptors move to the membrane",
        "Benzodiazepine binding to albumin rises as the patient becomes acidotic",
        "SV2A expression falls during prolonged seizures",
        "Extracellular chloride falls, reversing the GABA-A current"
       ],
       "answer": 1,
       "explain": "Receptor trafficking during ongoing seizures removes the benzodiazepine target from the synapse and adds excitatory receptors. It is the mechanistic basis of time-dependent pharmacoresistance and of treating early with an adequate dose."
      },
      {
       "type": "mcq",
       "id": "q-1vnbrwi",
       "stem": "Which change in hippocampal sclerosis creates a new recurrent excitatory circuit?",
       "options": [
        "Dispersion of the dentate granule cell layer",
        "Loss of CA2 pyramidal neurons",
        "Mossy fiber sprouting of dentate granule cell axons onto other granule cells",
        "Gliosis of the alveus",
        "Loss of entorhinal input to CA1"
       ],
       "answer": 2,
       "explain": "Granule cell axons (mossy fibers) normally project to CA3. After hilar cell loss they sprout into the inner molecular layer and excite other granule cells. CA2 is relatively spared in the common form of hippocampal sclerosis."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-1mxozyg",
         "front": "Benzodiazepine versus barbiturate at the GABA-A receptor",
         "back": "Benzodiazepines increase how often the chloride channel opens; barbiturates increase how long it stays open (and open it directly at high doses)."
        },
        {
         "id": "c-7uxa2k",
         "front": "T-type calcium channels",
         "back": "Thalamic relay neurons. They generate the burst firing behind 3-Hz spike-and-wave and are blocked by ethosuximide."
        },
        {
         "id": "c-bcrpuj",
         "front": "Why GABA can excite in the newborn",
         "back": "High intracellular chloride (NKCC1 outweighs KCC2) makes the GABA-A current depolarizing."
        },
        {
         "id": "c-ll45tb",
         "front": "Paroxysmal depolarizing shift",
         "back": "The intracellular correlate of an interictal spike: a prolonged depolarization with a burst of action potentials, then hyperpolarization."
        },
        {
         "id": "c-4gbnpf",
         "front": "Kindling",
         "back": "Repeated subthreshold stimulation that progressively lowers seizure threshold until seizures occur spontaneously. A model of epileptogenesis."
        },
        {
         "id": "c-ah9t9e",
         "front": "Drugs that can aggravate absence or myoclonus",
         "back": "Carbamazepine, oxcarbazepine, phenytoin, gabapentin, pregabalin, vigabatrin, tiagabine."
        },
        {
         "id": "c-1qt8wr",
         "front": "Does phenytoin after head injury prevent epilepsy?",
         "back": "No. It reduces seizures in the first week only; no ASM prevents epileptogenesis."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "ILAE practical clinical definition of epilepsy (2014)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/24730690/"
      },
      {
       "title": "GABA-A receptor trafficking in status epilepticus (Naylor 2005)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/16120773/"
      },
      {
       "title": "Phenytoin after head injury (Temkin 1990)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/2115976/"
      },
      {
       "title": "Bumetanide for neonatal seizures, NEMO (2015)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/25765333/"
      }
     ],
     "words": 1070,
     "bank": 11
    },
    {
     "id": "eeg-generators",
     "n": "2",
     "title": "What the scalp can see",
     "tags": [
      "eeg-fundamentals",
      "epilepsy-surgery"
     ],
     "updated": false,
     "goals": [
      "Name the generator of the scalp EEG",
      "predict how dipole orientation changes what EEG and MEG record",
      "explain why normal scalp EEG never excludes a focal seizure"
     ],
     "blocks": [
      {
       "type": "p",
       "text": "The scalp EEG is the summed **postsynaptic potentials** of cortical pyramidal neurons, not their action potentials. Postsynaptic currents last tens to hundreds of milliseconds and overlap in time; action potentials last about a millisecond and cancel out. Pyramidal cells in layers III and V are lined up with their apical dendrites perpendicular to the cortical surface, so their currents add into an open field that reaches the scalp."
      },
      {
       "type": "h3",
       "text": "What it takes to reach the scalp"
      },
      {
       "type": "li",
       "text": "**Synchrony.** Thousands of neurons have to change together."
      },
      {
       "type": "li",
       "text": "**Orientation.** Cells aligned in parallel sum; randomly oriented cells cancel."
      },
      {
       "type": "li",
       "text": "**Area.** Comparisons of simultaneous intracranial and scalp recordings suggest that most scalp-visible spikes involve at least about 10 cm² of synchronously active cortex, and many involve more. Smaller discharges stay invisible from the scalp."
      },
      {
       "type": "li",
       "text": "**Distance and the skull.** The skull attenuates and blurs the field. Over a skull defect the EEG is higher in amplitude and sharper (a breach rhythm), which is easy to over-read."
      },
      {
       "type": "h3",
       "text": "Dipoles"
      },
      {
       "type": "p",
       "text": "A patch of active cortex behaves like a dipole: negative at one end, positive at the other."
      },
      {
       "type": "li",
       "text": "A **radial** dipole, on the crown of a gyrus, points at the scalp. EEG sees a single negative maximum over it."
      },
      {
       "type": "li",
       "text": "A **tangential** dipole, on the wall of a sulcus, lies parallel to the scalp. EEG shows a negative end and a positive end at separate electrodes; the centrotemporal spikes of self-limited epilepsy with centrotemporal spikes typically show this pattern, negative centrotemporally and positive frontally."
      },
      {
       "type": "li",
       "text": "**MEG** records the magnetic field around the current and is most sensitive to tangential sources, and it is not smeared by the skull. EEG and MEG are complementary rather than redundant."
      },
      {
       "type": "h3",
       "text": "Polarity does not tell you excitation from inhibition"
      },
      {
       "type": "p",
       "text": "Excitation near the surface and inhibition deep in the cortex both make the surface negative; the reverse pair makes it positive. Most epileptiform discharges are surface negative, but positive spikes occur with sulcal generators, over skull defects, and in newborns (positive rolandic sharp waves in intraventricular hemorrhage)."
      },
      {
       "type": "h3",
       "text": "Why scalp EEG misses seizures"
      },
      {
       "type": "p",
       "text": "Mesial temporal, insular, orbitofrontal, interhemispheric and deep sulcal cortex are far from the electrodes or oriented badly. Focal seizures with preserved consciousness often have no scalp correlate at all. The first change seen at the scalp may be propagated activity rather than the true onset, which is why intracranial recording exists."
      },
      {
       "type": "note",
       "label": "Board pearl",
       "text": "A normal scalp EEG during a typical focal aware seizure does not make the event nonepileptic."
      },
      {
       "type": "mcq",
       "id": "q-4u8749",
       "stem": "Which structure's activity is most likely to be missed entirely by scalp EEG but detected by MEG?",
       "options": [
        "A spike on the crown of a lateral frontal gyrus",
        "A spike confined to the wall of a sulcus, generating a tangential dipole",
        "Generalized 3-Hz spike-and-wave",
        "A posterior dominant rhythm at 10 Hz",
        "Vertex waves of stage N2 sleep"
       ],
       "answer": 1,
       "explain": "MEG is most sensitive to tangential (sulcal) sources and is not attenuated by the skull. EEG favors radial sources at the gyral crown. A small sulcal source can be invisible on EEG yet clear on MEG."
      },
      {
       "type": "mcq",
       "id": "q-1q85bzj",
       "stem": "During video-EEG a patient has her typical aura of rising epigastric discomfort and déjà vu, fully aware throughout. The scalp EEG shows no change. What is the best interpretation?",
       "options": [
        "The event is not epileptic",
        "The electrodes were placed incorrectly",
        "A focal seizure with preserved consciousness often has no scalp correlate",
        "She should be diagnosed with functional seizures",
        "The montage should be changed to a common average reference to reveal it"
       ],
       "answer": 2,
       "explain": "Small, deep or mesial seizures frequently produce no scalp change. The history, stereotypy and any later evolution matter more than the absence of an ictal scalp pattern for an aura."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-1tbccoa",
         "front": "Generator of the scalp EEG",
         "back": "Summed postsynaptic potentials of pyramidal neurons (layers III and V), not action potentials."
        },
        {
         "id": "c-4mwisu",
         "front": "Radial versus tangential dipole",
         "back": "Radial (gyral crown) points at the scalp and gives one negative maximum; tangential (sulcal wall) gives separate negative and positive ends and is best seen with MEG."
        },
        {
         "id": "c-1qriaq0",
         "front": "How much cortex for a scalp spike",
         "back": "At least about 10 cm² of synchronous cortex for most scalp-visible spikes."
        },
        {
         "id": "c-i6eazs",
         "front": "Breach rhythm",
         "back": "Higher-amplitude, sharper, faster activity over a skull defect. A normal finding after craniotomy, not epileptiform."
        },
        {
         "id": "c-1dikdrj",
         "front": "Does surface negativity mean excitation?",
         "back": "No. Superficial excitation and deep inhibition both produce surface negativity."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "Intracranial correlates of scalp interictal spikes (Tao 2005)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/15857432/"
      },
      {
       "title": "IFCN glossary of EEG terms (2017)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/30214992/"
      }
     ],
     "words": 693,
     "bank": 6
    },
    {
     "id": "eeg-technique",
     "n": "3",
     "title": "Recording technique",
     "tags": [
      "eeg-fundamentals",
      "acns-terminology"
     ],
     "updated": true,
     "goals": [
      "Localize a discharge from a bipolar and a referential montage",
      "choose filter, sensitivity and sampling settings and predict what changing them does",
      "list the technical requirements for a study of suspected electrocerebral inactivity"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "Amplifiers and polarity"
      },
      {
       "type": "p",
       "text": "Every channel is a differential amplifier that displays input 1 minus input 2. By convention, when input 1 is **negative** relative to input 2 the trace goes **up**. The amplifier rejects what is common to both inputs, which is how mains interference is removed. Rejection fails when the two electrode impedances differ, so 60-Hz noise in a single channel usually means one poor electrode, not a bad ground."
      },
      {
       "type": "h3",
       "text": "Montages and localization"
      },
      {
       "type": "li",
       "text": "**Bipolar chains** (longitudinal or transverse) compare neighboring electrodes. A discharge is localized by **phase reversal**: for a surface-negative maximum at T7 in the chain F7–T7, T7–P7, the channel above deflects down and the channel below deflects up, so the two point toward each other. A positive maximum points them away from each other."
      },
      {
       "type": "li",
       "text": "**End of the chain.** A maximum at the first or last electrode of a chain (Fp1, O1) has no neighbor on one side and shows no phase reversal. Check it in a referential montage."
      },
      {
       "type": "li",
       "text": "**Referential montages** compare every electrode with one reference (ear, Cz, the common average, or a weighted Laplacian). The maximum is the largest deflection, as long as the reference itself is quiet. An ear reference picks up temporal discharges, which then appear inverted and widespread across every channel that uses it."
      },
      {
       "type": "li",
       "text": "Bipolar montages sharpen local detail and cancel shared noise but can hide a widespread field. Referential montages preserve true waveform and amplitude but are only as good as the reference."
      },
      {
       "type": "h3",
       "text": "Filters, sensitivity and time base"
      },
      {
       "type": "table",
       "head": [
        "Setting",
        "Routine standard",
        "What changing it does"
       ],
       "rows": [
        [
         "Low-frequency filter (high-pass)",
         "1 Hz, a time constant of about 0.16 s",
         "Raising it removes slow waves, including real delta and slow sweat artifact"
        ],
        [
         "High-frequency filter (low-pass)",
         "70 Hz",
         "Lowering it smooths muscle into rhythmic sharp activity that can mimic beta or spikes"
        ],
        [
         "Notch filter",
         "60 Hz (50 Hz outside North America)",
         "Hides mains noise without fixing the electrode that caused it; use sparingly"
        ],
        [
         "Sensitivity",
         "7 µV/mm (5–10 in adults)",
         "A smaller number means more gain; 2 µV/mm for suspected electrocerebral inactivity"
        ],
        [
         "Time base",
         "30 mm/s, 10 s per page",
         "15 mm/s in neonates and for long reviews compresses slow activity"
        ]
       ]
      },
      {
       "type": "p",
       "text": "A filter can make epileptiform activity easier to see by removing competing frequencies. It cannot amplify it, and aggressive filtering creates sharp-looking transients of its own."
      },
      {
       "type": "h3",
       "text": "Sampling and aliasing"
      },
      {
       "type": "p",
       "text": "The sampling rate must be more than twice the highest frequency present (the Nyquist limit), and in practice about three to five times the high-frequency filter setting. An analogue anti-aliasing filter has to act before digitization, because an aliased signal cannot be separated afterward. A 60-Hz signal sampled at 100 Hz reappears as a false 40-Hz wave. Routine studies sample at 256–512 Hz; recording high-frequency oscillations needs 2 kHz or more."
      },
      {
       "type": "h3",
       "text": "Electrodes"
      },
      {
       "type": "li",
       "text": "The 10–20 system places electrodes at 10% or 20% of skull distances. In the modified combinatorial (10–10) names, T3/T4 became **T7/T8** and T5/T6 became **P7/P8**."
      },
      {
       "type": "li",
       "text": "An inferior temporal chain (F9/F10, T9/T10, P9/P10) samples basal temporal cortex better than the standard array and is part of the IFCN 25-electrode array."
      },
      {
       "type": "li",
       "text": "Sphenoidal and nasopharyngeal electrodes add little over the inferior chain and are rarely used now."
      },
      {
       "type": "li",
       "text": "Keep impedances low (under about 5 kΩ) and, more importantly, balanced. A near-zero impedance between two electrodes suggests a salt bridge."
      },
      {
       "type": "h3",
       "text": "Safety"
      },
      {
       "type": "p",
       "text": "Use a single ground per patient and hospital-grade equipment, keep leakage current minimal, and never let the patient be grounded through several devices at once, which creates ground loops."
      },
      {
       "type": "h3",
       "text": "Suspected electrocerebral inactivity"
      },
      {
       "type": "p",
       "text": "The technical protocol calls for at least eight scalp electrodes with interelectrode distances of 10 cm or more, sensitivity of 2 µV/mm, at least 30 minutes of recording, documented integrity of the system, and stimulation to test reactivity."
      },
      {
       "type": "p",
       "updated": true,
       "label": "Brain death",
       "text": "The 2023 AAN/AAP/CNS/SCCM guideline does not accept EEG as an ancillary test for brain death/death by neurologic criteria. EEG reflects cortical activity, not brainstem function. Electrocerebral inactivity is an EEG finding, not a determination of death."
      },
      {
       "type": "mcq",
       "id": "q-12316rx",
       "stem": "In a longitudinal bipolar chain Fp1–F7, F7–T7, T7–P7, P7–O1, a sharp transient deflects downward in F7–T7 and upward in T7–P7 at the same instant. Where is its maximum and what is its polarity?",
       "options": [
        "F7, surface positive",
        "T7, surface negative",
        "T7, surface positive",
        "P7, surface negative",
        "The finding cannot be localized in a bipolar montage"
       ],
       "answer": 1,
       "explain": "With T7 the most negative electrode, F7–T7 (F7 minus T7) is positive and deflects down, and T7–P7 is negative and deflects up. Deflections pointing toward each other mark a negative phase reversal at the shared electrode."
      },
      {
       "type": "mcq",
       "id": "q-1ypi2j3",
       "stem": "A spike is largest in the channel Fp2–F8 of the longitudinal bipolar montage and does not phase-reverse anywhere. What is the best next step?",
       "options": [
        "Report a right frontal spike with a positive field",
        "Increase the low-frequency filter to 5 Hz",
        "Review it in a referential montage, because the maximum may be at the end of the chain",
        "Discard it as eye movement",
        "Switch the notch filter off"
       ],
       "answer": 2,
       "explain": "A maximum at the first electrode of a chain has no neighbor on one side, so no phase reversal appears. A referential montage shows whether Fp2 carries the maximum. Eye movement is a possibility to consider, but not the conclusion before checking the field."
      },
      {
       "type": "mcq",
       "id": "q-12n8sj6",
       "stem": "A 60-Hz signal is digitized at 100 samples per second without an anti-aliasing filter. What appears in the recording?",
       "options": [
        "A 60-Hz sine wave of reduced amplitude",
        "A flat line",
        "A spurious 40-Hz wave",
        "A spurious 160-Hz wave",
        "Nothing; the notch filter removes it after digitization"
       ],
       "answer": 2,
       "explain": "Frequencies above half the sampling rate fold back. With a 100-Hz sampling rate a 60-Hz input appears at 100 − 60 = 40 Hz. It cannot be removed after digitization, which is why the anti-aliasing filter must act first."
      },
      {
       "type": "match",
       "id": "match-eeg-settings",
       "prompt": "Match each recording parameter to its usual value",
       "pairs": [
        [
         "Low-frequency filter, routine adult study",
         "1 Hz"
        ],
        [
         "High-frequency filter, routine adult study",
         "70 Hz"
        ],
        [
         "Sensitivity, routine adult study",
         "7 µV/mm"
        ],
        [
         "Sensitivity, suspected electrocerebral inactivity",
         "2 µV/mm"
        ],
        [
         "Time base",
         "30 mm/s"
        ],
        [
         "Sampling for high-frequency oscillations",
         "2 kHz or more"
        ]
       ]
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-1g4y4yv",
         "front": "EEG polarity convention",
         "back": "Input 1 negative relative to input 2 deflects the trace up."
        },
        {
         "id": "c-1i4jikl",
         "front": "Negative phase reversal in a bipolar chain",
         "back": "The two channels sharing the electrode deflect toward each other."
        },
        {
         "id": "c-1tc18bf",
         "front": "End-of-chain problem",
         "back": "A maximum at the first or last electrode of a chain shows no phase reversal; check a referential montage."
        },
        {
         "id": "c-ctn4jy",
         "front": "60-Hz noise in one channel",
         "back": "Impedance mismatch at one electrode, not a bad ground."
        },
        {
         "id": "c-10ls9d7",
         "front": "Time constant of a 1-Hz low-frequency filter",
         "back": "About 0.16 s (1 ÷ 2π × 1 Hz)."
        },
        {
         "id": "c-1qps833",
         "front": "Renamed 10–20 electrodes",
         "back": "T3/T4 → T7/T8 and T5/T6 → P7/P8."
        },
        {
         "id": "c-92k3xp",
         "front": "Aliasing rule",
         "back": "Sample at more than twice the highest frequency (in practice three to five times the high-frequency filter), with an anti-aliasing filter before digitization."
        }
       ]
      },
      {
       "type": "note",
       "label": "Try it",
       "text": "Place the electrodes yourself and check a montage in the [10–20 map lesson](eeg-fundamentals.html#montages-10-20-map)."
      }
     ],
     "sources": [
      {
       "title": "ACNS minimum technical standards for EEG (2016)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/27482788/"
      },
      {
       "title": "ACNS electrode position nomenclature (2016)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/27482794/"
      },
      {
       "title": "2023 US brain death consensus guideline",
       "url": "https://pubmed.ncbi.nlm.nih.gov/37821233/"
      }
     ],
     "words": 1112,
     "bank": 9
    }
   ],
   "intro": [
    "How seizures start, spread and stop, and why the scalp sees some of it and misses the rest. Everything later in the review leans on these three sections."
   ]
  },
  {
   "id": "normal",
   "n": "2",
   "title": "Normal EEG, variants and artifacts",
   "short": "Normal EEG",
   "sections": [
    {
     "id": "normal-adult",
     "n": "1",
     "title": "The normal adult EEG, awake and asleep",
     "tags": [
      "eeg-fundamentals"
     ],
     "updated": false,
     "goals": [
      "Describe the normal awake rhythms and how they react",
      "recognize the markers of each sleep stage",
      "know which asymmetries are allowed"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "Awake"
      },
      {
       "type": "li",
       "text": "**Posterior dominant rhythm (alpha).** 8.5–13 Hz in adults, maximal occipitally with eyes closed, and attenuated by eye opening and mental effort. A PDR below 8 Hz in an adult is abnormal. Amplitude is often higher on the right; an amplitude asymmetry over about 50%, or a frequency difference over 1 Hz, is abnormal. The side that fails to attenuate with eye opening is usually the abnormal one."
      },
      {
       "type": "li",
       "text": "**Beta.** Frontocentral fast activity, increased by benzodiazepines and barbiturates. A focal loss of beta suggests cortical dysfunction or fluid between cortex and electrode; a focal increase is expected over a skull defect."
      },
      {
       "type": "li",
       "text": "**Mu rhythm.** 7–11 Hz arch-shaped (arciform) central rhythm that attenuates with movement, or the thought of movement, of the opposite limb, not with eye opening."
      },
      {
       "type": "li",
       "text": "**Lambda waves.** Positive occipital sharp transients while the eyes scan a patterned scene. They disappear with eye closure."
      },
      {
       "type": "li",
       "text": "**Theta.** Small amounts of frontocentral theta are normal in drowsiness, and mild temporal theta is common in older adults."
      },
      {
       "type": "h3",
       "text": "Asleep"
      },
      {
       "type": "table",
       "head": [
        "Stage",
        "What identifies it"
       ],
       "rows": [
        [
         "N1 (drowsiness)",
         "Alpha drops out; slow roving eye movements; vertex sharp waves; positive occipital sharp transients of sleep (POSTS)"
        ],
        [
         "N2",
         "Sleep spindles (about 11–16 Hz, central) and K-complexes"
        ],
        [
         "N3",
         "High-amplitude delta (over 75 µV) filling at least 20% of the epoch"
        ],
        [
         "REM",
         "Low-voltage mixed frequencies, rapid eye movements, sawtooth waves, loss of chin muscle tone"
        ]
       ]
      },
      {
       "type": "p",
       "text": "Spindles are generated in the thalamus, paced by the reticular nucleus. REM sleep in the first minutes of a routine EEG is unusual and suggests sleep deprivation, narcolepsy, or withdrawal from REM-suppressing drugs."
      },
      {
       "type": "p",
       "text": "Photic stimulation normally produces **photic driving**: occipital activity time-locked to the flash rate or its harmonics. Its absence is not abnormal."
      },
      {
       "type": "note",
       "label": "Board pearl",
       "text": "Sleep is the best activator of focal interictal discharges. NREM sleep increases their frequency and field; REM sleep narrows the field, so a discharge that persists in REM is usually close to the focus."
      },
      {
       "type": "mcq",
       "id": "q-435kho",
       "stem": "A central 9-Hz arch-shaped rhythm on the left does not change with eye opening but attenuates when the patient is asked to make a fist with her right hand. What is it?",
       "options": [
        "Posterior dominant rhythm spreading forward",
        "Mu rhythm",
        "Wicket spikes",
        "Breach rhythm",
        "A focal seizure"
       ],
       "answer": 1,
       "explain": "Mu rhythm is a central arciform rhythm that attenuates with movement, or planned movement, of the contralateral limb and not with eye opening. It is a normal finding."
      },
      {
       "type": "mcq",
       "id": "q-1kriaho",
       "stem": "Which finding in an adult's awake EEG is abnormal?",
       "options": [
        "A 10-Hz posterior rhythm 30% higher in amplitude on the right",
        "Beta activity that increases after lorazepam",
        "A posterior dominant rhythm of 7 Hz",
        "Lambda waves while reading",
        "Mu rhythm over the left central region"
       ],
       "answer": 2,
       "explain": "An adult posterior dominant rhythm below 8 Hz is slow and suggests a diffuse encephalopathy or medication effect. The other findings are normal."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-i30ip7",
         "front": "Normal adult posterior dominant rhythm",
         "back": "8.5–13 Hz, occipital, attenuates with eye opening. Below 8 Hz in an adult is abnormal."
        },
        {
         "id": "c-8qamoc",
         "front": "Abnormal alpha asymmetry",
         "back": "Amplitude difference over about 50%, or frequency difference over 1 Hz."
        },
        {
         "id": "c-1masxz8",
         "front": "Mu rhythm reacts to",
         "back": "Movement or planned movement of the opposite limb, not eye opening."
        },
        {
         "id": "c-g7ai9o",
         "front": "Lambda waves",
         "back": "Positive occipital sharp transients during visual scanning with the eyes open."
        },
        {
         "id": "c-21zrqx",
         "front": "N2 sleep markers",
         "back": "Sleep spindles and K-complexes."
        },
        {
         "id": "c-164j1kl",
         "front": "N3 sleep",
         "back": "Delta over 75 µV in at least 20% of the epoch."
        },
        {
         "id": "c-9g0krw",
         "front": "REM markers",
         "back": "Low-voltage mixed activity, rapid eye movements, sawtooth waves, atonia."
        },
        {
         "id": "c-e0cvr0",
         "front": "Where spindles come from",
         "back": "The thalamus, paced by the reticular nucleus."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "IFCN glossary of EEG terms (2017)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/30214992/"
      },
      {
       "title": "ACNS minimum technical standards (2016)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/27482788/"
      }
     ],
     "words": 557,
     "bank": 8
    },
    {
     "id": "benign-variants",
     "n": "2",
     "title": "Normal variants that look epileptiform",
     "tags": [
      "eeg-fundamentals",
      "acns-terminology"
     ],
     "updated": false,
     "goals": [
      "Recognize the variants most often misread as spikes",
      "apply the IFCN criteria for a true interictal discharge",
      "explain the cost of over-reading"
     ],
     "blocks": [
      {
       "type": "p",
       "text": "A variant is a pattern that looks unusual but carries no link to epilepsy. The trap is that most of them appear in drowsiness and light sleep, just when real discharges are also activated."
      },
      {
       "type": "table",
       "head": [
        "Variant",
        "Who and when",
        "How to recognize it"
       ],
       "rows": [
        [
         "Small sharp spikes (benign epileptiform transients of sleep)",
         "Adults, drowsiness and light sleep",
         "Under 50 µV and under 50 ms, mono- or diphasic, often bilateral independent; no aftergoing slow wave; vanish in deeper sleep"
        ],
        [
         "Wicket spikes",
         "Older adults, drowsiness",
         "6–11 Hz arch-shaped temporal waves singly or in trains; no slow wave, no background disruption"
        ],
        [
         "14- and 6-Hz positive bursts",
         "Adolescents, light sleep",
         "Positive comb-like bursts posterior temporal; best seen with a contralateral ear reference"
        ],
        [
         "6-Hz spike-and-wave (phantom)",
         "Adolescents and adults",
         "Brief, low-amplitude 5–7 Hz bursts. Anterior, high-amplitude bursts in waking men have more link to epilepsy than occipital, low-amplitude bursts in drowsy women"
        ],
        [
         "Rhythmic mid-temporal theta of drowsiness",
         "Young adults, drowsiness",
         "5–7 Hz notched, flat-topped temporal theta; no evolution"
        ],
        [
         "Subclinical rhythmic electrographic discharge of adults (SREDA)",
         "Over about 50",
         "Abrupt rhythmic theta–delta, usually parietal–posterior temporal, lasting tens of seconds; the patient is unaffected and there is no postictal slowing"
        ],
        [
         "Midline theta (Ciganek rhythm)",
         "Adults, wake or drowsiness",
         "5–7 Hz rhythmic theta at Cz"
        ],
        [
         "Hypnagogic hypersynchrony",
         "Children, about 3 months to 13 years",
         "Bursts of high-amplitude 3–5 Hz activity at sleep onset, sometimes with sharp components"
        ],
        [
         "Posterior slow waves of youth",
         "Children and adolescents",
         "Delta mixed into the posterior rhythm, attenuating with eye opening"
        ],
        [
         "Breach rhythm",
         "After craniotomy",
         "Sharper, faster, higher-amplitude activity over the bone defect"
        ]
       ]
      },
      {
       "type": "figure",
       "html": "variant-gallery",
       "caption": "The variants drawn at one scale. Small sharp spikes are tiny and brief; wicket spikes and the positive bursts are arch- or comb-shaped trains; SREDA starts and stops abruptly with no slowing afterward. Synthetic sketches, not recordings.",
       "n": 1
      },
      {
       "type": "h3",
       "text": "When is a sharp wave a real discharge?"
      },
      {
       "type": "p",
       "text": "The IFCN lists six features of an interictal epileptiform discharge:"
      },
      {
       "type": "li",
       "text": "Di- or triphasic waveform with a sharp or spiky morphology"
      },
      {
       "type": "li",
       "text": "A different duration from the surrounding background (shorter or longer)"
      },
      {
       "type": "li",
       "text": "An asymmetric waveform: a steep phase on one side and a slower phase on the other"
      },
      {
       "type": "li",
       "text": "An aftergoing slow wave"
      },
      {
       "type": "li",
       "text": "Disruption of the background around it"
      },
      {
       "type": "li",
       "text": "A field over the scalp that fits a real cortical source"
      },
      {
       "type": "figure",
       "html": "ied-criteria",
       "caption": "The six features marked on a left temporal spike-and-wave, and a wicket spike train, which has only the sharp look and a field.",
       "n": 2
      },
      {
       "type": "p",
       "text": "In a clinical validation study, requiring at least five of the six gave specificity above 95%; four of six was a little more sensitive but less specific. Spike means 20–70 ms and sharp wave 70–200 ms; the distinction is duration only and carries no clinical weight."
      },
      {
       "type": "note",
       "label": "Board pearl",
       "text": "If in doubt, do not call it. A false \"epileptiform\" report is one of the commonest routes to a wrong epilepsy diagnosis, lifelong medication, and loss of a driving license."
      },
      {
       "type": "case",
       "id": "case-sharp-transient",
       "title": "Is this spike real?",
       "intro": [
        "A 58-year-old man is referred after one episode of transient confusion. His routine EEG was read elsewhere as \"left temporal epileptiform discharges\"."
       ],
       "steps": [
        {
         "narrative": [
          "You review the study. In drowsiness there are trains of 8-Hz arch-shaped waves over the left mid-temporal region, each wave sharp-looking. None has an aftergoing slow wave and the background between them is normal."
         ],
         "stem": "What is the most likely pattern?",
         "options": [
          "Left temporal spikes",
          "Wicket spikes, a normal variant",
          "Rhythmic mid-temporal theta of drowsiness",
          "Small sharp spikes",
          "Lambda waves"
         ],
         "answer": 1,
         "explain": "Wicket spikes are 6–11 Hz arch-shaped temporal waves in older adults during drowsiness, singly or in trains, without an aftergoing slow wave or background disruption."
        },
        {
         "narrative": [
          "He was started on levetiracetam after the first report and now feels irritable. He has had no further events."
         ],
         "stem": "What is the best next step?",
         "options": [
          "Add lamotrigine for better control",
          "Obtain a PET scan to find the focus",
          "Revisit the diagnosis: reassess the event itself and consider stopping the ASM",
          "Continue levetiracetam indefinitely because the EEG was abnormal",
          "Order sphenoidal electrodes"
         ],
         "answer": 2,
         "explain": "The EEG does not support epilepsy. The diagnosis rests on the event, which needs its own work-up (transient global amnesia, syncope, TIA, a seizure). Treating a normal variant exposes the patient to side effects without benefit."
        }
       ],
       "outro": [
        "Over-read variants, most often wicket spikes, small sharp spikes and rhythmic mid-temporal theta, are a leading cause of epilepsy misdiagnosis. The EEG report should say \"normal variant\" plainly."
       ]
      },
      {
       "type": "mcq",
       "id": "q-jdf0y4",
       "stem": "Which feature best distinguishes small sharp spikes from a true interictal epileptiform discharge?",
       "options": [
        "They appear only in wakefulness",
        "They are always unilateral",
        "They are low in amplitude and very brief, with no aftergoing slow wave, and disappear in deeper sleep",
        "They are followed by a prominent slow wave",
        "They phase-reverse at a single electrode"
       ],
       "answer": 2,
       "explain": "Small sharp spikes are under 50 µV and 50 ms, occur in drowsiness and light sleep, are often bilateral independent, have no slow wave and disappear in deep sleep."
      },
      {
       "type": "mcq",
       "id": "q-1fd7jbl",
       "stem": "A 70-year-old woman has a 40-second run of abrupt rhythmic 5-Hz activity over the parietal regions during wakefulness. She keeps talking normally throughout, and there is no slowing afterward. What is it?",
       "options": [
        "Subclinical rhythmic electrographic discharge of adults (SREDA)",
        "A focal seizure with preserved consciousness",
        "Rhythmic mid-temporal theta of drowsiness",
        "Lateralized rhythmic delta activity",
        "Hypnagogic hypersynchrony"
       ],
       "answer": 0,
       "explain": "SREDA occurs in older adults, is abrupt and rhythmic, lasts tens of seconds, has no clinical accompaniment and no postictal change. It can be mistaken for a seizure because it has a start and an end."
      },
      {
       "type": "match",
       "id": "match-variants",
       "prompt": "Match each variant to its defining feature",
       "pairs": [
        [
         "Wicket spikes",
         "Arch-shaped 6–11 Hz temporal trains in older adults"
        ],
        [
         "14- and 6-Hz positive bursts",
         "Positive comb-like posterior temporal bursts in adolescents"
        ],
        [
         "Mu rhythm",
         "Attenuates with movement of the opposite limb"
        ],
        [
         "Lambda waves",
         "Occipital positive transients during visual scanning"
        ],
        [
         "Hypnagogic hypersynchrony",
         "High-amplitude 3–5 Hz bursts at sleep onset in children"
        ],
        [
         "Breach rhythm",
         "Sharper, faster activity over a skull defect"
        ]
       ]
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-1r0vkxv",
         "front": "IFCN features of an interictal epileptiform discharge",
         "back": "Sharp di- or triphasic waveform, different duration from background, asymmetric phases, aftergoing slow wave, background disruption, plausible scalp field."
        },
        {
         "id": "c-18nktj0",
         "front": "How many IFCN criteria for high specificity",
         "back": "At least five of six (specificity above 95% in validation)."
        },
        {
         "id": "c-j04qke",
         "front": "Spike versus sharp wave",
         "back": "Spike 20–70 ms; sharp wave 70–200 ms. Duration only; no clinical difference."
        },
        {
         "id": "c-aj4yc6",
         "front": "6-Hz spike-and-wave more linked to epilepsy",
         "back": "Waking, high-amplitude, anterior, in men (WHAM), versus female, occipital, low-amplitude, drowsy (FOLD)."
        },
        {
         "id": "c-d3y9kg",
         "front": "SREDA",
         "back": "Older adults; abrupt rhythmic theta–delta posteriorly lasting tens of seconds, no clinical change, no postictal slowing."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "IFCN glossary and IED criteria (Kane 2017)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/30214992/"
      },
      {
       "title": "Validation of the IFCN criteria (Kural 2020)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/32321764/"
      }
     ],
     "words": 985,
     "bank": 14
    },
    {
     "id": "artifacts",
     "n": "3",
     "title": "Artifacts",
     "tags": [
      "eeg-fundamentals"
     ],
     "updated": false,
     "goals": [
      "Identify eye, muscle, cardiac and electrode artifacts from their field",
      "name the ICU artifacts that mimic seizures",
      "use video and extra channels to settle doubt"
     ],
     "blocks": [
      {
       "type": "p",
       "text": "The single most useful question is whether the field makes sense. Brain activity spreads to neighboring electrodes with a gradient; an electrode artifact stays in one place, and a cardiac artifact keeps time with the ECG."
      },
      {
       "type": "h3",
       "text": "Physiological artifacts"
      },
      {
       "type": "li",
       "text": "**Eyes.** The cornea is positive relative to the retina. On a blink the eyes roll up (Bell phenomenon) and Fp1/Fp2 go positive. Looking left brings the cornea toward F7, which goes positive while F8 goes negative. **Lateral rectus spikes** are brief muscle potentials at F7 or F8 at the start of each horizontal saccade. Slow lateral roving eye movements mark drowsiness."
      },
      {
       "type": "li",
       "text": "**Muscle.** Very fast, spiky activity over the frontalis and temporalis muscles; rhythmic bursts with chewing. Lowering the high-frequency filter makes muscle look like rhythmic beta or spikes."
      },
      {
       "type": "li",
       "text": "**Glossokinetic.** The tongue is also a dipole (tip negative). Talking and swallowing produce frontotemporal slow waves."
      },
      {
       "type": "li",
       "text": "**Cardiac.** ECG artifact is time-locked to the QRS complex, often most obvious in referential montages to the ear and in people with short, thick necks. Pulse artifact is a slow wave at one electrode over an artery, about 200 ms after the QRS."
      },
      {
       "type": "li",
       "text": "**Sweat.** Very slow (under 0.5 Hz) drifting potentials."
      },
      {
       "type": "h3",
       "text": "Electrodes and equipment"
      },
      {
       "type": "li",
       "text": "**Electrode pop.** Abrupt, steep transient confined to one electrode with no field."
      },
      {
       "type": "li",
       "text": "**Salt bridge.** Paste joins two electrodes; the channel between them goes nearly flat."
      },
      {
       "type": "li",
       "text": "**Mains interference.** 60 Hz (50 Hz in Europe); in one channel, a high-impedance electrode."
      },
      {
       "type": "li",
       "text": "**ICU sources.** Ventilator (rhythmic slow waves at the breathing rate), IV pumps and drips, bed vibration and percussion, chest physiotherapy, ECMO and pacemakers. Check the rate against the device and look at the video before calling a seizure."
      },
      {
       "type": "mcq",
       "id": "q-nj37c8",
       "stem": "A patient in the ICU has rhythmic 0.3-Hz sharp slow waves in all channels. They occur 18 times a minute, which matches the ventilator setting, and do not evolve. What should you do first?",
       "options": [
        "Load levetiracetam",
        "Correlate with the ventilator and video; this is most likely ventilator artifact",
        "Call it generalized periodic discharges",
        "Report electrographic status epilepticus",
        "Increase the low-frequency filter to 5 Hz and report what remains"
       ],
       "answer": 1,
       "explain": "A rhythmic pattern at exactly the device rate, without evolution or a cerebral field, is artifact until proven otherwise. Treating artifact exposes the patient to unnecessary drugs and sedation."
      },
      {
       "type": "mcq",
       "id": "q-160pwme",
       "stem": "On eye opening and closing, which electrodes show the largest positive deflection from a blink?",
       "options": [
        "Fp1 and Fp2",
        "F7 and F8",
        "T7 and T8",
        "O1 and O2",
        "Cz"
       ],
       "answer": 0,
       "explain": "With a blink the eyes roll upward (Bell phenomenon) and the positively charged cornea moves toward the frontopolar electrodes."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-j7fhnb",
         "front": "Cornea polarity",
         "back": "Positive relative to the retina."
        },
        {
         "id": "c-mhi3jp",
         "front": "Blink artifact",
         "back": "Positive at Fp1 and Fp2 as the eyes roll up."
        },
        {
         "id": "c-1p7uu3t",
         "front": "Looking to the left",
         "back": "F7 goes positive, F8 negative."
        },
        {
         "id": "c-1ct9m9c",
         "front": "Lateral rectus spike",
         "back": "Brief muscle potential at F7 or F8 at the start of a horizontal saccade."
        },
        {
         "id": "c-2ni7k0",
         "front": "Pulse artifact",
         "back": "Slow wave at one electrode over an artery about 200 ms after the QRS."
        },
        {
         "id": "c-dlrw7f",
         "front": "Electrode pop",
         "back": "Abrupt transient confined to one electrode, with no field."
        },
        {
         "id": "c-1h559pm",
         "front": "Salt bridge",
         "back": "Near-flat channel between two electrodes joined by paste."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "IFCN glossary of EEG terms (2017)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/30214992/"
      },
      {
       "title": "ACNS critical care EEG terminology (2021)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/33475321/"
      }
     ],
     "words": 499,
     "bank": 13
    },
    {
     "id": "maturation",
     "n": "4",
     "title": "From preterm to adolescence",
     "tags": [
      "eeg-fundamentals"
     ],
     "updated": false,
     "goals": [
      "Date a neonatal EEG from its patterns",
      "recognize abnormal neonatal backgrounds",
      "know when the posterior rhythm and sleep markers appear in childhood"
     ],
     "blocks": [
      {
       "type": "p",
       "text": "Neonatal EEG is read against **postmenstrual age** (gestational age plus weeks since birth), because the brain matures on its own schedule whether the baby is inside or outside the womb. A background that lags two weeks or more behind the expected age is **dysmature**."
      },
      {
       "type": "h3",
       "text": "Neonatal landmarks"
      },
      {
       "type": "table",
       "head": [
        "Postmenstrual age",
        "What you expect"
       ],
       "rows": [
        [
         "Under about 30 weeks",
         "Discontinuous background (tracé discontinu) with long, very flat interburst intervals; little difference between states"
        ],
        [
         "About 26–30 weeks",
         "Temporal theta bursts (temporal sawtooth)"
        ],
        [
         "About 28–36 weeks",
         "Delta brushes (fast activity riding on delta), most prominent around 32–34 weeks and largely gone by term"
        ],
        [
         "About 34–44 weeks",
         "Frontal sharp transients (encoches frontales) and rhythmic frontal delta (anterior dysrhythmia) in transitional sleep"
        ],
        [
         "Term",
         "Continuous in wake and active sleep; tracé alternant (bursts with lower-voltage, not flat, intervals) in quiet sleep"
        ],
        [
         "About 44–46 weeks",
         "Tracé alternant gives way to continuous slow-wave sleep"
        ]
       ]
      },
      {
       "type": "h3",
       "text": "Abnormal neonatal backgrounds"
      },
      {
       "type": "li",
       "text": "Burst-suppression or a persistently low-voltage, invariant background (the strongest markers of poor outcome after hypoxic-ischemic injury)"
      },
      {
       "type": "li",
       "text": "Absent sleep–wake cycling at an age when it should be present"
      },
      {
       "type": "li",
       "text": "Persistent asymmetry or focal attenuation"
      },
      {
       "type": "li",
       "text": "Positive rolandic sharp waves, which point to white matter injury"
      },
      {
       "type": "li",
       "text": "Excessive multifocal sharp transients for age"
      },
      {
       "type": "h3",
       "text": "Childhood"
      },
      {
       "type": "table",
       "head": [
        "Age",
        "Landmark"
       ],
       "rows": [
        [
         "About 6–8 weeks after term",
         "Sleep spindles appear (asynchronous until about age 2)"
        ],
        [
         "About 3–4 months",
         "Posterior rhythm about 4 Hz; hypnagogic hypersynchrony begins to appear"
        ],
        [
         "About 5–6 months",
         "Vertex waves and K-complexes"
        ],
        [
         "About 12 months",
         "Posterior rhythm about 6 Hz"
        ],
        [
         "About 3 years",
         "Posterior rhythm reaches 8 Hz"
        ],
        [
         "Later childhood",
         "9–10 Hz, with posterior slow waves of youth common into adolescence"
        ]
       ]
      },
      {
       "type": "figure",
       "html": "maturation",
       "caption": "The landmarks in the two tables above as timelines. Neonatal patterns are dated by postmenstrual age; the posterior rhythm speeds up through childhood. Point at or tab to a bar or point for its age range.",
       "n": 3
      },
      {
       "type": "mcq",
       "id": "q-1br7nj1",
       "stem": "An EEG in an infant shows frequent delta brushes in all states, temporal sawtooth theta, and a discontinuous background without clear state differentiation. What postmenstrual age fits best?",
       "options": [
        "24 weeks",
        "30 weeks",
        "38 weeks",
        "44 weeks",
        "3 months after term"
       ],
       "answer": 1,
       "explain": "Temporal theta is prominent around 26–30 weeks and delta brushes are abundant by then; the background is still discontinuous without clear state change. At term, delta brushes are sparse and quiet sleep shows tracé alternant."
      },
      {
       "type": "mcq",
       "id": "q-14g2t79",
       "stem": "At what age does the posterior dominant rhythm typically first reach 8 Hz?",
       "options": [
        "4 months",
        "12 months",
        "About 3 years",
        "8 years",
        "15 years"
       ],
       "answer": 2,
       "explain": "The posterior rhythm is about 4 Hz at 3–4 months, 6 Hz at a year and 8 Hz by about 3 years, rising to 9–10 Hz through childhood."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-1x2n14x",
         "front": "Postmenstrual age",
         "back": "Gestational age at birth plus weeks since birth; the age a neonatal EEG is read against."
        },
        {
         "id": "c-1pw0ask",
         "front": "Dysmaturity",
         "back": "A background lagging two weeks or more behind postmenstrual age."
        },
        {
         "id": "c-1qh37ko",
         "front": "Delta brushes peak",
         "back": "About 32–34 weeks; largely gone by term."
        },
        {
         "id": "c-rmouc9",
         "front": "Tracé alternant",
         "back": "Term quiet sleep: bursts alternating with lower-voltage (not flat) intervals; gone by about 44–46 weeks."
        },
        {
         "id": "c-1u2boax",
         "front": "Encoches frontales",
         "back": "Frontal sharp transients in transitional sleep, about 34–44 weeks."
        },
        {
         "id": "c-g98ynu",
         "front": "Positive rolandic sharp waves",
         "back": "Marker of white matter injury in the preterm brain."
        },
        {
         "id": "c-kha5l0",
         "front": "When spindles appear",
         "back": "About 6–8 weeks after term; synchronous by about age 2."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "ACNS standardized neonatal EEG terminology (2013)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/23545767/"
      },
      {
       "title": "ACNS guideline on continuous EEG in neonates (2011)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/22146359/"
      }
     ],
     "words": 515,
     "bank": 11
    },
    {
     "id": "activation",
     "n": "5",
     "title": "Activation procedures",
     "tags": [
      "eeg-fundamentals"
     ],
     "updated": false,
     "goals": [
      "Know what hyperventilation, photic stimulation and sleep add",
      "tell a photoparoxysmal from a photomyogenic response",
      "list the contraindications to hyperventilation"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "Hyperventilation"
      },
      {
       "type": "p",
       "text": "Three to five minutes of deep breathing, ideally eyes closed. It reliably provokes absences in untreated childhood and juvenile absence epilepsy, so a routine EEG without hyperventilation can miss the diagnosis. Slow, high-amplitude **buildup** is normal in children and in hypoglycemia; it should settle within about a minute of stopping. Avoid hyperventilation after recent stroke, TIA or intracranial hemorrhage, in moyamoya, sickle cell disease or trait, significant cardiopulmonary disease and raised intracranial pressure."
      },
      {
       "type": "h3",
       "text": "Photic stimulation"
      },
      {
       "type": "p",
       "text": "Flashes at a range of frequencies, typically about 1 to 30 Hz, with eyes open, closing and closed. Eye closure is the most provocative moment."
      },
      {
       "type": "li",
       "text": "**Photoparoxysmal response.** Generalized spike- or polyspike-and-wave evoked by the flashes: a cortical, epileptic response most common in juvenile myoclonic epilepsy and eyelid myoclonia with absences."
      },
      {
       "type": "li",
       "text": "**Photomyogenic (photomyoclonic) response.** Frontal muscle jerks time-locked to the flash that stop when the flashes stop. It reflects muscle, often with anxiety, alcohol or sedative withdrawal, not epilepsy."
      },
      {
       "type": "li",
       "text": "**Photic driving** at the flash rate is normal."
      },
      {
       "type": "h3",
       "text": "Sleep and sleep deprivation"
      },
      {
       "type": "p",
       "text": "Sleep increases interictal discharges, especially in NREM. Sleep deprivation raises the yield further, partly independently of the sleep it produces. A sleep-deprived EEG is the usual next step after a normal routine study when suspicion remains."
      },
      {
       "type": "mcq",
       "id": "q-2jcui6",
       "stem": "During photic stimulation there are frontal spike-like potentials time-locked to each flash, with visible eyelid and forehead twitching. They stop the moment the stimulator is switched off. What is this?",
       "options": [
        "Photoparoxysmal response",
        "Photomyogenic response",
        "Photic driving",
        "Occipital spikes of photosensitive epilepsy",
        "Eyelid myoclonia"
       ],
       "answer": 1,
       "explain": "A photomyogenic response is muscle activity time-locked to the flash in the frontal regions that ends with the stimulus. It is not an epileptic response."
      },
      {
       "type": "mcq",
       "id": "q-fpimni",
       "stem": "In which patient should hyperventilation be omitted?",
       "options": [
        "A 7-year-old with suspected absence seizures",
        "A 25-year-old with suspected juvenile myoclonic epilepsy",
        "A 16-year-old with sickle cell disease",
        "A 40-year-old with focal seizures and a normal MRI",
        "A 70-year-old with transient confusion and no cardiopulmonary disease"
       ],
       "answer": 2,
       "explain": "Hyperventilation lowers cerebral blood flow and is avoided in sickle cell disease or trait, moyamoya, recent stroke, TIA or hemorrhage, significant cardiopulmonary disease and raised intracranial pressure."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-w9whss",
         "front": "Hyperventilation duration",
         "back": "Three to five minutes."
        },
        {
         "id": "c-1a8wo1u",
         "front": "Normal hyperventilation buildup",
         "back": "High-amplitude slowing, common in children and with low glucose; settles within about a minute."
        },
        {
         "id": "c-vvzih1",
         "front": "Contraindications to hyperventilation",
         "back": "Recent stroke, TIA or hemorrhage; moyamoya; sickle cell disease or trait; significant cardiopulmonary disease; raised intracranial pressure."
        },
        {
         "id": "c-17gkuwn",
         "front": "Most provocative moment in photic stimulation",
         "back": "Eye closure."
        },
        {
         "id": "c-1n48vo8",
         "front": "Photoparoxysmal response",
         "back": "Generalized spike-wave evoked by flashes; epileptic; most common in JME and eyelid myoclonia with absences."
        },
        {
         "id": "c-fcsaaf",
         "front": "Photomyogenic response",
         "back": "Frontal muscle time-locked to flashes, stops with the stimulus; not epileptic."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "ACNS minimum technical standards (2016)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/27482788/"
      },
      {
       "title": "ILAE idiopathic generalized epilepsy syndromes (2022)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/35503716/"
      }
     ],
     "words": 422,
     "bank": 5
    }
   ],
   "intro": [
    "Most EEG errors are over-reading: a normal variant or an artifact called epileptiform, and a patient treated for epilepsy they do not have. This part is about knowing normal well enough to leave it alone."
   ]
  },
  {
   "id": "abnormal",
   "n": "3",
   "title": "Epileptiform and ictal EEG",
   "short": "Epileptiform EEG",
   "sections": [
    {
     "id": "ieds",
     "n": "1",
     "title": "Interictal epileptiform discharges",
     "tags": [
      "eeg-fundamentals",
      "ilae-classification"
     ],
     "updated": false,
     "goals": [
      "Weigh an interictal discharge by its location and the clinical question",
      "estimate the yield of routine, repeat and sleep-deprived EEG",
      "recognize the nonepileptiform abnormalities that still localize"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "What a discharge means"
      },
      {
       "type": "p",
       "text": "An interictal epileptiform discharge (IED) raises the probability that a person who has had a seizure has epilepsy and predicts recurrence after a first unprovoked seizure. It does not diagnose epilepsy on its own: a small percentage of healthy adults and a few percent of healthy children have them, most often centrotemporal or generalized discharges in children."
      },
      {
       "type": "p",
       "text": "Location matters:"
      },
      {
       "type": "li",
       "text": "**Anterior temporal spikes** are strongly associated with seizures."
      },
      {
       "type": "li",
       "text": "**Centrotemporal spikes** are common in school-age children, and most children who have them never have a seizure."
      },
      {
       "type": "li",
       "text": "**Occipital spikes** in young children, particularly with fixation-off sensitivity, often belong to self-limited syndromes."
      },
      {
       "type": "li",
       "text": "**Temporal intermittent rhythmic delta activity** (TIRDA) carries roughly the same meaning as temporal spikes in temporal lobe epilepsy."
      },
      {
       "type": "li",
       "text": "**Generalized spike-and-wave** at 3 Hz with a normal background points to an idiopathic generalized epilepsy; even brief subclinical bursts can impair attention in a child."
      },
      {
       "type": "p",
       "text": "In temporal lobe epilepsy, bilateral independent temporal spikes are common. When about 80% or more come from one side, seizures usually start on that side."
      },
      {
       "type": "h3",
       "text": "Yield"
      },
      {
       "type": "li",
       "text": "A first routine EEG shows IEDs in roughly a third to a half of people with epilepsy."
      },
      {
       "type": "li",
       "text": "Yield rises with repeat studies, sleep and sleep deprivation, and recording soon (within a day or two) after a seizure."
      },
      {
       "type": "li",
       "text": "Prolonged ambulatory or inpatient video-EEG is the definitive test when routine studies are unhelpful."
      },
      {
       "type": "h3",
       "text": "Abnormal but not epileptiform"
      },
      {
       "type": "li",
       "text": "**Focal polymorphic delta**, continuous and arrhythmic, localizes a structural lesion, usually involving white matter."
      },
      {
       "type": "li",
       "text": "**Focal attenuation** means dysfunctional cortex or something between cortex and electrode (subdural fluid, hematoma)."
      },
      {
       "type": "li",
       "text": "**Frontal intermittent rhythmic delta (FIRDA)** is nonspecific: encephalopathy, raised pressure or deep lesions."
      },
      {
       "type": "li",
       "text": "**Occipital intermittent rhythmic delta (OIRDA)** in a child is supportive of absence epilepsy."
      },
      {
       "type": "mcq",
       "id": "q-1dzwm1h",
       "stem": "A 34-year-old has a first unprovoked generalized convulsion. Neurological examination and MRI are normal. A routine EEG the next day is normal. What is the best interpretation?",
       "options": [
        "Epilepsy is excluded",
        "The event was psychogenic",
        "A normal EEG lowers but does not remove the chance of epilepsy; a sleep-deprived EEG can raise the yield",
        "ASM treatment is mandatory",
        "An intracranial EEG is indicated"
       ],
       "answer": 2,
       "explain": "A single routine EEG misses interictal discharges in about half or more of people with epilepsy. Repeat or sleep-deprived recordings increase the yield; the decision to treat rests on the whole picture of recurrence risk."
      },
      {
       "type": "mcq",
       "id": "q-1pld4a8",
       "stem": "Which interictal finding on a routine EEG has the weakest association with actually having seizures?",
       "options": [
        "Right anterior temporal spikes in an adult",
        "Centrotemporal spikes in an 8-year-old evaluated for headaches",
        "Left temporal intermittent rhythmic delta activity",
        "Generalized 3-Hz spike-and-wave in a 7-year-old with staring spells",
        "Right frontal spikes in a patient with nocturnal hypermotor events"
       ],
       "answer": 1,
       "explain": "Centrotemporal spikes are common in healthy school-age children and most never have seizures. The other findings are strongly linked to epilepsy in their clinical context."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-nnrfh1",
         "front": "Does an IED diagnose epilepsy?",
         "back": "No. It raises the probability and predicts recurrence, but healthy people can have them."
        },
        {
         "id": "c-dzefhd",
         "front": "Yield of a first routine EEG in epilepsy",
         "back": "About a third to a half."
        },
        {
         "id": "c-ue49ao",
         "front": "How to raise EEG yield",
         "back": "Repeat studies, sleep, sleep deprivation, early recording after a seizure, prolonged video-EEG."
        },
        {
         "id": "c-1n6a177",
         "front": "TIRDA",
         "back": "Temporal intermittent rhythmic delta; as significant as temporal spikes for temporal lobe epilepsy."
        },
        {
         "id": "c-14znqit",
         "front": "FIRDA",
         "back": "Frontal intermittent rhythmic delta; nonspecific (encephalopathy, deep lesions)."
        },
        {
         "id": "c-925jyu",
         "front": "OIRDA",
         "back": "Occipital intermittent rhythmic delta; supports absence epilepsy in a child."
        },
        {
         "id": "c-1lnpby8",
         "front": "Continuous polymorphic delta",
         "back": "Localizes a structural lesion, usually involving white matter."
        }
       ]
      },
      {
       "type": "note",
       "label": "Try it",
       "text": "[Interictal discharges in EEG Fundamentals](eeg-fundamentals.html#waveforms-interictal-discharges) shows each defining feature on a tracing."
      }
     ],
     "sources": [
      {
       "title": "IFCN glossary of EEG terms (2017)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/30214992/"
      },
      {
       "title": "AAN/AES first unprovoked seizure guideline (2015)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/25901057/"
      }
     ],
     "words": 566,
     "bank": 9
    },
    {
     "id": "ictal-scalp",
     "n": "2",
     "title": "Seizures on the EEG",
     "tags": [
      "eeg-fundamentals",
      "epilepsy-surgery"
     ],
     "updated": false,
     "goals": [
      "Recognize evolution as the core of an ictal pattern",
      "use onset patterns and postictal signs to localize",
      "know which seizures scalp EEG misses"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "Evolution is the signature"
      },
      {
       "type": "p",
       "text": "A seizure pattern changes as it goes: its frequency, shape or location shifts in sequence, usually building then fading. Artifact and rhythmic encephalopathic patterns tend to stay the same. In the ICU the ACNS 2021 definition makes this operational: discharges averaging more than 2.5 Hz for at least 10 seconds, or any pattern with definite evolution lasting at least 10 seconds (see the critical care part)."
      },
      {
       "type": "h3",
       "text": "Scalp onset patterns"
      },
      {
       "type": "li",
       "text": "**Mesial temporal onset** typically shows a regular 5–9 Hz rhythm over the inferior temporal electrodes, appearing within about 30 seconds of clinical onset."
      },
      {
       "type": "li",
       "text": "**Neocortical temporal onset** tends to be slower, more irregular and less well lateralized."
      },
      {
       "type": "li",
       "text": "**Frontal lobe seizures** are often brief, from sleep and hypermotor; the scalp EEG is frequently hidden by movement artifact or shows little change."
      },
      {
       "type": "li",
       "text": "**Attenuation (electrodecrement)**, rhythmic spiking and rhythmic delta are other common onset forms."
      },
      {
       "type": "h3",
       "text": "Intracranial onset patterns"
      },
      {
       "type": "p",
       "text": "**Low-voltage fast activity** at onset marks the seizure-onset zone and is associated with better surgical outcome when that tissue is resected. A **hypersynchronous onset** of repetitive high-amplitude spikes is typical of hippocampal sclerosis. Widespread onset across many contacts, or clinical signs before any EEG change, suggest you are recording spread rather than onset."
      },
      {
       "type": "h3",
       "text": "After the seizure"
      },
      {
       "type": "p",
       "text": "Lateralized postictal slowing points to the side of onset. Postictal aphasia points to the dominant hemisphere, and postictal nose-wiping is usually done with the hand on the same side as the focus."
      },
      {
       "type": "note",
       "label": "Board pearl",
       "text": "A normal scalp EEG during a focal aware seizure is expected, not suspicious. Focal seizures with preserved consciousness show a scalp correlate in only a minority of cases."
      },
      {
       "type": "mcq",
       "id": "q-k3iqvi",
       "stem": "During video-EEG, a patient has a seizure with an epigastric aura, staring and oral automatisms. Twenty seconds after clinical onset a regular 6-Hz rhythm appears over F7, T7 and the inferior temporal electrode T9. What does this pattern suggest?",
       "options": [
        "Mesial temporal onset on the left",
        "Left frontal onset",
        "Neocortical lateral temporal onset",
        "A generalized seizure",
        "Artifact from chewing"
       ],
       "answer": 0,
       "explain": "A regular 5–9 Hz inferior temporal rhythm within about 30 seconds of clinical onset is typical of hippocampal onset. Neocortical temporal onsets are slower, more irregular and less lateralized."
      },
      {
       "type": "mcq",
       "id": "q-189jvr",
       "stem": "Which intracranial seizure-onset pattern best marks the seizure-onset zone and predicts a good surgical outcome if resected?",
       "options": [
        "Rhythmic delta starting in several lobes at once",
        "Attenuation appearing 20 seconds after clinical onset",
        "Low-voltage fast activity confined to a few contacts",
        "Periodic spikes after a clinical seizure has started",
        "Slowing without evolution"
       ],
       "answer": 2,
       "explain": "Focal low-voltage fast activity is the classic marker of the true onset zone. Onsets that are widespread, late, or after clinical onset suggest propagated activity."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-6bu0fg",
         "front": "What makes a pattern ictal",
         "back": "Evolution: sequential change in frequency, morphology or location."
        },
        {
         "id": "c-ncr6bm",
         "front": "Mesial temporal scalp onset",
         "back": "Regular 5–9 Hz inferior temporal rhythm within about 30 seconds of clinical onset."
        },
        {
         "id": "c-5vn7qs",
         "front": "Intracranial marker of the onset zone",
         "back": "Focal low-voltage fast activity."
        },
        {
         "id": "c-1gxasxp",
         "front": "Hypersynchronous onset",
         "back": "Repetitive high-amplitude spikes at onset; typical of hippocampal sclerosis."
        },
        {
         "id": "c-4dmkrl",
         "front": "Postictal aphasia",
         "back": "Dominant hemisphere."
        },
        {
         "id": "c-n2ioiy",
         "front": "Postictal nose-wiping",
         "back": "Usually with the hand on the side of the focus."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "ACNS critical care EEG terminology (2021)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/33475321/"
      },
      {
       "title": "ILAE 2025 seizure classification",
       "url": "https://pubmed.ncbi.nlm.nih.gov/40264351/"
      }
     ],
     "words": 495,
     "bank": 10
    },
    {
     "id": "generalized-eeg",
     "n": "3",
     "title": "Generalized patterns and what they predict",
     "tags": [
      "eeg-fundamentals",
      "ilae-classification"
     ],
     "updated": false,
     "goals": [
      "Match generalized EEG signatures to syndromes",
      "recognize the patterns of the developmental and epileptic encephalopathies",
      "avoid being misled by focal features in generalized epilepsy"
     ],
     "blocks": [
      {
       "type": "table",
       "head": [
        "Pattern",
        "Typical frequency and context",
        "Points toward"
       ],
       "rows": [
        [
         "Generalized spike-and-wave",
         "About 3 Hz, abrupt start and stop, normal background, provoked by hyperventilation",
         "Childhood absence epilepsy; juvenile absence epilepsy (often a little faster, with polyspikes)"
        ],
        [
         "Generalized polyspike-and-wave",
         "Often 3–6 Hz, on awakening, frequently photosensitive",
         "Juvenile myoclonic epilepsy"
        ],
        [
         "Slow spike-and-wave",
         "Under 2.5 Hz, with a slow background",
         "Lennox–Gastaut syndrome"
        ],
        [
         "Generalized paroxysmal fast activity",
         "About 10 Hz or faster, mostly in NREM sleep, with or without tonic seizures",
         "Lennox–Gastaut syndrome"
        ],
        [
         "Hypsarrhythmia",
         "Chaotic, very high-amplitude slowing with multifocal spikes",
         "Infantile epileptic spasms syndrome"
        ],
        [
         "Burst-suppression",
         "Bursts separated by near-flat periods, from the first weeks of life",
         "Early infantile developmental and epileptic encephalopathy"
        ],
        [
         "Spike-wave activation in sleep",
         "Near-continuous spike-and-wave in NREM sleep",
         "Developmental and epileptic encephalopathy (or epileptic encephalopathy) with spike-wave activation in sleep"
        ]
       ]
      },
      {
       "type": "figure",
       "html": "generalized-ladder",
       "caption": "Generalized patterns on one time base and one scale. Count the complexes in a second to separate 3-Hz spike-and-wave, faster polyspike-and-wave and slow spike-and-wave. Synthetic sketches, not recordings.",
       "n": 4
      },
      {
       "type": "h3",
       "text": "Details that get tested"
      },
      {
       "type": "li",
       "text": "An **epileptic spasm** on EEG is usually a high-amplitude slow wave followed by attenuation, sometimes with superimposed fast activity."
      },
      {
       "type": "li",
       "text": "In **epilepsy with myoclonic absences**, 3-Hz spike-and-wave is accompanied by rhythmic jerks of the shoulders and arms."
      },
      {
       "type": "li",
       "text": "**Idiopathic generalized epilepsies can look focal.** Fragments of generalized discharges often appear maximal on one side, and myoclonic or tonic–clonic seizures may start asymmetrically. Mistaking this for focal epilepsy leads to sodium channel blockers that worsen absences and myoclonus."
      },
      {
       "type": "li",
       "text": "The background is normal in idiopathic generalized epilepsy. A slow, disorganized background with generalized discharges argues for a developmental and epileptic encephalopathy."
      },
      {
       "type": "mcq",
       "id": "q-1dgmxqb",
       "stem": "A 15-year-old has morning jerks of her arms, one generalized convulsion after an all-night party, and a routine EEG with 4–5 Hz generalized polyspike-and-wave that is activated by photic stimulation. Some discharges are maximal on the right. What is the diagnosis and the key treatment pitfall?",
       "options": [
        "Right frontal lobe epilepsy; start carbamazepine",
        "Juvenile myoclonic epilepsy; avoid carbamazepine and oxcarbazepine",
        "Childhood absence epilepsy; start ethosuximide",
        "Lennox–Gastaut syndrome; start rufinamide",
        "Juvenile absence epilepsy; start phenytoin"
       ],
       "answer": 1,
       "explain": "Morning myoclonus, a convulsion after sleep deprivation, and photosensitive generalized polyspike-and-wave define juvenile myoclonic epilepsy. Asymmetric discharges are common and should not redirect the diagnosis. Carbamazepine and oxcarbazepine can worsen myoclonus and absences."
      },
      {
       "type": "mcq",
       "id": "q-1b48ify",
       "stem": "Which EEG finding best fits Lennox–Gastaut syndrome?",
       "options": [
        "3-Hz spike-and-wave provoked by hyperventilation with a normal background",
        "Slow spike-and-wave under 2.5 Hz and bursts of fast activity in sleep, with a slow background",
        "Hypsarrhythmia",
        "Centrotemporal spikes activated by sleep",
        "Generalized polyspike-and-wave provoked by photic stimulation"
       ],
       "answer": 1,
       "explain": "Lennox–Gastaut syndrome is defined by multiple seizure types including tonic seizures, slow spike-and-wave, and generalized paroxysmal fast activity in sleep, with cognitive impairment."
      },
      {
       "type": "match",
       "id": "match-generalized-eeg",
       "prompt": "Match each EEG pattern to the syndrome it points to",
       "pairs": [
        [
         "3-Hz spike-and-wave with a normal background",
         "Childhood absence epilepsy"
        ],
        [
         "Photosensitive polyspike-and-wave on awakening",
         "Juvenile myoclonic epilepsy"
        ],
        [
         "Slow spike-and-wave with paroxysmal fast activity in sleep",
         "Lennox–Gastaut syndrome"
        ],
        [
         "Hypsarrhythmia",
         "Infantile epileptic spasms syndrome"
        ],
        [
         "Burst-suppression in the first weeks of life",
         "Early infantile developmental and epileptic encephalopathy"
        ],
        [
         "Near-continuous spike-and-wave in NREM sleep",
         "Spike-wave activation in sleep (DEE-SWAS or EE-SWAS)"
        ]
       ]
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-rzbb3e",
         "front": "Childhood absence epilepsy EEG",
         "back": "About 3-Hz generalized spike-and-wave with abrupt onset, normal background, provoked by hyperventilation."
        },
        {
         "id": "c-6dbqns",
         "front": "Juvenile myoclonic epilepsy EEG",
         "back": "3–6 Hz generalized polyspike-and-wave, frequently photosensitive."
        },
        {
         "id": "c-1ndbgmm",
         "front": "Lennox–Gastaut EEG",
         "back": "Slow spike-and-wave under 2.5 Hz plus paroxysmal fast activity in sleep, slow background."
        },
        {
         "id": "c-z6w0ke",
         "front": "Hypsarrhythmia",
         "back": "Chaotic, very high-amplitude slowing with multifocal spikes; infantile epileptic spasms syndrome."
        },
        {
         "id": "c-ib3x1x",
         "front": "EEG of an epileptic spasm",
         "back": "High-amplitude slow wave followed by attenuation, sometimes with fast activity."
        },
        {
         "id": "c-vwusst",
         "front": "Focal-looking discharges in a teenager with morning myoclonus",
         "back": "Still juvenile myoclonic epilepsy; asymmetry is common in generalized epilepsies."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "ILAE idiopathic generalized epilepsy syndromes (2022)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/35503716/"
      },
      {
       "title": "ILAE syndromes with onset in childhood (2022)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/35503717/"
      },
      {
       "title": "ILAE syndromes with onset in neonates and infants (2022)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/35503712/"
      }
     ],
     "words": 581,
     "bank": 10
    },
    {
     "id": "neonatal-seizures",
     "n": "4",
     "title": "Neonatal seizures",
     "tags": [
      "ilae-classification",
      "seizure-management",
      "eeg-fundamentals"
     ],
     "updated": true,
     "goals": [
      "Apply the ILAE 2021 neonatal classification",
      "know why EEG confirmation is required",
      "follow the 2023 ILAE treatment recommendations"
     ],
     "blocks": [
      {
       "type": "p",
       "updated": true,
       "label": "ILAE 2021",
       "text": "Neonatal seizures have their own classification. They are defined on EEG, because many neonatal seizures have no clinical sign and many odd neonatal movements are not seizures. Events without an EEG correlate are not classified as seizures."
      },
      {
       "type": "h3",
       "text": "Classification"
      },
      {
       "type": "p",
       "text": "Neonatal seizures are almost always focal in onset, so the classification does not ask focal versus generalized. A seizure is either **with clinical signs**, described by its predominant feature (automatisms, clonic, epileptic spasms, myoclonic, tonic, autonomic, behavioral arrest, or sequential), or **electrographic only**."
      },
      {
       "type": "h3",
       "text": "On the EEG"
      },
      {
       "type": "p",
       "text": "An electrographic seizure is a sudden, repetitive, evolving pattern lasting at least 10 seconds. Neonatal status epilepticus is seizure activity adding up to more than half of any one-hour period. After an ASM, clinical signs often stop while electrographic seizures continue (**electroclinical uncoupling**), which is why monitoring must continue after treatment. Continuous conventional EEG is the gold standard; amplitude-integrated EEG is a useful screen but misses brief, focal and low-amplitude seizures."
      },
      {
       "type": "h3",
       "text": "Causes"
      },
      {
       "type": "li",
       "text": "Hypoxic-ischemic encephalopathy is the commonest cause in term neonates, followed by stroke, hemorrhage, infection and metabolic disturbances (glucose, calcium, magnesium, sodium)."
      },
      {
       "type": "li",
       "text": "**Neonatal-onset epilepsies**: self-limited (familial) neonatal epilepsy with KCNQ2 or KCNQ3 variants, self-limited neonatal-infantile epilepsy (often SCN2A), KCNQ2 developmental and epileptic encephalopathy, and early infantile developmental and epileptic encephalopathy."
      },
      {
       "type": "li",
       "text": "**Vitamin-responsive epilepsies**: pyridoxine-dependent epilepsy (ALDH7A1) and PNPO deficiency."
      },
      {
       "type": "h3",
       "text": "Treatment (ILAE 2023)"
      },
      {
       "type": "li",
       "text": "**Phenobarbital first line**, whatever the cause. The exception is a likely channelopathy (a family history, for example), when phenytoin or carbamazepine is preferred."
      },
      {
       "type": "li",
       "text": "**Second line**: phenytoin, levetiracetam, midazolam or lidocaine. Levetiracetam is preferred with cardiac disease."
      },
      {
       "type": "li",
       "text": "**Treat electrographic-only seizures too**: a lower seizure burden may improve outcome."
      },
      {
       "type": "li",
       "text": "**Stop ASMs before discharge** after acute provoked seizures that have resolved, even if the MRI or EEG is abnormal."
      },
      {
       "type": "li",
       "text": "**Therapeutic hypothermia** reduces seizure burden in hypoxic-ischemic encephalopathy."
      },
      {
       "type": "li",
       "text": "**Try pyridoxine** when features suggest a B6-dependent epilepsy and seizures continue after second-line drugs."
      },
      {
       "type": "p",
       "text": "In the NEOLEV2 trial, phenobarbital stopped seizures more often than levetiracetam as a first drug."
      },
      {
       "type": "case",
       "id": "case-neonatal",
       "title": "A term newborn with HIE",
       "intro": [
        "A term neonate is born after placental abruption, with Apgar scores of 1 and 4 and a cord pH of 6.9. Therapeutic hypothermia is started at 3 hours of age. At 14 hours the nurse notices intermittent lip-smacking and a few seconds of rhythmic left-arm jerking."
       ],
       "steps": [
        {
         "narrative": [],
         "stem": "What is the most important next step?",
         "options": [
          "Treat on clinical grounds and skip EEG to save time",
          "Start continuous conventional EEG, and treat seizures confirmed on EEG",
          "Obtain amplitude-integrated EEG only and stop if it is normal",
          "Obtain an MRI before any treatment",
          "Observe; neonatal movements are rarely seizures"
         ],
         "answer": 1,
         "explain": "Neonatal seizures are defined on EEG. Continuous conventional EEG is the gold standard in high-risk neonates such as those with HIE on hypothermia. aEEG alone can miss brief or focal seizures."
        },
        {
         "narrative": [
          "cEEG shows a 90-second right central seizure with evolving rhythmic sharp waves, followed by two more within the hour."
         ],
         "stem": "Which ASM should be given first?",
         "options": [
          "Phenobarbital",
          "Levetiracetam",
          "Lorazepam infusion",
          "Topiramate",
          "Carbamazepine"
         ],
         "answer": 0,
         "explain": "The ILAE 2023 guideline recommends phenobarbital as first-line whatever the cause, unless a channelopathy is likely. It outperformed levetiracetam in NEOLEV2."
        },
        {
         "narrative": [
          "After phenobarbital, the arm jerking stops but the EEG still shows seizures every 20 minutes."
         ],
         "stem": "What does this represent, and what next?",
         "options": [
          "Artifact from hypothermia; no action needed",
          "Electroclinical uncoupling; give a second-line ASM",
          "Treatment success; stop the EEG",
          "Nonepileptic events; stop phenobarbital",
          "Brain death; discuss withdrawal of care"
         ],
         "answer": 1,
         "explain": "After an ASM, clinical signs often disappear while electrographic seizures continue. Electrographic-only seizures should still be treated; phenytoin, levetiracetam, midazolam or lidocaine are second-line options."
        },
        {
         "narrative": [
          "Seizures stop on day 2. MRI on day 5 shows bilateral basal ganglia and thalamic injury. The EEG no longer shows seizures."
         ],
         "stem": "What should happen to her ASMs before discharge?",
         "options": [
          "Continue phenobarbital for at least a year because the MRI is abnormal",
          "Switch to levetiracetam for six months",
          "Stop them before discharge, because the seizures were acute provoked",
          "Continue both drugs until the EEG normalizes",
          "Start vigabatrin prophylaxis against infantile spasms"
         ],
         "answer": 2,
         "explain": "The ILAE 2023 guideline recommends stopping ASMs before discharge after acute provoked neonatal seizures without evidence of neonatal-onset epilepsy, regardless of MRI or EEG findings."
        }
       ],
       "outro": [
        "Hypoxic-ischemic encephalopathy is the commonest cause of neonatal seizures at term. The pattern to remember: EEG confirms, phenobarbital first, treat what the EEG shows, and stop provoked-seizure treatment before home."
       ]
      },
      {
       "type": "mcq",
       "id": "q-1ygztfh",
       "stem": "A 3-day-old term boy with normal pregnancy and delivery has brief tonic seizures with apnea. His father had \"fits\" as a newborn that stopped by six months. Interictal EEG is normal between seizures. Which drug do the ILAE 2023 recommendations favor first?",
       "options": [
        "Phenobarbital",
        "A sodium channel blocker such as carbamazepine or phenytoin",
        "Levetiracetam",
        "Vigabatrin",
        "Pyridoxine"
       ],
       "answer": 1,
       "explain": "A family history of self-limited neonatal seizures suggests a KCNQ2/KCNQ3 channelopathy. For a likely channelopathy the guideline recommends phenytoin or carbamazepine first rather than phenobarbital."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-1as1b9y",
         "front": "Minimum duration of a neonatal electrographic seizure",
         "back": "10 seconds."
        },
        {
         "id": "c-d6g9js",
         "front": "Neonatal status epilepticus (ACNS)",
         "back": "Seizures totaling more than half of any one-hour period."
        },
        {
         "id": "c-1mmq1wq",
         "front": "Electroclinical uncoupling",
         "back": "Clinical signs stop after an ASM while electrographic seizures continue."
        },
        {
         "id": "c-1qkl70p",
         "front": "First-line ASM for neonatal seizures (ILAE 2023)",
         "back": "Phenobarbital, unless a channelopathy is likely (then phenytoin or carbamazepine)."
        },
        {
         "id": "c-19qr2h6",
         "front": "Second-line neonatal ASMs",
         "back": "Phenytoin, levetiracetam, midazolam or lidocaine; levetiracetam if cardiac disease."
        },
        {
         "id": "c-p0xqjy",
         "front": "Acute provoked neonatal seizures at discharge",
         "back": "Stop ASMs before discharge, whatever the MRI or EEG."
        },
        {
         "id": "c-nir2qs",
         "front": "Commonest cause of seizures in term neonates",
         "back": "Hypoxic-ischemic encephalopathy."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "ILAE classification of seizures in the neonate (2021)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/33522601/"
      },
      {
       "title": "ILAE treatment of neonatal seizures (2023)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/37655702/"
      },
      {
       "title": "NEOLEV2 trial (2020)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/32385134/"
      },
      {
       "title": "ACNS neonatal EEG terminology (2013)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/23545767/"
      }
     ],
     "words": 755,
     "bank": 11
    }
   ],
   "intro": [
    "What an abnormal EEG adds to the diagnosis, and what it cannot do. Interictal discharges raise or lower the probability of epilepsy; the seizure pattern, when you capture one, tells you where and what kind."
   ]
  },
  {
   "id": "icu",
   "n": "4",
   "title": "Critical care EEG",
   "short": "Critical care",
   "sections": [
    {
     "id": "acns-terminology",
     "n": "1",
     "title": "ACNS 2021 terminology",
     "tags": [
      "acns-terminology"
     ],
     "updated": true,
     "goals": [
      "Name rhythmic and periodic patterns with main terms and modifiers",
      "describe the background in standard terms",
      "apply the ACNS definitions of electrographic and electroclinical seizures and status"
     ],
     "blocks": [
      {
       "type": "p",
       "updated": true,
       "label": "ACNS 2021",
       "text": "The old labels are retired. PLEDs are now LPDs, BIPLEDs are BIPDs, GPEDs are GPDs, and triphasic waves are GPDs with triphasic morphology. \"Epileptiform\" was dropped because these patterns sit on a spectrum and naming them should not presume they are seizures."
      },
      {
       "type": "h3",
       "text": "Building a name"
      },
      {
       "type": "p",
       "text": "A rhythmic or periodic pattern gets two main terms and any modifiers."
      },
      {
       "type": "li",
       "text": "**Main term 1, where:** generalized (G), lateralized (L), bilateral independent (BI), unilateral independent (UI), multifocal (Mf)."
      },
      {
       "type": "li",
       "text": "**Main term 2, what:** periodic discharges (PDs), rhythmic delta activity (RDA), spike-and-wave or sharp-and-wave (SW)."
      },
      {
       "type": "li",
       "text": "**Plus modifiers:** +F superimposed fast activity (PDs or RDA), +R superimposed rhythmic activity (PDs only), +S superimposed sharp waves or spikes (RDA only). Plus features raise the likelihood that a pattern is ictal."
      },
      {
       "type": "li",
       "text": "**Other modifiers:** prevalence, duration, frequency, number of phases, sharpness, amplitude, polarity, stimulus-induced (SI-), evolving or fluctuating, triphasic morphology, and anterior–posterior lag."
      },
      {
       "type": "p",
       "text": "Try it in the [ACNS name builder](acns_criteria.html#name-builder-section)."
      },
      {
       "type": "h3",
       "text": "Prevalence and duration"
      },
      {
       "type": "table",
       "head": [
        "Prevalence term",
        "Share of the record",
        "Duration term",
        "Length of a run"
       ],
       "rows": [
        [
         "Continuous",
         "90% or more",
         "Very long",
         "1 hour or more"
        ],
        [
         "Abundant",
         "50–89%",
         "Long",
         "10–59 minutes"
        ],
        [
         "Frequent",
         "10–49%",
         "Intermediate",
         "1–9.9 minutes"
        ],
        [
         "Occasional",
         "1–9%",
         "Brief",
         "10–59 seconds"
        ],
        [
         "Rare",
         "Under 1%",
         "Very brief",
         "Under 10 seconds"
        ]
       ]
      },
      {
       "type": "h3",
       "text": "The background"
      },
      {
       "type": "li",
       "text": "**Continuity:** continuous; nearly continuous (under 10% attenuation or suppression); discontinuous (10–49%); burst-attenuation or burst-suppression (50–99%); attenuation or suppression (over 99%)."
      },
      {
       "type": "li",
       "text": "**Suppression** means under 10 µV; **attenuation** means 10 µV or more but under half the background voltage."
      },
      {
       "type": "figure",
       "html": "continuity-model",
       "caption": "The continuity scale as a slider: set the share of the record that is suppressed or attenuated and read its ACNS name. A synthetic 20-second trace; the shaded stretches are the low-voltage periods.",
       "n": 5
      },
      {
       "type": "li",
       "text": "**Reactivity** is a change in cerebral activity with stimulation. Muscle or eye artifact alone does not count, and neither do stimulus-induced patterns (SIRPIDs)."
      },
      {
       "type": "li",
       "text": "Also describe symmetry, the dominant frequency, the presence of sleep transients and state changes, and whether bursts are **highly epileptiform** or **identical**."
      },
      {
       "type": "h3",
       "text": "What counts as a seizure"
      },
      {
       "type": "table",
       "head": [
        "Term",
        "Definition"
       ],
       "rows": [
        [
         "Electrographic seizure",
         "Epileptiform discharges averaging more than 2.5 Hz for 10 seconds or more, or any pattern with definite evolution lasting 10 seconds or more"
        ],
        [
         "Electrographic status epilepticus",
         "Electrographic seizure for 10 continuous minutes or more, or for 20% or more of any 60-minute period"
        ],
        [
         "Electroclinical seizure",
         "Any EEG pattern with a definite time-locked clinical correlate (any duration), or EEG and clinical improvement after a parenteral ASM"
        ],
        [
         "Electroclinical status epilepticus",
         "Electroclinical seizure for 10 continuous minutes or more, or 20% or more of any hour; 5 continuous minutes if there is bilateral tonic–clonic activity"
        ],
        [
         "Possible electroclinical status",
         "A pattern on the ictal–interictal continuum for 10 minutes or 20% of an hour that improves on EEG, but not clinically, after a parenteral ASM"
        ]
       ]
      },
      {
       "type": "mcq",
       "id": "q-jfnqq4",
       "stem": "Which is the current ACNS name for a pattern formerly called \"BIPLEDs with superimposed rhythmic activity\"?",
       "options": [
        "BIRDs",
        "BIPDs+R",
        "BI-LRDA+S",
        "GPDs+R",
        "BIPLEDs are still the preferred term"
       ],
       "answer": 1,
       "explain": "Bilateral independent periodic epileptiform discharges are now bilateral independent periodic discharges (BIPDs). Superimposed rhythmic activity on PDs is the +R modifier."
      },
      {
       "type": "mcq",
       "id": "q-d76b9y",
       "stem": "A comatose patient has lateralized rhythmic delta at 1.5 Hz for three hours without evolution or plus features. By ACNS 2021 criteria, what is this?",
       "options": [
        "Electrographic status epilepticus",
        "An electrographic seizure",
        "On the ictal–interictal continuum",
        "LRDA that meets neither seizure nor IIC criteria as described",
        "BIRDs"
       ],
       "answer": 3,
       "explain": "Rhythmic delta does not count toward the 2.5 Hz rule and has no evolution. Lateralized RDA reaches the continuum only above 1 Hz with a plus modifier or fluctuation. It still carries an increased seizure risk and warrants continued monitoring."
      },
      {
       "type": "mcq",
       "id": "q-b48zfv",
       "stem": "A patient has repetitive left face twitching, each twitch time-locked to a right central periodic discharge at 0.8 Hz, lasting 15 minutes. What is the diagnosis by ACNS criteria?",
       "options": [
        "LPDs, interictal",
        "Ictal–interictal continuum only",
        "Electroclinical status epilepticus",
        "Electrographic seizure without clinical correlate",
        "Artifact"
       ],
       "answer": 2,
       "explain": "Any EEG pattern with a definite time-locked clinical correlate is an electroclinical seizure regardless of frequency. Lasting 10 continuous minutes or more, it is electroclinical status epilepticus (here, focal motor status)."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-1puuw3n",
         "front": "Main term 1 options",
         "back": "Generalized, lateralized, bilateral independent, unilateral independent, multifocal."
        },
        {
         "id": "c-b8r4jm",
         "front": "Main term 2 options",
         "back": "Periodic discharges, rhythmic delta activity, spike-and-wave or sharp-and-wave."
        },
        {
         "id": "c-e9o25t",
         "front": "+R and +S",
         "back": "+R superimposed rhythmic activity applies to PDs; +S superimposed sharp waves applies to RDA."
        },
        {
         "id": "c-jk8gqz",
         "front": "Electrographic seizure (ACNS 2021)",
         "back": "Discharges averaging over 2.5 Hz for 10 s or more, or definite evolution for 10 s or more."
        },
        {
         "id": "c-m3c7nu",
         "front": "Electrographic status epilepticus",
         "back": "10 continuous minutes, or 20% of any hour."
        },
        {
         "id": "c-1wyw37r",
         "front": "Electroclinical status with convulsions",
         "back": "5 continuous minutes of bilateral tonic–clonic activity."
        },
        {
         "id": "c-47tfhq",
         "front": "Suppression versus attenuation",
         "back": "Suppression under 10 µV; attenuation 10 µV or more but under half of background."
        },
        {
         "id": "c-1rpdr6k",
         "front": "Do SIRPIDs count as reactivity?",
         "back": "No."
        }
       ]
      },
      {
       "type": "note",
       "label": "Try it",
       "text": "Name patterns on synthetic 16-channel strips in [Read the Strip](acns_criteria.html#strip-drill-section), then turn your findings into ACNS wording in [Write the Report](acns_criteria.html#report-builder-section)."
      }
     ],
     "sources": [
      {
       "title": "ACNS standardized critical care EEG terminology (2021)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/33475321/"
      },
      {
       "title": "ACNS 2021 terminology document",
       "url": "https://www.acns.org/UserFiles/file/ACNSStandardizedCriticalCareEEGTerminology_rev2021.pdf"
      }
     ],
     "words": 782,
     "bank": 11
    },
    {
     "id": "iic-and-birds",
     "n": "2",
     "title": "The ictal–interictal continuum and seizure risk",
     "tags": [
      "acns-terminology",
      "seizure-management"
     ],
     "updated": false,
     "goals": [
      "Apply the IIC criteria",
      "recognize BIRDs",
      "estimate seizure risk with 2HELPS2B",
      "decide when an antiseizure trial is reasonable"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "Which patterns carry seizure risk"
      },
      {
       "type": "p",
       "text": "In a large multicenter cohort, lateralized periodic discharges, lateralized rhythmic delta and bilateral independent periodic discharges were all associated with seizures. Generalized periodic discharges were associated with seizures at higher frequencies and with plus features. Generalized rhythmic delta, including frontally predominant GRDA (the old FIRDA), was not associated with seizures. For every pattern, a higher frequency and plus features increased the risk."
      },
      {
       "type": "h3",
       "text": "The ictal–interictal continuum (IIC)"
      },
      {
       "type": "p",
       "text": "A pattern is on the IIC if it does not meet seizure criteria and, over at least 10 seconds, it is:"
      },
      {
       "type": "li",
       "text": "PDs or spike-and-wave averaging more than 1 and up to 2.5 Hz; or"
      },
      {
       "type": "li",
       "text": "PDs or spike-and-wave at 0.5–1 Hz with a plus modifier or fluctuation; or"
      },
      {
       "type": "li",
       "text": "lateralized rhythmic delta above 1 Hz with a plus modifier or fluctuation."
      },
      {
       "type": "figure",
       "html": "iic-map",
       "caption": "The same criteria by average frequency over 10 seconds, next to the seizure threshold. Point at or tab to a band for its criterion.",
       "n": 6
      },
      {
       "type": "p",
       "text": "GRDA never qualifies. The IIC is a description, not a diagnosis. Whether to treat depends on the clinical picture, imaging, and sometimes a monitored trial."
      },
      {
       "type": "h3",
       "text": "Brief potentially ictal rhythmic discharges (BIRDs)"
      },
      {
       "type": "p",
       "text": "Rhythmic activity faster than 4 Hz, at least six waves, lasting 0.5 to under 10 seconds, not a normal variant and not part of burst-suppression. **Definite BIRDs** evolve, or match the patient's seizures or interictal discharges; **possible BIRDs** are only sharply contoured. BIRDs are strongly associated with seizures."
      },
      {
       "type": "h3",
       "text": "A treatment trial"
      },
      {
       "type": "p",
       "text": "When a pattern on the IIC might be causing the patient's impairment, a trial of a parenteral ASM (a small benzodiazepine dose, or a nonsedating drug such as levetiracetam, valproate or lacosamide) with EEG and bedside examination can help. Clear clinical and EEG improvement makes it an electroclinical seizure. EEG improvement alone is ambiguous, especially after a benzodiazepine that also sedates."
      },
      {
       "type": "h3",
       "text": "2HELPS2B"
      },
      {
       "type": "p",
       "text": "Six items from the first hours of EEG and the history estimate the risk of seizures during monitoring. In a later validation, a score of 0 after the first hour identified patients at low enough risk that a shorter recording may suffice."
      },
      {
       "type": "calc",
       "name": "twohelps2b"
      },
      {
       "type": "case",
       "id": "case-iic",
       "title": "Periodic discharges after a stroke",
       "intro": [
        "A 71-year-old is admitted with a left MCA infarct and is more confused on day 2 than expected. Continuous EEG shows left hemispheric periodic discharges at 1.5 Hz, sharply contoured, with intermittent superimposed rhythmic activity. No evolution is seen."
       ],
       "steps": [
        {
         "narrative": [],
         "stem": "How are these discharges named, and where do they sit?",
         "options": [
          "GPDs, interictal",
          "LPDs+R at 1.5 Hz, on the ictal–interictal continuum",
          "Electrographic status epilepticus",
          "LRDA+S, not on the continuum",
          "BIRDs"
         ],
         "answer": 1,
         "explain": "They are lateralized periodic discharges with superimposed rhythmic activity (+R). PDs above 1 and up to 2.5 Hz are on the IIC even without plus features; they do not meet seizure criteria without evolution or a rate above 2.5 Hz."
        },
        {
         "narrative": [
          "Her 2HELPS2B items: LPDs present, plus features present, frequency not above 2 Hz, no sporadic discharges, no prior seizure, no BIRDs."
         ],
         "stem": "What is her score and estimated seizure risk?",
         "options": [
          "1, about 12%",
          "2, about 27%",
          "3, about 50%",
          "4, about 73%",
          "0, under 5%"
         ],
         "answer": 1,
         "explain": "LPDs (1) plus features (1) = 2, an estimated seizure risk of about 27% in the derivation cohort. Continue monitoring for at least 24 hours."
        },
        {
         "narrative": [],
         "stem": "The team wonders whether her confusion is ictal. What is the most reasonable test?",
         "options": [
          "Load phenobarbital to burst-suppression",
          "Ignore the pattern; LPDs are always interictal",
          "A monitored trial of a nonsedating parenteral ASM, watching both the EEG and her examination",
          "Repeat MRI",
          "Lumbar puncture"
         ],
         "answer": 2,
         "explain": "Clear EEG and clinical improvement after a parenteral ASM would make this an electroclinical seizure and justify treatment. A nonsedating drug avoids confusing sedation with improvement."
        }
       ],
       "outro": [
        "LPDs after a cortical stroke are common. Name them precisely, estimate risk, monitor long enough, and treat on the combination of EEG, examination and trial response rather than the pattern alone."
       ]
      },
      {
       "type": "mcq",
       "id": "q-nnam9c",
       "stem": "Which pattern meets the ACNS 2021 criteria for the ictal–interictal continuum?",
       "options": [
        "GRDA at 2 Hz with superimposed fast activity",
        "GPDs at 2 Hz without plus features",
        "LRDA at 1 Hz with superimposed sharp waves",
        "LPDs at 0.7 Hz without plus features or fluctuation",
        "LPDs at 3 Hz for 12 seconds"
       ],
       "answer": 1,
       "explain": "PDs averaging more than 1 and up to 2.5 Hz qualify on frequency alone. GRDA never qualifies; LRDA needs to be above 1 Hz; PDs at 0.5–1 Hz need a plus modifier or fluctuation; LPDs above 2.5 Hz for 10 seconds or more are an electrographic seizure."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-iu7ouw",
         "front": "IIC criterion 1",
         "back": "PDs or spike-and-wave averaging more than 1 and up to 2.5 Hz over 10 s."
        },
        {
         "id": "c-jo6hxt",
         "front": "IIC criterion 2",
         "back": "PDs or spike-and-wave at 0.5–1 Hz with a plus modifier or fluctuation."
        },
        {
         "id": "c-je6w8u",
         "front": "IIC criterion 3",
         "back": "Lateralized RDA above 1 Hz with a plus modifier or fluctuation."
        },
        {
         "id": "c-qd6itz",
         "front": "Does GRDA qualify for the IIC?",
         "back": "Never."
        },
        {
         "id": "c-1yihzir",
         "front": "BIRDs",
         "back": "Rhythmic activity over 4 Hz, at least six waves, 0.5 to under 10 s, not a variant or part of burst-suppression."
        },
        {
         "id": "c-s37kwl",
         "front": "Heaviest 2HELPS2B item",
         "back": "BIRDs (2 points)."
        },
        {
         "id": "c-199jqe6",
         "front": "2HELPS2B risk bands",
         "back": "0 about 5%; 1 12%; 2 27%; 3 50%; 4 73%; 5 88%; 6–7 over 95%."
        },
        {
         "id": "c-1ywf6eb",
         "front": "Which rhythmic ICU pattern is not linked to seizures?",
         "back": "GRDA, including frontally predominant GRDA."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "ACNS critical care EEG terminology (2021)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/33475321/"
      },
      {
       "title": "2HELPS2B derivation (Struck 2017)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/29052706/"
      },
      {
       "title": "Periodic and rhythmic patterns and seizure risk (Rodriguez Ruiz 2017)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/27992625/"
      }
     ],
     "words": 771,
     "bank": 10
    },
    {
     "id": "coma-prognosis",
     "n": "3",
     "title": "Coma, cardiac arrest and prognosis",
     "tags": [
      "acns-terminology",
      "seizure-management"
     ],
     "updated": false,
     "goals": [
      "Grade encephalopathy on EEG",
      "interpret EEG after cardiac arrest within a multimodal framework",
      "avoid the confounders that make early prognosis wrong"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "Encephalopathy on EEG"
      },
      {
       "type": "p",
       "text": "As encephalopathy deepens, the EEG usually slows from theta to delta, loses reactivity and state changes, becomes discontinuous, and finally suppresses. GPDs with triphasic morphology and generalized rhythmic delta are common along the way. Sedatives, hypothermia and metabolic derangement do the same, which is why the background must be read with the drug chart and temperature in hand."
      },
      {
       "type": "h3",
       "text": "After cardiac arrest"
      },
      {
       "type": "p",
       "text": "EEG is one part of a multimodal assessment. European resuscitation and intensive care guidelines advise prognosticating no earlier than 72 hours after return of circulation, once sedation, hypothermia and metabolic confounders have been dealt with. Poor outcome is considered likely when at least two of these are present:"
      },
      {
       "type": "li",
       "text": "no pupillary and corneal reflexes at 72 hours or later"
      },
      {
       "type": "li",
       "text": "bilaterally absent N20 somatosensory evoked potentials"
      },
      {
       "type": "li",
       "text": "a highly malignant EEG after 24 hours"
      },
      {
       "type": "li",
       "text": "neuron-specific enolase above 60 µg/L at 48 and/or 72 hours"
      },
      {
       "type": "li",
       "text": "status myoclonus within 72 hours"
      },
      {
       "type": "li",
       "text": "diffuse, extensive anoxic injury on CT or MRI"
      },
      {
       "type": "p",
       "text": "**Highly malignant** EEG patterns are a suppressed background with or without periodic discharges, and burst-suppression. **Identical bursts** after arrest are especially ominous. A continuous, reactive background is a favorable sign."
      },
      {
       "type": "h3",
       "text": "Classic coma patterns"
      },
      {
       "type": "table",
       "head": [
        "Pattern",
        "Meaning"
       ],
       "rows": [
        [
         "Burst-suppression",
         "Deep anesthesia, hypothermia, or severe anoxic injury; identical, highly epileptiform bursts after arrest are malignant"
        ],
        [
         "Alpha coma",
         "Diffuse, nonreactive alpha-frequency activity; after anoxia usually a poor sign"
        ],
        [
         "Spindle coma",
         "Sleep-like spindles in coma; often trauma or brainstem lesions, with a better outlook than alpha coma"
        ],
        [
         "Electrocerebral inactivity",
         "No cerebral activity over 2 µV with the technical protocol met"
        ]
       ]
      },
      {
       "type": "case",
       "id": "case-cardiac-arrest",
       "title": "Unresponsive after cardiac arrest",
       "intro": [
        "A 58-year-old is resuscitated after 25 minutes of out-of-hospital arrest with a non-shockable rhythm. He is managed with targeted temperature control and propofol."
       ],
       "steps": [
        {
         "narrative": [
          "At 12 hours the EEG shows burst-suppression with long flat periods."
         ],
         "stem": "What is the correct interpretation at this point?",
         "options": [
          "Prognosis is poor; discuss withdrawal of care today",
          "Burst-suppression at 12 hours on propofol and temperature control is not interpretable in isolation; reassess after confounders clear",
          "He is brain dead",
          "Start a pentobarbital infusion",
          "The EEG is normal for temperature control"
         ],
         "answer": 1,
         "explain": "Sedation and temperature control can themselves cause burst-suppression. Guidelines place EEG within multimodal assessment no earlier than 72 hours after return of circulation, and a highly malignant EEG counts only after 24 hours."
        },
        {
         "narrative": [
          "At 80 hours, off sedation and normothermic, he has absent pupillary and corneal reflexes, bilaterally absent N20 responses, and an EEG with a suppressed background and no reactivity."
         ],
         "stem": "How should these findings be weighed?",
         "options": [
          "Each alone is enough to predict poor outcome",
          "Several concordant predictors make a poor neurological outcome very likely",
          "They cannot be interpreted until day 14",
          "The EEG outweighs the other findings",
          "Obtain a Wada test"
         ],
         "answer": 1,
         "explain": "Poor outcome is considered likely when at least two robust predictors agree after 72 hours with confounders excluded. Here the examination, SSEPs and EEG agree."
        }
       ],
       "outro": [
        "Multimodal, delayed and confounder-free: that is the framework. A single early EEG under sedation should never drive a prognosis on its own."
       ]
      },
      {
       "type": "mcq",
       "id": "q-1gcaoer",
       "stem": "Which EEG finding after cardiac arrest is a favorable prognostic sign?",
       "options": [
        "Burst-suppression with identical bursts at 36 hours",
        "Suppressed background with periodic discharges at 48 hours",
        "A continuous background that reacts to stimulation",
        "Alpha coma that does not react to stimulation",
        "Electrocerebral inactivity at 72 hours"
       ],
       "answer": 2,
       "explain": "A continuous, reactive background early after arrest is associated with good recovery. The other patterns are malignant or highly malignant."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-1o3csw6",
         "front": "Earliest multimodal prognosis after cardiac arrest",
         "back": "72 hours after return of circulation, with confounders excluded."
        },
        {
         "id": "c-1a13gtz",
         "front": "Highly malignant EEG after arrest",
         "back": "Suppressed background with or without periodic discharges, or burst-suppression."
        },
        {
         "id": "c-158qmne",
         "front": "Identical bursts after arrest",
         "back": "A particularly malignant pattern."
        },
        {
         "id": "c-v2rcff",
         "front": "Favorable EEG after arrest",
         "back": "Continuous, reactive background."
        },
        {
         "id": "c-1bhxrtd",
         "front": "NSE threshold in prognosis guidance",
         "back": "Above 60 µg/L at 48 and/or 72 hours."
        },
        {
         "id": "c-1b1aj3b",
         "front": "Alpha coma after anoxia",
         "back": "Diffuse nonreactive alpha activity; usually poor prognosis."
        },
        {
         "id": "c-gr2nbc",
         "front": "Spindle coma",
         "back": "Often trauma or brainstem lesions; better outlook than alpha coma."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "ERC–ESICM post-resuscitation care guidelines (2021)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/33765189/"
      },
      {
       "title": "Standardized EEG after cardiac arrest (Westhall 2016)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/26865516/"
      },
      {
       "title": "ACNS critical care EEG terminology (2021)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/33475321/"
      }
     ],
     "words": 558,
     "bank": 10
    },
    {
     "id": "periodic-by-cause",
     "n": "4",
     "title": "Periodic patterns by cause",
     "tags": [
      "acns-terminology",
      "ilae-classification"
     ],
     "updated": false,
     "goals": [
      "Link characteristic periodic patterns to their causes",
      "recognize drug-induced encephalopathies that mimic status",
      "know when a periodic pattern should change the work-up"
     ],
     "blocks": [
      {
       "type": "table",
       "head": [
        "Pattern",
        "Typical period",
        "Think of"
       ],
       "rows": [
        [
         "Temporal LPDs with fever and confusion",
         "About 1–2 s",
         "Herpes simplex encephalitis; start acyclovir while waiting for PCR"
        ],
        [
         "Generalized periodic sharp waves, often with startle myoclonus",
         "About 0.5–2 s",
         "Sporadic Creutzfeldt–Jakob disease; they appear as the illness progresses and fade late and in sleep"
        ],
        [
         "Very high-amplitude stereotyped complexes with time-locked myoclonus",
         "Every 4–15 s",
         "Subacute sclerosing panencephalitis"
        ],
        [
         "GPDs with triphasic morphology and anterior–posterior lag",
         "About 1–2 Hz",
         "Hepatic, renal or septic encephalopathy; lithium, baclofen, cefepime (especially in renal failure)"
        ],
        [
         "Extreme delta brush",
         "Continuous rhythmic delta with superimposed beta",
         "Anti-NMDA receptor encephalitis"
        ],
        [
         "LPDs after an acute lesion",
         "Variable",
         "Stroke, tumor, abscess, trauma"
        ]
       ]
      },
      {
       "type": "h3",
       "text": "Triphasic morphology: metabolic or ictal?"
      },
      {
       "type": "p",
       "text": "GPDs with triphasic morphology used to be read as metabolic by default. Features that favor a toxic-metabolic cause are frontal predominance, an anterior-to-posterior lag, a slower rate, and reactivity to stimulation. None is decisive. Clinical and EEG improvement after a benzodiazepine suggests nonconvulsive status, but benzodiazepines also dampen metabolic patterns and sedate the patient, so EEG improvement alone proves little. Cefepime neurotoxicity in renal failure can produce frank nonconvulsive status."
      },
      {
       "type": "h3",
       "text": "Drug effects to recognize"
      },
      {
       "type": "li",
       "text": "Benzodiazepines and barbiturates: excess beta."
      },
      {
       "type": "li",
       "text": "Anesthetics (propofol, barbiturates) and hypothermia: slowing, then burst-suppression."
      },
      {
       "type": "li",
       "text": "Clozapine, lithium, and high-dose baclofen can produce generalized discharges and myoclonus."
      },
      {
       "type": "mcq",
       "id": "q-1cq7dgi",
       "stem": "A 66-year-old with end-stage renal disease is started on cefepime for pneumonia. Two days later she is confused with multifocal myoclonus. EEG shows continuous generalized periodic discharges with triphasic morphology at 2 Hz. What is the most appropriate step?",
       "options": [
        "Attribute it to uremia and observe",
        "Recognize possible cefepime neurotoxicity: stop or change the antibiotic, consider dialysis, and consider a monitored benzodiazepine or ASM trial",
        "Start a propofol infusion to burst-suppression",
        "Diagnose Creutzfeldt–Jakob disease",
        "Order an MRI and wait for the result before any change"
       ],
       "answer": 1,
       "explain": "Cefepime accumulates in renal failure and can cause encephalopathy with triphasic GPDs and nonconvulsive status. Removing the drug (and dialysis) is the key step; a monitored trial helps decide whether seizures are present."
      },
      {
       "type": "mcq",
       "id": "q-rcwl2",
       "stem": "A 62-year-old has three months of rapidly progressive dementia, ataxia and startle-sensitive myoclonus. EEG shows generalized periodic sharp waves about once a second. What is the most likely diagnosis?",
       "options": [
        "Subacute sclerosing panencephalitis",
        "Sporadic Creutzfeldt–Jakob disease",
        "Herpes simplex encephalitis",
        "Hepatic encephalopathy",
        "Anti-NMDA receptor encephalitis"
       ],
       "answer": 1,
       "explain": "Roughly 1-Hz generalized periodic sharp waves with rapidly progressive dementia and myoclonus is the classic picture of sporadic CJD. SSPE complexes recur every 4–15 seconds."
      },
      {
       "type": "match",
       "id": "match-periodic",
       "prompt": "Match each pattern to its most likely cause",
       "pairs": [
        [
         "Temporal LPDs with fever and aphasia",
         "Herpes simplex encephalitis"
        ],
        [
         "Generalized periodic sharp waves about 1 Hz with rapid dementia",
         "Sporadic Creutzfeldt–Jakob disease"
        ],
        [
         "Complexes every 4–15 seconds with time-locked jerks",
         "Subacute sclerosing panencephalitis"
        ],
        [
         "Extreme delta brush",
         "Anti-NMDA receptor encephalitis"
        ],
        [
         "Triphasic GPDs after an antibiotic in renal failure",
         "Cefepime neurotoxicity"
        ]
       ]
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-1r4soy6",
         "front": "HSV encephalitis EEG",
         "back": "Temporal LPDs, often about 1–2 s apart."
        },
        {
         "id": "c-dzcjeu",
         "front": "Sporadic CJD EEG",
         "back": "Generalized periodic sharp waves about 0.5–2 s apart, appearing as the disease progresses."
        },
        {
         "id": "c-9rrkgn",
         "front": "SSPE EEG",
         "back": "High-amplitude stereotyped complexes every 4–15 s with time-locked myoclonus."
        },
        {
         "id": "c-1b6p9sx",
         "front": "Extreme delta brush",
         "back": "Anti-NMDA receptor encephalitis."
        },
        {
         "id": "c-1e9fdqg",
         "front": "Features favoring a metabolic triphasic pattern",
         "back": "Frontal predominance, anterior–posterior lag, slower rate, reactivity."
        },
        {
         "id": "c-lzdd37",
         "front": "Cefepime neurotoxicity",
         "back": "Encephalopathy, myoclonus, triphasic GPDs or NCSE, mostly in renal failure."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "ACNS critical care EEG terminology (2021)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/33475321/"
      },
      {
       "title": "Periodic and rhythmic patterns and seizure risk (Rodriguez Ruiz 2017)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/27992625/"
      },
      {
       "title": "Autoimmune-associated seizures and epilepsy, ILAE (2020)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/32544279/"
      }
     ],
     "words": 518,
     "bank": 7
    },
    {
     "id": "qeeg-trends",
     "n": "5",
     "title": "Quantitative EEG trends",
     "tags": [
      "acns-terminology",
      "seizure-management"
     ],
     "updated": true,
     "goals": [
      "Explain what the common qEEG trends compute",
      "use trends to find events and confirm them on the raw EEG",
      "recognize the alpha/delta ratio warning after subarachnoid hemorrhage"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "What trends are for"
      },
      {
       "type": "p",
       "text": "Quantitative EEG (qEEG) compresses hours of recording into displays of power, rhythmicity, amplitude and symmetry over time. Trends show where to look; they do not replace reading the raw EEG. Spectrogram-guided review cut the time to review 24 hours of EEG from 38 to 8 minutes, with 87% sensitivity for seizures. But when experts read trends without the raw EEG, they missed about a third of seizures and raised false alarms at about one an hour. An automated seizure detector found only about a quarter of seizures."
      },
      {
       "type": "h3",
       "text": "The common trends"
      },
      {
       "type": "li",
       "text": "**Power spectrogram (CDSA)**: time across, frequency up, power as color. Seizures often appear as a flame, an abrupt rise in power with smooth edges."
      },
      {
       "type": "li",
       "text": "**Rhythmicity spectrogram**: power belonging to rhythmic activity only; seizures show a band that drifts in frequency."
      },
      {
       "type": "li",
       "text": "**Amplitude-integrated EEG**: upper and lower amplitude margins on a semi-logarithmic scale. The lower margin rises in seizures and falls toward zero in burst suppression."
      },
      {
       "type": "li",
       "text": "**Suppression percentage**: the share of each epoch below a low voltage threshold, high in burst suppression."
      },
      {
       "type": "li",
       "text": "**Asymmetry spectrogram**: the difference between the hemispheres at each frequency."
      },
      {
       "type": "li",
       "text": "**Alpha/delta ratio (ADR) and relative alpha variability**: trended over days after subarachnoid hemorrhage."
      },
      {
       "type": "h3",
       "text": "Pitfalls"
      },
      {
       "type": "p",
       "text": "Slow, low-amplitude and very focal seizures, and seizures on a background of periodic discharges, are the ones trends miss. Chewing, patting and ventilator artifact add power and rhythmicity. A single bad electrode makes a false asymmetry."
      },
      {
       "type": "p",
       "updated": true,
       "label": "ADR after subarachnoid hemorrhage",
       "text": "In poor-grade subarachnoid hemorrhage, a post-stimulation ADR that falls by more than 10% from baseline for six consecutive readings, or by more than 50% in any single reading, was associated with delayed cerebral ischemia. In a prospective study, prespecified EEG alarms (falling alpha variability, falling ADR, worsening focal slowing, new epileptiform abnormalities) preceded DCI by a median of about two days."
      },
      {
       "type": "note",
       "label": "Try it",
       "text": "Read six ICU panels, and check the raw EEG under each finding, in the [Persyst trend lab](natus_instructions.html#pq-lab-section)."
      },
      {
       "type": "mcq",
       "id": "q-ni4vk2",
       "stem": "A seizure detector alarms twice an hour in a ventilated patient. The spectrogram shows brief vertical bands of power, mostly above 15 Hz, on both sides at once, with abrupt onset and offset. What is the next step?",
       "options": [
        "Load an antiseizure medication",
        "Review the raw EEG at each alarm; this pattern suggests muscle or movement artifact",
        "Increase the detector sensitivity",
        "Order an MRI",
        "Ignore all further alarms"
       ],
       "answer": 1,
       "explain": "Seizures build and evolve; artifact starts and stops abruptly and fills the fast frequencies on both sides. Every alarm needs the raw EEG before anyone acts on it."
      },
      {
       "type": "mcq",
       "id": "q-jmj2yn",
       "stem": "On day 5 after a poor-grade subarachnoid hemorrhage, the right-sided post-stimulation alpha/delta ratio has fallen by 55% from baseline over 12 hours, and the left is unchanged. What does this suggest?",
       "options": [
        "Right hemisphere seizures",
        "Normal day-to-night variation",
        "Possible delayed cerebral ischemia in the right hemisphere",
        "A disconnected electrode on the left",
        "Deepening sedation"
       ],
       "answer": 2,
       "explain": "A regional fall of more than 50% in the post-stimulation ADR is associated with delayed cerebral ischemia. It should prompt urgent clinical review and vascular assessment. Sedation would lower the ADR on both sides."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-138c6ta",
         "front": "Solid flame on a spectrogram",
         "back": "An abrupt rise in power with smooth edges; the typical seizure signature."
        },
        {
         "id": "c-1bit9ew",
         "front": "Stripes on a spectrogram",
         "back": "Alternating power and suppression: burst suppression."
        },
        {
         "id": "c-1li6xgk",
         "front": "Broadband monotonous spectrogram",
         "back": "Sustained high power across a wide band: ongoing status or continuous periodic discharges."
        },
        {
         "id": "c-i4h9c9",
         "front": "Clue that a spectrogram event is artifact",
         "back": "Abrupt onset and offset, fast frequencies, both sides at once, no evolution."
        },
        {
         "id": "c-1tudwif",
         "front": "aEEG in burst suppression",
         "back": "The lower margin falls toward zero."
        },
        {
         "id": "c-1paqbyt",
         "front": "ADR thresholds for DCI",
         "back": "A fall of more than 10% for six consecutive readings, or more than 50% in any single reading."
        },
        {
         "id": "c-11zwtaw",
         "front": "Sensitivity of an automated seizure detector in the ICU",
         "back": "About 26% in one multi-reader study; always read the raw EEG."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "Spectrogram screening of adult EEGs (Moura 2014)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/24857926/"
      },
      {
       "title": "Sensitivity of qEEG for seizure identification in the ICU (Haider 2016)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/27466474/"
      },
      {
       "title": "qEEG panels read by non-neurophysiologists (Swisher 2015)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/26241242/"
      },
      {
       "title": "ADR and delayed cerebral ischemia (Claassen 2004)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/15546778/"
      },
      {
       "title": "cEEG predicts delayed cerebral ischemia (Rosenthal 2018)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/29659050/"
      },
      {
       "title": "qEEG in the ICU with Persyst (Veciana de las Heras 2024)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/39335433/"
      }
     ],
     "words": 614,
     "bank": 6
    }
   ],
   "intro": [
    "Continuous EEG in the ICU finds seizures that nobody can see, and it produces patterns that are neither clearly seizures nor clearly safe. This part covers the ACNS vocabulary, the criteria that decide what to treat, and what the EEG says about prognosis."
   ]
  },
  {
   "id": "diagnosis",
   "n": "5",
   "title": "Diagnosis and classification",
   "short": "Diagnosis",
   "sections": [
    {
     "id": "definitions",
     "n": "1",
     "title": "Definitions that change management",
     "tags": [
      "ilae-classification",
      "seizure-management"
     ],
     "updated": false,
     "goals": [
      "Apply the ILAE 2014 practical definition of epilepsy",
      "separate acute symptomatic from unprovoked seizures",
      "define resolved and drug-resistant epilepsy"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "Epilepsy (ILAE 2014)"
      },
      {
       "type": "p",
       "text": "Epilepsy is present when any of these apply:"
      },
      {
       "type": "li",
       "text": "at least two unprovoked (or reflex) seizures occurring more than 24 hours apart"
      },
      {
       "type": "li",
       "text": "one unprovoked (or reflex) seizure with a probability of further seizures similar to the recurrence risk after two, at least 60% over the next 10 years"
      },
      {
       "type": "li",
       "text": "a diagnosis of an epilepsy syndrome"
      },
      {
       "type": "p",
       "text": "The middle criterion is where judgment comes in. A remote structural cause (an old stroke or injury) together with epileptiform discharges usually puts the risk high enough."
      },
      {
       "type": "h3",
       "text": "Acute symptomatic (provoked) seizures"
      },
      {
       "type": "p",
       "text": "A seizure in close time relation to an acute brain insult is **acute symptomatic**, not epilepsy, however many occur. Working time windows:"
      },
      {
       "type": "li",
       "text": "within a week of stroke, traumatic brain injury, anoxia or intracranial surgery"
      },
      {
       "type": "li",
       "text": "during the active phase of CNS infection or autoimmune inflammation"
      },
      {
       "type": "li",
       "text": "within 24 hours of a severe metabolic derangement, for example glucose under 36 mg/dL (2.0 mmol/L), sodium under 115 mmol/L, calcium under 5 mg/dL, or magnesium under 0.8 mg/dL"
      },
      {
       "type": "li",
       "text": "alcohol withdrawal, typically 7–48 hours after the last drink, and drug intoxication or withdrawal"
      },
      {
       "type": "p",
       "text": "Seizures after the acute period (a late seizure a year after a stroke, for example) are **unprovoked**, and a single one carries a high recurrence risk."
      },
      {
       "type": "h3",
       "text": "Resolved"
      },
      {
       "type": "p",
       "text": "Epilepsy is considered resolved when someone with an age-dependent syndrome is past the applicable age, or has been seizure-free for 10 years with the last 5 off ASMs. \"Resolved\" was chosen over \"cured\" because the underlying tendency may persist."
      },
      {
       "type": "h3",
       "text": "Drug-resistant epilepsy (ILAE 2010)"
      },
      {
       "type": "p",
       "text": "Failure of adequate trials of two tolerated, appropriately chosen and appropriately used ASM schedules, as monotherapy or in combination, to achieve sustained seizure freedom. Sustained means 12 months, or three times the longest pre-treatment interval between seizures, whichever is longer. A drug stopped early for side effects, used at a low dose, or not taken does not count as a failed trial."
      },
      {
       "type": "calc",
       "name": "definition-check"
      },
      {
       "type": "mcq",
       "id": "q-92ulmp",
       "stem": "A 58-year-old had a left MCA stroke two years ago. She now has a first unprovoked focal seizure with impaired consciousness, and her EEG shows left temporal sharp waves. Which statement best reflects the ILAE 2014 definition?",
       "options": [
        "She cannot have epilepsy until a second seizure occurs",
        "She can be diagnosed with epilepsy after one seizure, because her recurrence risk is at least 60%",
        "This is an acute symptomatic seizure",
        "Epilepsy requires an MRI lesion that has grown",
        "The diagnosis depends on whether she is treated"
       ],
       "answer": 1,
       "explain": "One unprovoked seizure with a recurrence risk of at least 60% over 10 years meets the practical definition. A remote stroke plus epileptiform discharges is the typical example. Two years after the stroke, the seizure is not acute symptomatic."
      },
      {
       "type": "mcq",
       "id": "q-1rjb2mt",
       "stem": "A 45-year-old has had three generalized convulsions, each within 36 hours of stopping heavy drinking. Between these episodes he is well. What is the correct classification?",
       "options": [
        "Epilepsy, because he has had more than two seizures",
        "Acute symptomatic seizures related to alcohol withdrawal",
        "Juvenile myoclonic epilepsy",
        "Drug-resistant epilepsy",
        "Reflex epilepsy"
       ],
       "answer": 1,
       "explain": "Seizures within the withdrawal window are provoked. Their number does not make them epilepsy; management is withdrawal treatment and abstinence support, not long-term ASMs."
      },
      {
       "type": "mcq",
       "id": "q-v9cmld",
       "stem": "Which patient meets the ILAE definition of drug-resistant epilepsy?",
       "options": [
        "Failed levetiracetam at 250 mg twice daily and lamotrigine stopped after a rash on day 10",
        "Seizures continue on carbamazepine; he admits taking it only occasionally",
        "Seizures continue after adequate, tolerated trials of lamotrigine and then levetiracetam at therapeutic doses",
        "Seizure-free for 18 months on the second drug",
        "Absence seizures continue on carbamazepine and phenytoin"
       ],
       "answer": 2,
       "explain": "Drug resistance requires two appropriate, tolerated, adequately used trials. Low doses, early withdrawal for side effects and non-adherence do not count, and carbamazepine and phenytoin are not appropriate for absence seizures."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-86047f",
         "front": "ILAE 2014: epilepsy after one seizure",
         "back": "One unprovoked seizure with a recurrence risk of at least 60% over 10 years."
        },
        {
         "id": "c-b0c0ra",
         "front": "Resolved epilepsy",
         "back": "Past the age of an age-dependent syndrome, or seizure-free 10 years with the last 5 off ASMs."
        },
        {
         "id": "c-3gs9ji",
         "front": "Drug-resistant epilepsy",
         "back": "Failure of two tolerated, appropriate, adequately used ASM trials to give sustained seizure freedom."
        },
        {
         "id": "c-q3ihry",
         "front": "Sustained seizure freedom",
         "back": "12 months, or three times the longest pre-treatment interseizure interval, whichever is longer."
        },
        {
         "id": "c-18iqxst",
         "front": "Acute symptomatic window after stroke or head injury",
         "back": "Within one week."
        },
        {
         "id": "c-1jjwtfd",
         "front": "Alcohol withdrawal seizure window",
         "back": "Typically 7–48 hours after the last drink."
        },
        {
         "id": "c-13k1bwu",
         "front": "Are reflex seizures provoked?",
         "back": "No. For the definition of epilepsy they count as unprovoked."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "ILAE practical clinical definition of epilepsy (2014)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/24730690/"
      },
      {
       "title": "ILAE definition of drug-resistant epilepsy (2010)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/19889013/"
      },
      {
       "title": "ILAE definition of acute symptomatic seizures (2010)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/19732133/"
      }
     ],
     "words": 705,
     "bank": 5
    },
    {
     "id": "seizure-classification",
     "n": "2",
     "title": "Classifying the seizure (ILAE 2025)",
     "tags": [
      "ilae-classification"
     ],
     "updated": true,
     "goals": [
      "Place a seizure in one of the four classes",
      "apply consciousness as a classifier correctly",
      "describe semiology in the basic and expanded formats"
     ],
     "blocks": [
      {
       "type": "p",
       "updated": true,
       "label": "ILAE 2025",
       "text": "The update keeps the 2017 structure but makes consciousness, judged by awareness and responsiveness together, the classifier for focal seizures and seizures of unknown onset. Motor versus non-motor onset is now a descriptor rather than a class, and negative myoclonus is recognized as a generalized seizure type."
      },
      {
       "type": "h3",
       "text": "Four classes"
      },
      {
       "type": "li",
       "text": "**Focal** seizures begin in networks within one hemisphere. Classify consciousness as preserved or impaired if you can; if you cannot tell, stay with \"focal seizure\". A focal seizure that spreads to both sides is a **focal to bilateral tonic–clonic** seizure."
      },
      {
       "type": "li",
       "text": "**Generalized** seizures engage bilateral networks rapidly. They are grouped into absence seizures (typical, atypical, myoclonic absence, eyelid myoclonia), generalized tonic–clonic seizures, and other generalized seizures (myoclonic, myoclonic–atonic, atonic, clonic, tonic, negative myoclonic, epileptic spasms). Consciousness is not used to classify them."
      },
      {
       "type": "li",
       "text": "**Unknown** whether focal or generalized: some features are known but the onset is not."
      },
      {
       "type": "li",
       "text": "**Unclassified**: the event is considered epileptic but there is too little information to go further."
      },
      {
       "type": "p",
       "text": "The update lists 21 seizure types in all. Neonatal seizures have their own classification."
      },
      {
       "type": "h3",
       "text": "Describing what happened"
      },
      {
       "type": "p",
       "text": "The basic version records whether the seizure had **observable** manifestations or not. The expanded version lists signs in the order they occurred: observable (motor signs, automatisms, autonomic signs, behavioral arrest) and non-observable (sensory, cognitive, emotional). The sequence often localizes better than the class."
      },
      {
       "type": "mcq",
       "id": "q-1ie5sym",
       "stem": "A woman describes a seizure beginning with a rising epigastric sensation. Her husband says she then stared and fumbled with her clothes and did not answer him; afterwards she could not recall the episode. How is this classified under ILAE 2025?",
       "options": [
        "Focal aware seizure with motor onset",
        "Focal seizure with impaired consciousness",
        "Generalized absence seizure",
        "Seizure of unknown onset",
        "Unclassified seizure"
       ],
       "answer": 1,
       "explain": "The epigastric aura indicates focal onset. She was unresponsive and could not recall the event, so consciousness was impaired. Automatisms and the aura are descriptors listed in sequence."
      },
      {
       "type": "mcq",
       "id": "q-1ysovbr",
       "stem": "A seizure is witnessed only in its convulsive phase; no one knows how it began, and the EEG and MRI are normal. How should it be classified?",
       "options": [
        "Generalized tonic–clonic seizure",
        "Focal to bilateral tonic–clonic seizure",
        "Bilateral tonic–clonic seizure of unknown onset",
        "Unclassified seizure",
        "Focal seizure with impaired consciousness"
       ],
       "answer": 2,
       "explain": "When the onset is not known but the seizure clearly became bilateral tonic–clonic, it is classified as of unknown onset. \"Unclassified\" is kept for events with too little information to characterize at all."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-mbyp02",
         "front": "The four seizure classes (ILAE 2025)",
         "back": "Focal; generalized; unknown whether focal or generalized; unclassified."
        },
        {
         "id": "c-bkpln9",
         "front": "Classifier for focal and unknown-onset seizures",
         "back": "Consciousness (impaired or preserved), judged by awareness and responsiveness."
        },
        {
         "id": "c-j7dlaq",
         "front": "If consciousness cannot be determined",
         "back": "Use the parent term, for example \"focal seizure\"."
        },
        {
         "id": "c-b48xja",
         "front": "Does consciousness classify generalized seizures?",
         "back": "No."
        },
        {
         "id": "c-wxmkuu",
         "front": "New generalized seizure type in 2025",
         "back": "Negative myoclonus."
        },
        {
         "id": "c-105456k",
         "front": "Basic versus expanded description",
         "back": "Basic: with or without observable manifestations. Expanded: the chronological sequence of semiology."
        },
        {
         "id": "c-o8r0gw",
         "front": "Number of seizure types in the 2025 classification",
         "back": "21."
        }
       ]
      },
      {
       "type": "note",
       "label": "Try it",
       "text": "Classify an event step by step on the [ILAE Classification page](ilae_classification.html)."
      }
     ],
     "sources": [
      {
       "title": "ILAE updated classification of epileptic seizures (2025)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/40264351/"
      },
      {
       "title": "Practical guide to the 2025 classification",
       "url": "https://pubmed.ncbi.nlm.nih.gov/41081650/"
      },
      {
       "title": "ILAE operational classification of seizure types (2017)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/28276060/"
      }
     ],
     "words": 485,
     "bank": 7
    },
    {
     "id": "epilepsy-classification",
     "n": "3",
     "title": "Classifying the epilepsy (ILAE 2017)",
     "tags": [
      "ilae-classification"
     ],
     "updated": false,
     "goals": [
      "Work through the three levels of the framework",
      "name the six etiology groups",
      "use the terms developmental and epileptic encephalopathy and self-limited correctly"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "Three levels, with etiology throughout"
      },
      {
       "type": "li",
       "text": "**Level 1, seizure type** (as above). Diagnosis can stop here when there is not enough information."
      },
      {
       "type": "li",
       "text": "**Level 2, epilepsy type**: focal, generalized, combined generalized and focal, or unknown. Combined epilepsy is real and easy to miss: Dravet and Lennox–Gastaut syndromes both belong here."
      },
      {
       "type": "li",
       "text": "**Level 3, epilepsy syndrome**: a characteristic cluster of seizure types, EEG and imaging features, age at onset and course. Not every epilepsy fits a syndrome."
      },
      {
       "type": "p",
       "text": "At every level, ask about **etiology**: structural, genetic, infectious, metabolic, immune or unknown. More than one can apply (tuberous sclerosis is both structural and genetic). Consider **comorbidities** (learning, psychiatric, behavioral, sleep) at every level too."
      },
      {
       "type": "h3",
       "text": "Terms that changed"
      },
      {
       "type": "li",
       "text": "**Developmental and epileptic encephalopathy** (DEE): impairment comes from both the underlying cause and the epileptic activity. **Epileptic encephalopathy**: the epileptic activity itself causes the impairment."
      },
      {
       "type": "li",
       "text": "**Self-limited** (likely to remit spontaneously) and **pharmacoresponsive** replaced \"benign\"."
      },
      {
       "type": "li",
       "text": "**Genetic** does not mean inherited: many pathogenic variants in DEEs arise de novo."
      },
      {
       "type": "mcq",
       "id": "q-wplidv",
       "stem": "A 6-year-old with Dravet syndrome has generalized tonic–clonic, myoclonic and focal seizures. What is her epilepsy type in the 2017 framework?",
       "options": [
        "Focal",
        "Generalized",
        "Combined generalized and focal",
        "Unknown",
        "Unclassified"
       ],
       "answer": 2,
       "explain": "Dravet syndrome has both generalized and focal seizure types, which places it in combined generalized and focal epilepsy, as does Lennox–Gastaut syndrome."
      },
      {
       "type": "mcq",
       "id": "q-1gcq7nq",
       "stem": "A child with tuberous sclerosis complex has focal seizures from a cortical tuber. How is the etiology best classified?",
       "options": [
        "Structural only",
        "Genetic only",
        "Structural and genetic",
        "Unknown",
        "Immune"
       ],
       "answer": 2,
       "explain": "Etiology categories are not exclusive. Tuberous sclerosis is caused by TSC1 or TSC2 variants (genetic) and produces tubers (structural)."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-n5b7f3",
         "front": "Three levels of the ILAE 2017 framework",
         "back": "Seizure type, epilepsy type, epilepsy syndrome."
        },
        {
         "id": "c-1m13fch",
         "front": "Epilepsy types",
         "back": "Focal; generalized; combined generalized and focal; unknown."
        },
        {
         "id": "c-220paf",
         "front": "Six etiology groups",
         "back": "Structural, genetic, infectious, metabolic, immune, unknown."
        },
        {
         "id": "c-1wxnq4c",
         "front": "Developmental and epileptic encephalopathy",
         "back": "Impairment from both the underlying cause and the epileptic activity."
        },
        {
         "id": "c-18z5jrw",
         "front": "What replaced \"benign\"",
         "back": "Self-limited and pharmacoresponsive."
        },
        {
         "id": "c-14ln29f",
         "front": "Syndromes with combined generalized and focal epilepsy",
         "back": "Dravet and Lennox–Gastaut syndromes."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "ILAE classification of the epilepsies (2017)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/28276062/"
      },
      {
       "title": "ILAE syndrome classification overview (2022)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/35503715/"
      }
     ],
     "words": 318,
     "bank": 4
    },
    {
     "id": "first-seizure",
     "n": "4",
     "title": "The first seizure",
     "tags": [
      "seizure-management",
      "ilae-classification"
     ],
     "updated": false,
     "goals": [
      "Work up a first seizure efficiently",
      "estimate recurrence risk from the factors that predict it",
      "share the decision about starting an ASM"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "Work-up"
      },
      {
       "type": "li",
       "text": "**History from a witness** is the most valuable test: onset, sequence, duration, recovery, triggers, and any earlier events that may have been seizures (morning jerks, auras, staring spells)."
      },
      {
       "type": "li",
       "text": "**Blood tests** guided by the situation: glucose, sodium, calcium, magnesium, and toxicology."
      },
      {
       "type": "li",
       "text": "**EEG**, ideally early and including sleep."
      },
      {
       "type": "li",
       "text": "**MRI** with an epilepsy protocol. CT is for the emergency question (bleeding, mass, trauma) or when MRI is not possible."
      },
      {
       "type": "li",
       "text": "**Lumbar puncture** if infection or inflammation is suspected."
      },
      {
       "type": "h3",
       "text": "Will it happen again?"
      },
      {
       "type": "p",
       "text": "After a first unprovoked seizure in an adult, the risk of recurrence is greatest in the first two years, in the range of roughly 20–45%. Four factors raise it:"
      },
      {
       "type": "li",
       "text": "a prior brain insult (remote symptomatic cause)"
      },
      {
       "type": "li",
       "text": "epileptiform discharges on EEG"
      },
      {
       "type": "li",
       "text": "a significant abnormality on brain imaging"
      },
      {
       "type": "li",
       "text": "a seizure from sleep"
      },
      {
       "type": "h3",
       "text": "To treat or not"
      },
      {
       "type": "p",
       "text": "Starting an ASM right away reduces the recurrence risk over the next two years by roughly a third. It does not improve the chance of long-term remission. Adverse effects are common but usually mild. The decision rests on recurrence risk and on what a second seizure would cost the patient (driving, work, safety), and is best made together."
      },
      {
       "type": "case",
       "id": "case-first-seizure",
       "title": "A first convulsion at 23",
       "intro": [
        "A 23-year-old graduate student had a generalized convulsion at 7 a.m. after a week of little sleep before exams. His roommate says he went stiff, then jerked for about a minute, and was confused for 20 minutes. He bit the side of his tongue."
       ],
       "steps": [
        {
         "narrative": [],
         "stem": "What single question is most likely to change the diagnosis?",
         "options": [
          "Whether he drinks coffee",
          "Whether he has had morning jerks or brief staring spells before",
          "Whether he has headaches",
          "Whether a parent has migraine",
          "Whether he has ever fainted at the dentist"
         ],
         "answer": 1,
         "explain": "A history of morning myoclonus or absences turns an apparently first seizure into juvenile myoclonic epilepsy, which changes the EEG expected, the drug choice and the prognosis."
        },
        {
         "narrative": [
          "He reports years of occasional arm jerks when tired in the morning. EEG shows 4–5 Hz generalized polyspike-and-wave with a photoparoxysmal response. MRI is normal."
         ],
         "stem": "What is the diagnosis?",
         "options": [
          "Acute symptomatic seizure from sleep deprivation",
          "Focal epilepsy with bilateral spread",
          "Juvenile myoclonic epilepsy",
          "Childhood absence epilepsy",
          "Epilepsy with generalized tonic–clonic seizures alone"
         ],
         "answer": 2,
         "explain": "Morning myoclonus, a convulsion after sleep deprivation, and photosensitive generalized polyspike-and-wave make this juvenile myoclonic epilepsy. Sleep deprivation is a trigger, not an acute brain insult, so the seizure is not acute symptomatic."
        },
        {
         "narrative": [],
         "stem": "Which ASM is the best first choice for a man with this syndrome?",
         "options": [
          "Valproate",
          "Carbamazepine",
          "Phenytoin",
          "Gabapentin",
          "Oxcarbazepine"
         ],
         "answer": 0,
         "explain": "Valproate is the most effective drug for idiopathic generalized epilepsy, and SANAD II confirmed its advantage over levetiracetam. In women who could become pregnant, levetiracetam or lamotrigine is usually preferred because of valproate's teratogenicity. Carbamazepine, oxcarbazepine, phenytoin and gabapentin can worsen myoclonus."
        },
        {
         "narrative": [],
         "stem": "What advice matters most for his safety now?",
         "options": [
          "He may drive as usual once treatment starts",
          "He must follow his state's driving restrictions, and avoid sleep deprivation and binge drinking",
          "He can stop the ASM after a year seizure-free",
          "He needs a helmet at all times",
          "He should avoid all screens"
         ],
         "answer": 1,
         "explain": "Driving rules follow local law. Sleep deprivation and alcohol are the main modifiable triggers in juvenile myoclonic epilepsy, which usually needs long-term treatment; relapse after withdrawal is common."
        }
       ],
       "outro": [
        "The history of earlier minor seizures is the most frequently missed piece of a \"first seizure\" work-up."
       ]
      },
      {
       "type": "mcq",
       "id": "q-1t7eucx",
       "stem": "Which factor does NOT independently raise recurrence risk after a first unprovoked seizure in adults, according to the AAN/AES guideline?",
       "options": [
        "A prior brain insult such as stroke",
        "Epileptiform discharges on EEG",
        "A significant abnormality on brain imaging",
        "A seizure arising from sleep",
        "Postictal confusion lasting 30 minutes"
       ],
       "answer": 4,
       "explain": "The guideline identifies prior brain insult, epileptiform EEG, significant imaging abnormality and nocturnal seizure. Postictal confusion is common and does not predict recurrence."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-6m6quv",
         "front": "Recurrence risk after a first unprovoked seizure",
         "back": "About 20–45% within two years, greatest early."
        },
        {
         "id": "c-5go5gv",
         "front": "Four predictors of recurrence",
         "back": "Prior brain insult, epileptiform EEG, significant imaging abnormality, seizure from sleep."
        },
        {
         "id": "c-1mibqej",
         "front": "Effect of immediate ASM treatment",
         "back": "Cuts early recurrence by about a third; does not change long-term remission."
        },
        {
         "id": "c-orbtdg",
         "front": "Imaging for a first seizure",
         "back": "MRI with an epilepsy protocol; CT for emergencies or if MRI is impossible."
        },
        {
         "id": "c-15td032",
         "front": "The most missed part of first-seizure history",
         "back": "Earlier minor seizures such as morning jerks, auras or absences."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "AAN/AES guideline: first unprovoked seizure in adults (2015)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/25901057/"
      },
      {
       "title": "SANAD II, generalized and unclassifiable epilepsy (2021)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/33838758/"
      }
     ],
     "words": 598,
     "bank": 8
    },
    {
     "id": "semiology",
     "n": "5",
     "title": "Semiology: localizing and lateralizing",
     "tags": [
      "epilepsy-surgery",
      "ilae-classification"
     ],
     "updated": false,
     "goals": [
      "Localize a seizure from its first sign and sequence",
      "use lateralizing signs with their reliability in mind",
      "recognize frontal and insular seizures that mimic other disorders"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "The first sign localizes; the sequence tells the path"
      },
      {
       "type": "table",
       "head": [
        "Region",
        "Typical features"
       ],
       "rows": [
        [
         "Mesial temporal",
         "Rising epigastric sensation, déjà vu, fear, smell or taste; motionless stare; oral then manual automatisms; lasts one to two minutes; postictal confusion"
        ],
        [
         "Lateral temporal",
         "Auditory aura (Heschl gyrus), vertigo, complex hallucinations; earlier clonic signs and faster spread"
        ],
        [
         "Frontal: orbitofrontal and cingulate",
         "Brief hypermotor seizures from sleep in clusters; thrashing, bicycling, pelvic thrusting, vocalization; fast recovery"
        ],
        [
         "Supplementary motor area",
         "Abrupt asymmetric bilateral tonic posturing (fencing), often with preserved consciousness"
        ],
        [
         "Primary motor",
         "Contralateral clonic jerks, sometimes marching (Jacksonian)"
        ],
        [
         "Parietal",
         "Contralateral tingling, pain, distorted body image, vertigo; may be silent until spread"
        ],
        [
         "Occipital",
         "Colored shapes or flashes, ictal blindness, eye deviation, blinking; postictal headache"
        ],
        [
         "Insula and operculum",
         "Throat constriction, perioral or widespread unpleasant sensations, hypersalivation, dysarthria; can mimic temporal or hypermotor frontal seizures"
        ],
        [
         "Hypothalamic hamartoma",
         "Gelastic seizures (laughter without mirth) starting in early childhood"
        ]
       ]
      },
      {
       "type": "h3",
       "text": "Lateralizing signs"
      },
      {
       "type": "table",
       "head": [
        "Sign",
        "Side of onset"
       ],
       "rows": [
        [
         "Dystonic posturing of one limb",
         "Opposite side"
        ],
        [
         "Unilateral manual automatisms (with dystonia of the other hand)",
         "Same side"
        ],
        [
         "Forced, sustained head version before generalization",
         "Opposite side"
        ],
        [
         "Figure-of-4 posture",
         "Opposite to the extended arm"
        ],
        [
         "Last clonic jerks of a convulsion",
         "Same side"
        ],
        [
         "Todd paralysis",
         "Opposite side"
        ],
        [
         "Postictal nose-wiping",
         "Hand on the same side"
        ],
        [
         "Postictal aphasia",
         "Dominant hemisphere"
        ],
        [
         "Preserved speech during an impaired-consciousness seizure",
         "Non-dominant hemisphere"
        ],
        [
         "Ictal vomiting, spitting or urge to urinate",
         "Non-dominant temporal lobe"
        ],
        [
         "Unilateral eye blinking",
         "Same side"
        ]
       ]
      },
      {
       "type": "note",
       "label": "Board pearl",
       "text": "Early, mild, non-forced head turning is unreliable and may be to the same side. Only forced, sustained version shortly before the convulsion lateralizes to the opposite hemisphere."
      },
      {
       "type": "match",
       "id": "match-lateralizing",
       "prompt": "Match each sign to the side of seizure onset it suggests",
       "pairs": [
        [
         "Dystonic posturing of the right hand",
         "Left hemisphere"
        ],
        [
         "Postictal wiping of the nose with the left hand",
         "Left hemisphere"
        ],
        [
         "Forced, sustained head version to the right just before convulsing",
         "Left hemisphere"
        ],
        [
         "Preserved speech during a focal impaired-consciousness seizure",
         "Non-dominant hemisphere"
        ],
        [
         "Ictal vomiting",
         "Non-dominant temporal lobe"
        ],
        [
         "Postictal aphasia",
         "Dominant hemisphere"
        ]
       ]
      },
      {
       "type": "mcq",
       "id": "q-3o7i4g",
       "stem": "A right-handed man's seizures begin with oral automatisms, then left-hand fumbling while the right arm becomes dystonic. Where do they most likely start?",
       "options": [
        "Right temporal lobe",
        "Left temporal lobe",
        "Left frontal lobe",
        "Right parietal lobe",
        "Generalized onset"
       ],
       "answer": 1,
       "explain": "Unilateral manual automatisms are ipsilateral and dystonic posturing is contralateral to the onset. Left-hand automatisms with right-arm dystonia point to the left temporal lobe, fitting the oral automatisms of mesial temporal onset."
      },
      {
       "type": "mcq",
       "id": "q-1pru0rr",
       "stem": "A 19-year-old has clusters of 30-second nocturnal episodes with violent thrashing and bicycling, several a night, with immediate recovery. Sleep specialists suspect a parasomnia. Which feature favors sleep-related hypermotor epilepsy?",
       "options": [
        "Episodes in the first third of the night only",
        "Onset in early childhood with resolution by adolescence",
        "Highly stereotyped, brief episodes in clusters from any sleep stage",
        "Complex, variable behaviors with no recall",
        "Walking around the house for 20 minutes"
       ],
       "answer": 2,
       "explain": "Hypermotor frontal seizures are brief, stereotyped, clustered and can arise at any time of night. NREM parasomnias occur mainly in the first third of the night, are variable and longer, and usually stop in adolescence."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-cmp8bj",
         "front": "Epigastric rising aura",
         "back": "Mesial temporal lobe."
        },
        {
         "id": "c-177ozpj",
         "front": "Auditory aura",
         "back": "Lateral temporal neocortex (Heschl gyrus)."
        },
        {
         "id": "c-ulq5z6",
         "front": "Fencing posture with preserved consciousness",
         "back": "Supplementary motor area."
        },
        {
         "id": "c-1lrjq6a",
         "front": "Gelastic seizures from early childhood",
         "back": "Hypothalamic hamartoma."
        },
        {
         "id": "c-vl126v",
         "front": "Throat constriction and perioral tingling",
         "back": "Insular or opercular onset."
        },
        {
         "id": "c-ir8bj0",
         "front": "Colored circles and ictal blindness",
         "back": "Occipital lobe."
        },
        {
         "id": "c-1m28p8o",
         "front": "Dystonic limb",
         "back": "Contralateral to onset."
        },
        {
         "id": "c-xkc6hd",
         "front": "Unilateral manual automatisms",
         "back": "Ipsilateral to onset."
        },
        {
         "id": "c-1ly0r6k",
         "front": "Figure-of-4",
         "back": "Onset opposite the extended arm."
        },
        {
         "id": "c-1ef8mgx",
         "front": "Last clonic jerk of a convulsion",
         "back": "Ipsilateral to onset."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "ILAE updated classification of epileptic seizures (2025)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/40264351/"
      },
      {
       "title": "Practical guide to the 2025 classification",
       "url": "https://pubmed.ncbi.nlm.nih.gov/41081650/"
      }
     ],
     "words": 563,
     "bank": 14
    },
    {
     "id": "mimics",
     "n": "6",
     "title": "Mimics of epilepsy",
     "tags": [
      "ilae-classification",
      "seizure-management"
     ],
     "updated": false,
     "goals": [
      "Separate syncope, functional seizures and sleep disorders from epileptic seizures at the bedside",
      "use video-EEG and laboratory adjuncts appropriately",
      "deliver a functional seizure diagnosis well"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "Syncope"
      },
      {
       "type": "p",
       "text": "A prodrome of light-headedness, warmth, sweating, tunnel vision or muffled hearing; a trigger (standing, heat, pain, venepuncture, micturition); pallor. Brief irregular jerks are common (**convulsive syncope**), usually lasting under 15 seconds and starting after the fall. Recovery of orientation is quick. Cardiac syncope (arrhythmia, structural disease, long QT) can happen during exertion or lying down and needs cardiac work-up."
      },
      {
       "type": "h3",
       "text": "Functional (dissociative) seizures"
      },
      {
       "type": "p",
       "text": "Features that favor a functional seizure:"
      },
      {
       "type": "li",
       "text": "eyes closed during the event, often resisting opening (the most reliable single sign)"
      },
      {
       "type": "li",
       "text": "long duration, waxing and waning, pauses"
      },
      {
       "type": "li",
       "text": "side-to-side head or body movements, asynchronous limb movements, pelvic thrusting"
      },
      {
       "type": "li",
       "text": "ictal crying, and memory of the event despite apparent unresponsiveness"
      },
      {
       "type": "li",
       "text": "occurring from apparent sleep, with the EEG showing wakefulness"
      },
      {
       "type": "p",
       "text": "The diagnostic standard is **video-EEG capture of a typical event** with no ictal change and a history consistent with the diagnosis. Some people have both epilepsy and functional seizures. Explain the diagnosis clearly and without blame, taper ASMs if there is no epilepsy, and refer for psychological treatment, where CBT-informed therapy has the best evidence."
      },
      {
       "type": "h3",
       "text": "Sleep disorders"
      },
      {
       "type": "li",
       "text": "**NREM arousal disorders** (confusional arousals, sleepwalking, night terrors): childhood, first third of the night, variable, longer, often with amnesia."
      },
      {
       "type": "li",
       "text": "**REM sleep behavior disorder**: older adults, dream enactment in the last part of the night; associated with synucleinopathies."
      },
      {
       "type": "li",
       "text": "**Sleep-related hypermotor epilepsy**: brief, stereotyped, clustered, any time of night."
      },
      {
       "type": "h3",
       "text": "Other mimics"
      },
      {
       "type": "p",
       "text": "Migraine aura spreads over minutes rather than seconds, with positive then negative symptoms. TIA gives negative symptoms. Transient global amnesia, paroxysmal kinesigenic dyskinesia (PRRT2; brief attacks on sudden movement, responsive to carbamazepine), hyperekplexia, hypoglycemia, and in infants breath-holding spells, Sandifer syndrome, shuddering attacks and benign myoclonus of infancy."
      },
      {
       "type": "h3",
       "text": "Laboratory adjuncts"
      },
      {
       "type": "p",
       "text": "Serum prolactin rises 10–20 minutes after a generalized tonic–clonic or focal impaired-consciousness seizure and then falls, so timing matters. A normal value does not exclude a seizure, and syncope can raise it too. Lateral tongue biting favors an epileptic seizure; incontinence does not discriminate."
      },
      {
       "type": "case",
       "id": "case-functional",
       "title": "Events that do not stop",
       "intro": [
        "A 27-year-old woman has had \"seizures\" for two years despite levetiracetam, lamotrigine and lacosamide. Events last 5–15 minutes, with her eyes shut, side-to-side head movements, and crying afterwards. She has been intubated twice in the emergency department."
       ],
       "steps": [
        {
         "narrative": [],
         "stem": "What is the most important next step?",
         "options": [
          "Add a fourth ASM",
          "Refer for epilepsy surgery evaluation",
          "Admit for video-EEG monitoring to capture typical events",
          "Measure prolactin at her next clinic visit",
          "Obtain a PET scan"
         ],
         "answer": 2,
         "explain": "Two failed ASMs should prompt review of the diagnosis. Video-EEG capture of typical events is the diagnostic standard for functional seizures and is required before surgery could ever be considered."
        },
        {
         "narrative": [
          "During monitoring she has three typical events. The EEG shows continuous posterior alpha throughout, with muscle artifact, and no ictal change. She recalls being spoken to during one event."
         ],
         "stem": "How should the diagnosis be communicated?",
         "options": [
          "Tell her the events are \"not real\" and discharge her",
          "Avoid naming the diagnosis to prevent distress",
          "Explain that these are functional (dissociative) seizures: real, common and treatable, and not epilepsy",
          "Say that the test was inconclusive",
          "Continue all ASMs in case some events are epileptic"
         ],
         "answer": 2,
         "explain": "A clear, non-judgmental explanation that names the condition and emphasizes that it is real and treatable improves engagement with treatment."
        },
        {
         "narrative": [],
         "stem": "What should happen to her ASMs and ongoing care?",
         "options": [
          "Continue all three indefinitely",
          "Taper the ASMs if there is no evidence of coexisting epilepsy, and refer for psychological therapy with neurology follow-up",
          "Stop all three abruptly today",
          "Switch to a benzodiazepine for events",
          "Discharge from neurology care"
         ],
         "answer": 1,
         "explain": "Without evidence of epilepsy, ASMs add risk without benefit and are tapered. Psychological therapy (CBT-informed approaches have the best evidence) with continued neurological support gives the best outcomes."
        }
       ],
       "outro": [
        "Two failed ASMs should always prompt a diagnostic review. About one person in five referred to epilepsy centers for drug-resistant epilepsy turns out to have functional seizures."
       ]
      },
      {
       "type": "mcq",
       "id": "q-1xdojqc",
       "stem": "Which sign most strongly favors a functional (dissociative) seizure over an epileptic seizure?",
       "options": [
        "Incontinence of urine",
        "Injury during the event",
        "Eyes closed during the event, resisting opening",
        "Occurrence during the day",
        "A raised creatine kinase the next day"
       ],
       "answer": 2,
       "explain": "Sustained eye closure during an event is one of the most reliable signs of a functional seizure; the eyes are usually open in epileptic convulsions. Incontinence and injury occur in both."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-1veric5",
         "front": "Most reliable bedside sign of a functional seizure",
         "back": "Eyes closed during the event, often resisting opening."
        },
        {
         "id": "c-hw1r33",
         "front": "Diagnostic standard for functional seizures",
         "back": "Video-EEG capture of a typical event without ictal change, with a consistent history."
        },
        {
         "id": "c-10yy064",
         "front": "Convulsive syncope",
         "back": "Brief irregular jerks, usually under 15 s, after the fall; quick reorientation."
        },
        {
         "id": "c-xsyso5",
         "front": "Prolactin after a seizure",
         "back": "Rises 10–20 minutes after a convulsion or focal impaired-consciousness seizure; also after syncope."
        },
        {
         "id": "c-1oezhe",
         "front": "Lateral tongue bite",
         "back": "Favors an epileptic seizure."
        },
        {
         "id": "c-135bjv2",
         "front": "PRRT2 attacks triggered by sudden movement",
         "back": "Paroxysmal kinesigenic dyskinesia; responds to low-dose carbamazepine."
        },
        {
         "id": "c-xu2pxi",
         "front": "Evidence-based treatment for functional seizures",
         "back": "Psychological therapy, especially CBT-informed approaches."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "ILAE minimum requirements for diagnosing psychogenic nonepileptic seizures (LaFrance 2013)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/24111933/"
      },
      {
       "title": "ILAE practical clinical definition of epilepsy (2014)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/24730690/"
      }
     ],
     "words": 679,
     "bank": 11
    }
   ],
   "intro": [
    "Before any drug is chosen the questions are the same: was it a seizure, was it provoked, is this epilepsy, and what kind. The definitions below are operational: each one changes what you do next."
   ]
  },
  {
   "id": "syndromes",
   "n": "6",
   "title": "Syndromes, genetics and causes",
   "short": "Syndromes",
   "sections": [
    {
     "id": "syndrome-framework",
     "n": "1",
     "title": "How the 2022 syndrome classification works",
     "tags": [
      "ilae-classification"
     ],
     "updated": true,
     "goals": [
      "Explain how syndromes are grouped",
      "use the renamed syndromes",
      "know what a syndrome diagnosis requires"
     ],
     "blocks": [
      {
       "type": "p",
       "updated": true,
       "label": "ILAE 2022",
       "text": "For the first time the ILAE published a full syndrome classification, in four papers by age at onset: neonates and infants, childhood, variable age, and the idiopathic generalized epilepsies. Most familiar names changed, and \"benign\" and \"catastrophic\" were retired."
      },
      {
       "type": "h3",
       "text": "The building blocks"
      },
      {
       "type": "li",
       "text": "Syndromes are grouped by **age at onset**: neonatal and infantile (up to 2 years), childhood, and variable age (childhood or adulthood)."
      },
      {
       "type": "li",
       "text": "Within each group they are **self-limited** (likely to remit), **developmental and epileptic encephalopathies**, or defined by a specific cause (**etiology-specific** syndromes such as KCNQ2-DEE, CDKL5-DEE or Sturge–Weber syndrome)."
      },
      {
       "type": "li",
       "text": "The **idiopathic generalized epilepsies** are four syndromes only: childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy, and epilepsy with generalized tonic–clonic seizures alone."
      },
      {
       "type": "li",
       "text": "Each syndrome lists **mandatory** criteria, **exclusionary** criteria, and **alerts**: features that are rare in the syndrome and should prompt caution before diagnosing it."
      },
      {
       "type": "figure",
       "html": "syndrome-ages",
       "caption": "Typical age at onset for the syndromes in this part, on a logarithmic age scale, using the ranges given in the sections that follow. Point at or tab to a bar for its EEG signature and a clue.",
       "n": 7
      },
      {
       "type": "h3",
       "text": "The rename table"
      },
      {
       "type": "table",
       "head": [
        "Older name",
        "2022 name"
       ],
       "rows": [
        [
         "Benign familial neonatal seizures",
         "Self-limited (familial) neonatal epilepsy (SeLNE)"
        ],
        [
         "Benign familial infantile seizures",
         "Self-limited (familial) infantile epilepsy (SeLIE)"
        ],
        [
         "Ohtahara syndrome and early myoclonic encephalopathy",
         "Early infantile developmental and epileptic encephalopathy (EIDEE)"
        ],
        [
         "West syndrome, infantile spasms",
         "Infantile epileptic spasms syndrome (IESS)"
        ],
        [
         "Benign epilepsy with centrotemporal spikes",
         "Self-limited epilepsy with centrotemporal spikes (SeLECTS)"
        ],
        [
         "Panayiotopoulos syndrome",
         "Self-limited epilepsy with autonomic seizures (SeLEAS)"
        ],
        [
         "Late-onset occipital epilepsy (Gastaut type)",
         "Childhood occipital visual epilepsy (COVE)"
        ],
        [
         "Doose syndrome, myoclonic-astatic epilepsy",
         "Epilepsy with myoclonic–atonic seizures (EMAtS)"
        ],
        [
         "Jeavons syndrome",
         "Epilepsy with eyelid myoclonia (EEM)"
        ],
        [
         "Continuous spike-and-wave in sleep, Landau–Kleffner syndrome",
         "DEE or EE with spike-wave activation in sleep (DEE-SWAS, EE-SWAS)"
        ],
        [
         "Epilepsy with generalized tonic–clonic seizures on awakening",
         "Epilepsy with generalized tonic–clonic seizures alone (GTCA)"
        ],
        [
         "Autosomal dominant nocturnal frontal lobe epilepsy",
         "Sleep-related hypermotor (hyperkinetic) epilepsy (SHE)"
        ],
        [
         "Autosomal dominant epilepsy with auditory features",
         "Epilepsy with auditory features (EAF)"
        ]
       ]
      },
      {
       "type": "match",
       "id": "match-renames",
       "prompt": "Match each older name to its 2022 name",
       "pairs": [
        [
         "West syndrome",
         "Infantile epileptic spasms syndrome"
        ],
        [
         "Panayiotopoulos syndrome",
         "Self-limited epilepsy with autonomic seizures"
        ],
        [
         "Doose syndrome",
         "Epilepsy with myoclonic–atonic seizures"
        ],
        [
         "Ohtahara syndrome",
         "Early infantile developmental and epileptic encephalopathy"
        ],
        [
         "Landau–Kleffner syndrome",
         "Epileptic encephalopathy with spike-wave activation in sleep"
        ],
        [
         "Benign rolandic epilepsy",
         "Self-limited epilepsy with centrotemporal spikes"
        ]
       ]
      },
      {
       "type": "mcq",
       "id": "q-144gnmb",
       "stem": "Which is NOT one of the four idiopathic generalized epilepsies in the 2022 classification?",
       "options": [
        "Childhood absence epilepsy",
        "Juvenile absence epilepsy",
        "Juvenile myoclonic epilepsy",
        "Epilepsy with generalized tonic–clonic seizures alone",
        "Epilepsy with myoclonic–atonic seizures"
       ],
       "answer": 4,
       "explain": "The IGEs are CAE, JAE, JME and GTCA. Epilepsy with myoclonic–atonic seizures is a generalized developmental and epileptic encephalopathy of childhood."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-c4bju7",
         "front": "What \"alerts\" mean in the 2022 syndrome criteria",
         "back": "Features rare in the syndrome that should prompt caution before diagnosing it."
        },
        {
         "id": "c-19xuckd",
         "front": "The four idiopathic generalized epilepsies",
         "back": "CAE, JAE, JME and GTCA."
        },
        {
         "id": "c-wv5y4c",
         "front": "Age groups in the 2022 syndrome papers",
         "back": "Neonatal and infantile, childhood, variable age (plus the IGE paper)."
        },
        {
         "id": "c-1yrncyy",
         "front": "Etiology-specific syndrome examples",
         "back": "KCNQ2-DEE, CDKL5-DEE, PCDH19 clustering epilepsy, GLUT1 deficiency, Sturge–Weber syndrome, gelastic seizures with hypothalamic hamartoma."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "ILAE syndrome classification overview (2022)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/35503715/"
      },
      {
       "title": "Syndromes with onset in neonates and infants (2022)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/35503712/"
      },
      {
       "title": "Syndromes with onset in childhood (2022)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/35503717/"
      },
      {
       "title": "Syndromes with onset at a variable age (2022)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/35503725/"
      },
      {
       "title": "Idiopathic generalized epilepsy syndromes (2022)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/35503716/"
      }
     ],
     "words": 492,
     "bank": 2
    },
    {
     "id": "syndromes-infancy",
     "n": "2",
     "title": "Neonatal and infantile onset",
     "tags": [
      "ilae-classification",
      "seizure-management"
     ],
     "updated": false,
     "goals": [
      "Recognize the self-limited and encephalopathic syndromes of the first two years",
      "treat infantile spasms urgently",
      "spot Dravet syndrome early"
     ],
     "blocks": [
      {
       "type": "table",
       "head": [
        "Syndrome",
        "Onset",
        "Key features",
        "Genes and notes"
       ],
       "rows": [
        [
         "SeLNE",
         "First days of life (typically day 2–7)",
         "Focal tonic or clonic seizures, often with apnea, in a well baby; remits within months",
         "KCNQ2 (most), KCNQ3; autosomal dominant"
        ],
        [
         "Self-limited neonatal-infantile epilepsy",
         "Days to the second year",
         "Clusters of focal seizures; remits",
         "Often SCN2A"
        ],
        [
         "SeLIE",
         "First two years, peak around 6 months",
         "Clusters of focal seizures; development normal",
         "PRRT2; relatives may have paroxysmal kinesigenic dyskinesia"
        ],
        [
         "EIDEE",
         "Under 3 months",
         "Tonic seizures, spasms, myoclonus; burst-suppression or severely abnormal EEG; severe delay",
         "STXBP1, KCNQ2, SCN2A, CDKL5 and structural causes"
        ],
        [
         "Epilepsy of infancy with migrating focal seizures",
         "First months",
         "Focal seizures shifting between hemispheres; developmental regression",
         "KCNT1 most often"
        ],
        [
         "IESS",
         "1–24 months",
         "Epileptic spasms in clusters on waking; hypsarrhythmia or multifocal discharges; developmental plateau",
         "Tuberous sclerosis, hypoxic-ischemic injury, malformations, genetic and metabolic causes"
        ],
        [
         "Dravet syndrome",
         "Usually 3–12 months",
         "Prolonged febrile and afebrile hemiclonic or generalized seizures; later myoclonic, atypical absence and focal seizures; development slows after year one",
         "SCN1A loss of function in most, usually de novo"
        ],
        [
         "Myoclonic epilepsy in infancy",
         "Infancy",
         "Myoclonic seizures in a developmentally normal child; responds to treatment",
         "Often no cause found"
        ]
       ]
      },
      {
       "type": "h3",
       "text": "Infantile epileptic spasms syndrome"
      },
      {
       "type": "li",
       "text": "Treat as an emergency. A shorter lag between onset and treatment is associated with better development."
      },
      {
       "type": "li",
       "text": "**First-line**: hormonal therapy (ACTH or high-dose prednisolone) or vigabatrin. **Vigabatrin is preferred when the cause is tuberous sclerosis.**"
      },
      {
       "type": "li",
       "text": "In the ICISS trial, hormonal therapy plus vigabatrin stopped spasms more often than hormonal therapy alone, without a difference in development at 18 months."
      },
      {
       "type": "li",
       "text": "Confirm response with EEG: resolution of hypsarrhythmia matters as well as clinical cessation."
      },
      {
       "type": "h3",
       "text": "Dravet syndrome"
      },
      {
       "type": "li",
       "text": "Suspect it in an infant with **prolonged, often hemiclonic, febrile seizures** before age one, triggered by fever, warm baths or vaccination, especially when they recur."
      },
      {
       "type": "li",
       "text": "Test for SCN1A early."
      },
      {
       "type": "li",
       "text": "**Avoid sodium channel blockers** (carbamazepine, oxcarbazepine, phenytoin, lamotrigine), which worsen seizures."
      },
      {
       "type": "li",
       "text": "Useful drugs: valproate, clobazam, stiripentol, fenfluramine and cannabidiol; the ketogenic diet also helps."
      },
      {
       "type": "li",
       "text": "SUDEP risk is high. Every family needs a rescue plan for prolonged seizures."
      },
      {
       "type": "case",
       "id": "case-dravet",
       "title": "Recurrent febrile status in an infant",
       "intro": [
        "A 7-month-old girl has a 35-minute right-sided clonic seizure with a temperature of 38.4 °C, eight days after her routine vaccinations. Development has been normal. MRI and CSF are normal."
       ],
       "steps": [
        {
         "narrative": [],
         "stem": "Six weeks later she has a 25-minute left-sided clonic seizure during a warm bath, without fever. Which diagnosis must be considered now?",
         "options": [
          "Simple febrile seizures",
          "Self-limited infantile epilepsy",
          "Dravet syndrome",
          "Infantile epileptic spasms syndrome",
          "Childhood absence epilepsy"
         ],
         "answer": 2,
         "explain": "Prolonged hemiclonic seizures of alternating side in the first year, triggered by fever, vaccination or warm baths in a previously normal infant, are the classic early picture of Dravet syndrome."
        },
        {
         "narrative": [],
         "stem": "What is the most useful next investigation?",
         "options": [
          "Repeat MRI in 3 months",
          "Genetic testing that includes SCN1A",
          "Lumbar puncture for lactate",
          "Prolonged ambulatory EEG only",
          "Muscle biopsy"
         ],
         "answer": 1,
         "explain": "More than 80% of children with Dravet syndrome have an SCN1A variant, usually de novo. Confirmation guides treatment and counseling. Early EEG and MRI are often normal."
        },
        {
         "narrative": [
          "She is found to carry a de novo SCN1A truncating variant. Her local doctor has started carbamazepine."
         ],
         "stem": "What is the most important change?",
         "options": [
          "Stop carbamazepine and use valproate or clobazam, then stiripentol, fenfluramine or cannabidiol as needed",
          "Increase carbamazepine to a higher target level",
          "Add lamotrigine",
          "Switch to phenytoin",
          "Add oxcarbazepine"
         ],
         "answer": 0,
         "explain": "Sodium channel blockers worsen seizures in SCN1A loss-of-function Dravet syndrome. Valproate and clobazam are first-line; stiripentol, fenfluramine and cannabidiol have randomized trial evidence."
        },
        {
         "narrative": [],
         "stem": "What must the family also have?",
         "options": [
          "Nothing further; the drugs will control her seizures",
          "A written rescue plan with a rescue benzodiazepine for prolonged seizures, and counseling about SUDEP",
          "Advice to avoid all vaccinations",
          "A referral for epilepsy surgery",
          "Daily antipyretics"
         ],
         "answer": 1,
         "explain": "Prolonged seizures and SUDEP are the main dangers. Vaccination should continue, with fever management around it; vaccination may trigger the first seizure but does not cause the syndrome."
        }
       ],
       "outro": [
        "Recurrent prolonged febrile seizures in the first year are not \"just febrile seizures\". Test early, and keep sodium channel blockers away."
       ]
      },
      {
       "type": "mcq",
       "id": "q-glerh6",
       "stem": "A 5-month-old with tuberous sclerosis complex develops clusters of flexor spasms on waking. EEG shows hypsarrhythmia. What is the preferred first treatment?",
       "options": [
        "Levetiracetam",
        "Vigabatrin",
        "Carbamazepine",
        "Wait two weeks to confirm the diagnosis",
        "Phenytoin"
       ],
       "answer": 1,
       "explain": "Vigabatrin is first-line for epileptic spasms in tuberous sclerosis. Treatment of infantile spasms should start without delay because a shorter lead time is associated with better development."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-rlm60n",
         "front": "SeLNE gene",
         "back": "KCNQ2 (most), KCNQ3."
        },
        {
         "id": "c-1o9n8hi",
         "front": "SeLIE gene",
         "back": "PRRT2; relatives may have paroxysmal kinesigenic dyskinesia."
        },
        {
         "id": "c-y7iugl",
         "front": "EIDEE replaced",
         "back": "Ohtahara syndrome and early myoclonic encephalopathy."
        },
        {
         "id": "c-1tp333m",
         "front": "Migrating focal seizures of infancy gene",
         "back": "KCNT1 most often."
        },
        {
         "id": "c-1py9bo",
         "front": "First-line for infantile spasms",
         "back": "Hormonal therapy (ACTH or high-dose prednisolone) or vigabatrin; vigabatrin if tuberous sclerosis."
        },
        {
         "id": "c-1cbgju7",
         "front": "ICISS trial finding",
         "back": "Hormonal therapy plus vigabatrin stopped spasms more often than hormones alone; no developmental difference at 18 months."
        },
        {
         "id": "c-1039q1t",
         "front": "Dravet red flags",
         "back": "Prolonged febrile hemiclonic seizures before age one, alternating sides, triggered by fever, baths or vaccination."
        },
        {
         "id": "c-1lxvf64",
         "front": "Drugs to avoid in Dravet syndrome",
         "back": "Sodium channel blockers: carbamazepine, oxcarbazepine, phenytoin, lamotrigine."
        },
        {
         "id": "c-hbqbof",
         "front": "Drugs with trial evidence in Dravet syndrome",
         "back": "Stiripentol, fenfluramine, cannabidiol (plus valproate and clobazam first-line)."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "Syndromes with onset in neonates and infants (2022)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/35503712/"
      },
      {
       "title": "ICISS trial (2017)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/27838190/"
      },
      {
       "title": "Fenfluramine in Dravet syndrome (2019)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/31862249/"
      },
      {
       "title": "EPISTOP, preventive vigabatrin in TSC (2021)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/33180985/"
      }
     ],
     "words": 728,
     "bank": 12
    },
    {
     "id": "syndromes-childhood",
     "n": "3",
     "title": "Childhood onset",
     "tags": [
      "ilae-classification",
      "seizure-management",
      "eeg-fundamentals"
     ],
     "updated": false,
     "goals": [
      "Distinguish the self-limited focal epilepsies of childhood",
      "manage childhood absence epilepsy",
      "recognize Lennox–Gastaut syndrome and spike-wave activation in sleep"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "Self-limited focal epilepsies"
      },
      {
       "type": "table",
       "head": [
        "Syndrome",
        "Age",
        "Seizures",
        "EEG",
        "Course"
       ],
       "rows": [
        [
         "SeLECTS",
         "About 4–10 years",
         "Hemifacial clonic seizures, drooling, speech arrest with preserved awareness, mostly from sleep; may become bilateral",
         "Centrotemporal spikes with a horizontal dipole, activated by sleep",
         "Remits by mid-adolescence; treatment often unnecessary"
        ],
        [
         "SeLEAS",
         "About 3–6 years",
         "Autonomic seizures from sleep: nausea, vomiting, pallor, then eye deviation and unresponsiveness; often prolonged",
         "Multifocal spikes, often occipital; may be normal",
         "Few seizures; remits within a few years"
        ],
        [
         "COVE",
         "Later childhood",
         "Brief elementary visual hallucinations, sometimes ictal blindness, often followed by headache",
         "Occipital spikes, often with fixation-off sensitivity",
         "Often remits; many need treatment"
        ],
        [
         "Photosensitive occipital lobe epilepsy",
         "Childhood and adolescence",
         "Visual seizures triggered by flicker, screens or video games",
         "Occipital spikes; photoparoxysmal response",
         "Avoid triggers; often needs treatment"
        ]
       ]
      },
      {
       "type": "h3",
       "text": "Generalized"
      },
      {
       "type": "li",
       "text": "**Childhood absence epilepsy**: onset about 2–12 years in a normal child, many typical absences a day, 3-Hz spike-and-wave provoked by hyperventilation. In the CAE trial, **ethosuximide** and valproate were more effective than lamotrigine, and ethosuximide had fewer attentional side effects, making it first choice for absence-only disease."
      },
      {
       "type": "li",
       "text": "**Epilepsy with myoclonic absences**: absences with rhythmic jerks of the shoulders and arms."
      },
      {
       "type": "li",
       "text": "**Epilepsy with eyelid myoclonia**: eyelid jerks with upward eye deviation on eye closure, with or without absence; photosensitive and often persistent."
      },
      {
       "type": "li",
       "text": "**Epilepsy with myoclonic–atonic seizures**: onset about 6 months to 6 years, often in a previously normal child; myoclonic–atonic drops. The ketogenic diet is often effective; avoid carbamazepine, oxcarbazepine and phenytoin."
      },
      {
       "type": "h3",
       "text": "Developmental and epileptic encephalopathies"
      },
      {
       "type": "li",
       "text": "**Lennox–Gastaut syndrome**: onset before 18 years (usually 1–8), multiple drug-resistant seizure types including **tonic seizures**, cognitive impairment, slow spike-and-wave and paroxysmal fast activity in sleep. Drop attacks cause injury. Options include valproate, clobazam, lamotrigine, rufinamide, topiramate, felbamate, cannabidiol and fenfluramine, the ketogenic diet, VNS and corpus callosotomy."
      },
      {
       "type": "li",
       "text": "**DEE-SWAS and EE-SWAS**: cognitive, language or behavioral regression with marked activation of spike-and-wave in NREM sleep. **Landau–Kleffner syndrome** is the form with acquired auditory verbal agnosia. Causes include early thalamic injury, GRIN2A variants, and atypical evolution of SeLECTS. Treatment: high-dose benzodiazepines, corticosteroids, and other broad-spectrum drugs; sodium channel blockers can make it worse."
      },
      {
       "type": "li",
       "text": "**Febrile infection-related epilepsy syndrome (FIRES)**: a previously healthy child develops super-refractory status days after a febrile illness. It sits within new-onset refractory status (NORSE). The ketogenic diet and anakinra are used; outcomes are often poor."
      },
      {
       "type": "li",
       "text": "**Hemiconvulsion–hemiplegia–epilepsy**: prolonged febrile hemiclonic status in a young child, then hemiplegia and later focal epilepsy."
      },
      {
       "type": "mcq",
       "id": "q-8n2fch",
       "stem": "A 7-year-old boy has had three episodes in which he woke at night with twitching of the left side of his mouth, drooling and inability to speak while fully aware. EEG shows right centrotemporal spikes activated by sleep. Development and examination are normal. What is the best advice?",
       "options": [
        "Start carbamazepine immediately and continue until age 18",
        "Obtain intracranial EEG",
        "Explain the self-limited course; treatment is optional and depends on seizure frequency and family preference",
        "Start a ketogenic diet",
        "Refer for epilepsy surgery"
       ],
       "answer": 2,
       "explain": "SeLECTS remits by mid-adolescence, most children have few seizures, and many need no ASM. Treatment is reasonable if seizures are frequent, prolonged or distressing."
      },
      {
       "type": "mcq",
       "id": "q-1b5tivq",
       "stem": "A 4-year-old girl has an hour-long nocturnal episode of vomiting, pallor and eye deviation, then becomes unresponsive. She recovers fully. EEG shows multifocal spikes, most often occipital. What is the most likely diagnosis?",
       "options": [
        "Childhood occipital visual epilepsy",
        "Self-limited epilepsy with autonomic seizures",
        "Migraine with aura",
        "Encephalitis",
        "Lennox–Gastaut syndrome"
       ],
       "answer": 1,
       "explain": "Prolonged nocturnal autonomic seizures with vomiting and pallor in a preschool child with occipital or multifocal spikes are typical of SeLEAS (formerly Panayiotopoulos syndrome). Most children have few seizures and remit."
      },
      {
       "type": "mcq",
       "id": "q-gwncab",
       "stem": "Which feature is required for a diagnosis of Lennox–Gastaut syndrome?",
       "options": [
        "Hypsarrhythmia",
        "Photosensitivity",
        "Tonic seizures",
        "Centrotemporal spikes",
        "Onset after age 18"
       ],
       "answer": 2,
       "explain": "Tonic seizures are a mandatory criterion, alongside other seizure types, slow spike-and-wave (or paroxysmal fast activity) and cognitive impairment, with onset before 18."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-w3x4ce",
         "front": "SeLECTS seizures",
         "back": "Hemifacial clonic, drooling, speech arrest with awareness, mostly from sleep."
        },
        {
         "id": "c-1ws8qf5",
         "front": "SeLECTS EEG",
         "back": "Centrotemporal spikes with a horizontal dipole, activated by sleep."
        },
        {
         "id": "c-s5tjie",
         "front": "SeLEAS",
         "back": "Prolonged nocturnal autonomic seizures (vomiting, pallor) at about 3–6 years."
        },
        {
         "id": "c-1k88pwy",
         "front": "COVE",
         "back": "Brief elementary visual seizures and ictal blindness with headache after; occipital spikes with fixation-off sensitivity."
        },
        {
         "id": "c-12dyg6j",
         "front": "CAE first choice (CAE trial)",
         "back": "Ethosuximide: as effective as valproate, better than lamotrigine, fewer attention effects."
        },
        {
         "id": "c-1s5zuq9",
         "front": "Epilepsy with eyelid myoclonia",
         "back": "Eyelid jerks on eye closure, photosensitive, often persistent."
        },
        {
         "id": "c-q20smn",
         "front": "Lennox–Gastaut mandatory seizure type",
         "back": "Tonic seizures."
        },
        {
         "id": "c-4ncyvz",
         "front": "Landau–Kleffner syndrome",
         "back": "EE-SWAS with acquired auditory verbal agnosia."
        },
        {
         "id": "c-1b3i5a6",
         "front": "FIRES",
         "back": "Super-refractory status days after a febrile illness in a healthy child; part of NORSE."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "Syndromes with onset in childhood (2022)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/35503717/"
      },
      {
       "title": "Childhood Absence Epilepsy trial (2010)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/20200383/"
      },
      {
       "title": "NORSE and FIRES consensus definitions (2018)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/29399791/"
      }
     ],
     "words": 725,
     "bank": 13
    },
    {
     "id": "syndromes-adult",
     "n": "4",
     "title": "Adolescence, adulthood and variable age",
     "tags": [
      "ilae-classification",
      "seizure-management",
      "epilepsy-surgery"
     ],
     "updated": false,
     "goals": [
      "Separate the idiopathic generalized epilepsies of adolescence",
      "recognize the familial focal epilepsies",
      "know the progressive myoclonus epilepsies"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "Idiopathic generalized epilepsies of adolescence"
      },
      {
       "type": "table",
       "head": [
        "Syndrome",
        "Onset",
        "Seizures",
        "Notes"
       ],
       "rows": [
        [
         "Juvenile absence epilepsy",
         "About 9–13 years",
         "Absences, less frequent than in CAE; most also have tonic–clonic seizures",
         "Usually needs long-term treatment"
        ],
        [
         "Juvenile myoclonic epilepsy",
         "About 10–24 years",
         "Myoclonus after waking; tonic–clonic seizures; absences in some",
         "Photosensitivity common; triggered by sleep loss and alcohol; relapse common after withdrawal"
        ],
        [
         "Epilepsy with generalized tonic–clonic seizures alone",
         "Teens to early adulthood",
         "Tonic–clonic seizures only, often after waking",
         "Triggered by sleep loss"
        ]
       ]
      },
      {
       "type": "p",
       "text": "Valproate is the most effective drug for idiopathic generalized epilepsy. For women who could become pregnant, levetiracetam or lamotrigine is usually preferred."
      },
      {
       "type": "h3",
       "text": "Focal epilepsies of variable age"
      },
      {
       "type": "li",
       "text": "**Sleep-related hypermotor (hyperkinetic) epilepsy**: brief, stereotyped hypermotor seizures from NREM sleep in clusters. Genetic forms involve nicotinic receptor genes (CHRNA4, CHRNB2, CHRNA2), KCNT1 and the GATOR1 genes; focal cortical dysplasia is a common structural cause."
      },
      {
       "type": "li",
       "text": "**Familial focal epilepsy with variable foci**: family members have focal seizures from different lobes. DEPDC5, NPRL2 and NPRL3 (the GATOR1 complex that regulates mTOR) are the main genes, and some carriers have focal cortical dysplasia."
      },
      {
       "type": "li",
       "text": "**Epilepsy with auditory features**: auditory auras (buzzing, ringing), sometimes receptive aphasia; LGI1 variants in some families."
      },
      {
       "type": "li",
       "text": "**Mesial temporal lobe epilepsy with hippocampal sclerosis**: often an early precipitating injury such as prolonged febrile seizures, a latent period, then focal impaired-consciousness seizures that frequently become drug-resistant. Surgery is highly effective."
      },
      {
       "type": "li",
       "text": "**Rasmussen syndrome**: a childhood-onset, T-cell-mediated inflammation of one hemisphere with epilepsia partialis continua, progressive hemiparesis and cognitive decline, and progressive atrophy on MRI. Hemispherotomy is the definitive treatment."
      },
      {
       "type": "h3",
       "text": "Progressive myoclonus epilepsies"
      },
      {
       "type": "p",
       "text": "Suspect one when myoclonus, generalized seizures and progressive cognitive and cerebellar decline occur together."
      },
      {
       "type": "table",
       "head": [
        "Disease",
        "Gene or defect",
        "Clues"
       ],
       "rows": [
        [
         "Unverricht–Lundborg disease",
         "CSTB repeat expansion (autosomal recessive)",
         "Stimulus-sensitive action myoclonus from about 6–15 years; slower cognitive decline; phenytoin worsens it"
        ],
        [
         "Lafora disease",
         "EPM2A or NHLRC1 (recessive)",
         "Teenage onset, occipital seizures with visual hallucinations, rapid dementia; Lafora bodies on axillary skin biopsy"
        ],
        [
         "Neuronal ceroid lipofuscinoses",
         "CLN genes (mostly recessive)",
         "Visual failure and regression; CLN2 disease has intraventricular enzyme replacement (cerliponase alfa)"
        ],
        [
         "MERRF",
         "Mitochondrial DNA (m.8344A>G)",
         "Maternal inheritance; ragged-red fibres, hearing loss, lipomas"
        ],
        [
         "Sialidosis type I",
         "NEU1 (recessive)",
         "Myoclonus with a cherry-red spot, little dementia early"
        ],
        [
         "DRPLA",
         "ATN1 CAG repeat (dominant)",
         "Ataxia, chorea, dementia; anticipation; more common in Japan"
        ]
       ]
      },
      {
       "type": "h3",
       "text": "Reflex epilepsies"
      },
      {
       "type": "p",
       "text": "Seizures reliably triggered by a stimulus: flicker (juvenile myoclonic epilepsy, eyelid myoclonia, photosensitive occipital epilepsy), reading (jaw jerks), startle (often with hemiparesis), hot water, music, eating, and thinking or manipulating objects (praxis induction, common in juvenile myoclonic epilepsy)."
      },
      {
       "type": "mcq",
       "id": "q-19ga7pj",
       "stem": "A 16-year-old develops worsening stimulus-sensitive myoclonus and generalized seizures. Over 18 months she has visual hallucinations, occipital seizures and rapidly declining school performance. Which test is most likely to confirm the diagnosis?",
       "options": [
        "Muscle biopsy for ragged-red fibres",
        "Axillary skin biopsy for Lafora bodies, or genetic testing of EPM2A and NHLRC1",
        "Fundoscopy for a cherry-red spot",
        "Serum ceruloplasmin",
        "CSF 14-3-3 protein"
       ],
       "answer": 1,
       "explain": "Teenage onset with occipital seizures, visual hallucinations and rapid dementia fits Lafora disease. Periodic acid–Schiff-positive Lafora bodies in sweat gland cells on axillary skin biopsy, or EPM2A/NHLRC1 testing, confirm it."
      },
      {
       "type": "mcq",
       "id": "q-1saz5js",
       "stem": "In a patient with Unverricht–Lundborg disease, which drug should be avoided because it can worsen myoclonus and cerebellar signs?",
       "options": [
        "Valproate",
        "Levetiracetam",
        "Clonazepam",
        "Phenytoin",
        "Piracetam"
       ],
       "answer": 3,
       "explain": "Phenytoin worsens Unverricht–Lundborg disease. Sodium channel blockers, gabapentin, pregabalin, vigabatrin and tiagabine can aggravate myoclonus in progressive myoclonus epilepsies."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-2yt6ue",
         "front": "Juvenile absence epilepsy versus CAE",
         "back": "Later onset (about 9–13), fewer absences, most also have tonic–clonic seizures, usually lifelong."
        },
        {
         "id": "c-imfjmo",
         "front": "Juvenile myoclonic epilepsy triggers",
         "back": "Sleep deprivation, alcohol, flicker; praxis-induced jerks."
        },
        {
         "id": "c-k8dsmv",
         "front": "SHE genes",
         "back": "CHRNA4, CHRNB2, CHRNA2, KCNT1, GATOR1 genes."
        },
        {
         "id": "c-1r7zt8z",
         "front": "Familial focal epilepsy with variable foci genes",
         "back": "DEPDC5, NPRL2, NPRL3 (GATOR1, mTOR pathway)."
        },
        {
         "id": "c-ujl2xp",
         "front": "Epilepsy with auditory features gene",
         "back": "LGI1 in some families."
        },
        {
         "id": "c-1l2kjv1",
         "front": "Rasmussen syndrome",
         "back": "Unihemispheric T-cell inflammation, epilepsia partialis continua, progressive hemiparesis; hemispherotomy."
        },
        {
         "id": "c-bcmkyz",
         "front": "Unverricht–Lundborg disease",
         "back": "CSTB repeat expansion; stimulus-sensitive myoclonus; avoid phenytoin."
        },
        {
         "id": "c-1xqoezm",
         "front": "Lafora disease",
         "back": "EPM2A or NHLRC1; occipital seizures, rapid dementia; Lafora bodies on axillary skin biopsy."
        },
        {
         "id": "c-1kh1yfg",
         "front": "CLN2 disease treatment",
         "back": "Intraventricular cerliponase alfa (enzyme replacement)."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "Idiopathic generalized epilepsy syndromes (2022)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/35503716/"
      },
      {
       "title": "Syndromes with onset at a variable age (2022)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/35503725/"
      },
      {
       "title": "SANAD II, generalized and unclassifiable epilepsy (2021)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/33838758/"
      }
     ],
     "words": 642,
     "bank": 9
    },
    {
     "id": "genetics",
     "n": "5",
     "title": "Genetic testing and precision treatment",
     "tags": [
      "ilae-classification",
      "seizure-management"
     ],
     "updated": true,
     "goals": [
      "Choose the first genetic test",
      "interpret the patterns of inheritance that matter for counseling",
      "match genes to treatment decisions"
     ],
     "blocks": [
      {
       "type": "p",
       "updated": true,
       "label": "Genetic testing",
       "text": "The National Society of Genetic Counselors guideline (2022) strongly recommends genetic testing for everyone with unexplained epilepsy, of any age, with exome or genome sequencing (or a panel of more than 25 genes) first and chromosomal microarray after, all with pre- and post-test counseling."
      },
      {
       "type": "h3",
       "text": "Diagnostic yield"
      },
      {
       "type": "p",
       "text": "In a systematic review, diagnostic yield was about 48% for genome sequencing, 24% for exome sequencing, 19% for multigene panels and 9% for chromosomal microarray. Yield was highest in developmental and epileptic encephalopathies and when there were neurodevelopmental comorbidities."
      },
      {
       "type": "h3",
       "text": "Genes that change treatment"
      },
      {
       "type": "table",
       "head": [
        "Finding",
        "What it changes"
       ],
       "rows": [
        [
         "SCN1A loss of function (Dravet syndrome)",
         "Avoid sodium channel blockers; use valproate, clobazam, stiripentol, fenfluramine, cannabidiol"
        ],
        [
         "SCN2A or SCN8A gain of function (usually onset in the first months)",
         "Sodium channel blockers, sometimes at high dose, can work well"
        ],
        [
         "SCN2A loss of function (later onset)",
         "Sodium channel blockers are unhelpful or harmful"
        ],
        [
         "KCNQ2 (self-limited neonatal epilepsy or DEE)",
         "Sodium channel blockers such as carbamazepine often effective"
        ],
        [
         "TSC1 or TSC2",
         "Vigabatrin for spasms; everolimus for refractory focal seizures; early surveillance EEG"
        ],
        [
         "SLC2A1 (GLUT1 deficiency)",
         "Ketogenic diet"
        ],
        [
         "ALDH7A1",
         "Lifelong pyridoxine, with lysine restriction"
        ],
        [
         "PNPO",
         "Pyridoxal 5′-phosphate"
        ],
        [
         "POLG",
         "Avoid valproate (risk of fatal liver failure)"
        ],
        [
         "CDKL5",
         "Ganaxolone is approved for CDKL5 deficiency disorder"
        ]
       ]
      },
      {
       "type": "h3",
       "text": "Inheritance patterns that matter"
      },
      {
       "type": "li",
       "text": "**Autosomal dominant with incomplete penetrance**: many familial focal epilepsies and self-limited syndromes (KCNQ2 neonatal, PRRT2 infantile, CHRNA4, LGI1, DEPDC5) and the GEFS+ spectrum."
      },
      {
       "type": "li",
       "text": "**De novo dominant**: most developmental and epileptic encephalopathies (SCN1A in Dravet, STXBP1, SCN2A, SCN8A, KCNT1). Recurrence risk for parents is low but not zero, because of parental mosaicism."
      },
      {
       "type": "li",
       "text": "**X-linked**: PCDH19 clustering epilepsy affects heterozygous girls and spares hemizygous boys (mosaic boys can be affected); CDKL5 mostly affects girls, usually de novo; DCX causes lissencephaly in boys and band heterotopia in girls; FLNA causes periventricular nodular heterotopia in women."
      },
      {
       "type": "li",
       "text": "**Autosomal recessive**: most progressive myoclonus epilepsies, pyridoxine-dependent epilepsy, POLG disease and most metabolic epilepsies."
      },
      {
       "type": "li",
       "text": "**Maternal (mitochondrial DNA)**: MERRF, MELAS."
      },
      {
       "type": "li",
       "text": "**Somatic (brain-only) variants**: focal cortical dysplasia type II and hemimegalencephaly (mTOR pathway genes) and Sturge–Weber syndrome (GNAQ). Blood testing will miss them."
      },
      {
       "type": "h3",
       "text": "Chromosomal disorders to recognize"
      },
      {
       "type": "li",
       "text": "**Angelman syndrome** (loss of maternal 15q11–q13, including UBE3A): absent speech, ataxia, happy affect, and high-amplitude notched delta on EEG. Loss of the paternal region gives Prader–Willi syndrome, where seizures are uncommon."
      },
      {
       "type": "li",
       "text": "**Ring chromosome 20**: frontal seizures and prolonged nonconvulsive status with confusion; needs a karyotype because mosaicism is missed by microarray."
      },
      {
       "type": "li",
       "text": "**Down syndrome**: infantile spasms in infancy and late-onset myoclonic epilepsy with Alzheimer disease in adulthood."
      },
      {
       "type": "li",
       "text": "**Wolf–Hirschhorn (4p deletion)** and **15q duplication** syndromes both have prominent epilepsy."
      },
      {
       "type": "mcq",
       "id": "q-ikwv7n",
       "stem": "A 4-year-old with global developmental delay and drug-resistant focal and generalized seizures has a normal MRI and a normal chromosomal microarray. What is the most appropriate next genetic test?",
       "options": [
        "No further testing; the microarray was normal",
        "Karyotype",
        "Exome or genome sequencing",
        "A single-gene test for SCN1A only",
        "Mitochondrial DNA testing only"
       ],
       "answer": 2,
       "explain": "Exome or genome sequencing has the highest diagnostic yield and is recommended first-tier for unexplained epilepsy, especially a developmental and epileptic encephalopathy. A normal microarray does not exclude a monogenic cause."
      },
      {
       "type": "mcq",
       "id": "q-6qh9t7",
       "stem": "A family has two daughters with clusters of febrile and afebrile seizures and intellectual disability, both carrying a PCDH19 variant. Their father carries the same variant and is healthy. How is this explained?",
       "options": [
        "Incomplete penetrance of an autosomal dominant variant",
        "Mitochondrial inheritance",
        "X-linked inheritance in which hemizygous males are spared and heterozygous females affected",
        "De novo variants in both daughters",
        "Autosomal recessive inheritance"
       ],
       "answer": 2,
       "explain": "PCDH19 clustering epilepsy follows an unusual X-linked pattern: disease results from a mixture of cells with and without the protein (cellular interference), so heterozygous females and mosaic males are affected while hemizygous males are not."
      },
      {
       "type": "match",
       "id": "match-genes",
       "prompt": "Match each gene to the treatment decision it drives",
       "pairs": [
        [
         "SCN1A loss of function",
         "Avoid sodium channel blockers"
        ],
        [
         "SLC2A1",
         "Ketogenic diet"
        ],
        [
         "POLG",
         "Avoid valproate"
        ],
        [
         "ALDH7A1",
         "Lifelong pyridoxine"
        ],
        [
         "TSC2 with infantile spasms",
         "Vigabatrin"
        ],
        [
         "CDKL5",
         "Ganaxolone"
        ]
       ]
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-1uohy6e",
         "front": "First-tier genetic test for unexplained epilepsy",
         "back": "Exome or genome sequencing (or a panel of more than 25 genes), then chromosomal microarray."
        },
        {
         "id": "c-ac2mzz",
         "front": "Diagnostic yields (systematic review)",
         "back": "Genome about 48%, exome 24%, panel 19%, microarray 9%."
        },
        {
         "id": "c-1seqvbe",
         "front": "Who has the highest genetic yield",
         "back": "Developmental and epileptic encephalopathies and neurodevelopmental comorbidities."
        },
        {
         "id": "c-zjrssi",
         "front": "PCDH19 inheritance",
         "back": "X-linked; heterozygous females and mosaic males affected, hemizygous males spared."
        },
        {
         "id": "c-17jdd8t",
         "front": "Somatic variants missed on blood testing",
         "back": "FCD type II and hemimegalencephaly (mTOR pathway), Sturge–Weber (GNAQ)."
        },
        {
         "id": "c-1upnm0e",
         "front": "Angelman syndrome EEG",
         "back": "High-amplitude notched delta."
        },
        {
         "id": "c-1tf8vq7",
         "front": "Ring chromosome 20 test",
         "back": "Karyotype, because mosaicism is missed by microarray."
        },
        {
         "id": "c-mcf5lb",
         "front": "SCN2A gain versus loss of function",
         "back": "Gain (early onset) responds to sodium channel blockers; loss (later onset) does not."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "NSGC guideline on genetic testing for unexplained epilepsy (2022)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/36281494/"
      },
      {
       "title": "Genetic testing for the epilepsies, systematic review (2022)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/34893972/"
      },
      {
       "title": "EPISTOP, preventive vigabatrin in TSC (2021)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/33180985/"
      }
     ],
     "words": 744,
     "bank": 11
    },
    {
     "id": "febrile",
     "n": "6",
     "title": "Febrile seizures",
     "tags": [
      "ilae-classification",
      "seizure-management"
     ],
     "updated": false,
     "goals": [
      "Define simple and complex febrile seizures",
      "counsel on recurrence and later epilepsy",
      "know which investigations are and are not needed"
     ],
     "blocks": [
      {
       "type": "p",
       "text": "A febrile seizure occurs with a temperature of at least 38 °C in a child aged 6 to 60 months, without CNS infection, an acute metabolic cause, or earlier afebrile seizures. They affect roughly 2–5% of children."
      },
      {
       "type": "table",
       "head": [
        "",
        "Simple",
        "Complex"
       ],
       "rows": [
        [
         "Duration",
         "Under 15 minutes",
         "15 minutes or longer"
        ],
        [
         "Type",
         "Generalized",
         "Focal features"
        ],
        [
         "Recurrence within 24 hours",
         "No",
         "Yes"
        ]
       ]
      },
      {
       "type": "p",
       "text": "**Febrile status epilepticus** (30 minutes or more) is associated with acute hippocampal injury on MRI and, in some children, later hippocampal sclerosis."
      },
      {
       "type": "h3",
       "text": "Counseling"
      },
      {
       "type": "li",
       "text": "**Recurrence**: about a third of children have another. The risk is higher with a first febrile seizure before about 18 months, a family history of febrile seizures, a lower temperature at the time, and a short interval between fever onset and the seizure."
      },
      {
       "type": "li",
       "text": "**Later epilepsy**: after a simple febrile seizure the risk is only slightly above that of the general population. It rises with complex features, a family history of epilepsy, and pre-existing neurodevelopmental problems."
      },
      {
       "type": "li",
       "text": "**Antipyretics** make the child comfortable but do not prevent recurrence. Continuous ASM prophylaxis is not recommended for simple febrile seizures. A rescue benzodiazepine can be offered for prolonged seizures."
      },
      {
       "type": "h3",
       "text": "Investigations after a simple febrile seizure in a well child"
      },
      {
       "type": "li",
       "text": "**Lumbar puncture** if there are meningeal signs or symptoms. Consider it between 6 and 12 months if Haemophilus influenzae type b or pneumococcal vaccination is incomplete or unknown, and when antibiotics were given beforehand."
      },
      {
       "type": "li",
       "text": "**EEG, neuroimaging and routine blood tests** are not needed to evaluate the seizure itself."
      },
      {
       "type": "calc",
       "name": "febrile-check"
      },
      {
       "type": "mcq",
       "id": "q-172p2vw",
       "stem": "A 14-month-old has a 4-minute generalized tonic–clonic seizure on the first day of a febrile illness. She is back to normal an hour later, has no meningeal signs, and is fully vaccinated. What investigation does she need?",
       "options": [
        "EEG within 24 hours",
        "MRI brain",
        "Lumbar puncture",
        "Serum electrolytes and calcium",
        "None directed at the seizure; evaluate the source of fever as usual"
       ],
       "answer": 4,
       "explain": "For a simple febrile seizure in a neurologically healthy, vaccinated child without meningeal signs, the AAP recommends against routine EEG, neuroimaging and blood tests, and LP is not routinely needed."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-6j1jlx",
         "front": "Febrile seizure age range",
         "back": "6 to 60 months."
        },
        {
         "id": "c-156hgxn",
         "front": "Complex febrile seizure",
         "back": "Focal features, 15 minutes or more, or recurrence within 24 hours."
        },
        {
         "id": "c-1bfftqx",
         "front": "Febrile status epilepticus",
         "back": "30 minutes or longer; linked to acute hippocampal injury."
        },
        {
         "id": "c-1wvequo",
         "front": "Recurrence after a first febrile seizure",
         "back": "About one in three."
        },
        {
         "id": "c-1rvib2u",
         "front": "Recurrence risk factors",
         "back": "Onset before about 18 months, family history, lower temperature, short fever-to-seizure interval."
        },
        {
         "id": "c-1f9iqae",
         "front": "Do antipyretics prevent recurrence?",
         "back": "No."
        },
        {
         "id": "c-1jrefkm",
         "front": "Workup after a simple febrile seizure",
         "back": "Evaluate the fever; no routine EEG, imaging or blood tests; LP only if indicated."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "AAP guideline: evaluation of the child with a simple febrile seizure (2011)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/21285335/"
      },
      {
       "title": "Syndromes with onset in childhood (2022)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/35503717/"
      }
     ],
     "words": 417,
     "bank": 5
    },
    {
     "id": "structural-causes",
     "n": "7",
     "title": "Structural causes",
     "tags": [
      "epilepsy-surgery",
      "ilae-classification"
     ],
     "updated": false,
     "goals": [
      "Recognize the malformations, tumors and vascular lesions that cause epilepsy",
      "manage seizure risk after stroke and head injury",
      "know when a lesion means surgery"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "Malformations of cortical development"
      },
      {
       "type": "li",
       "text": "**Focal cortical dysplasia** (ILAE classification updated in 2022): type I is abnormal cortical layering (Ia radial, Ib tangential, Ic both); type II has dysmorphic neurons (IIa) or dysmorphic neurons with balloon cells (IIb); type III sits next to another lesion (IIIa hippocampal sclerosis, IIIb tumor, IIIc vascular malformation, IIId other early-life injury). The 2022 update added **mild malformation with oligodendroglial hyperplasia (MOGHE)**. Type II is often caused by somatic mTOR-pathway variants. MRI shows blurring of the gray–white junction, cortical thickening, and in type IIb a **transmantle sign** tapering toward the ventricle. Complete resection of type II gives excellent results."
      },
      {
       "type": "li",
       "text": "**Heterotopia and lissencephaly**: periventricular nodular heterotopia (FLNA, mostly women); subcortical band heterotopia and lissencephaly (DCX, LIS1)."
      },
      {
       "type": "li",
       "text": "**Polymicrogyria**, often bilateral perisylvian with oromotor dysfunction; **schizencephaly**; **hemimegalencephaly** (somatic mTOR variants, often treated with hemispherotomy)."
      },
      {
       "type": "h3",
       "text": "Tumors"
      },
      {
       "type": "p",
       "text": "Low-grade, slow-growing tumors are the most epileptogenic. **Ganglioglioma** (often BRAF V600E) and **dysembryoplastic neuroepithelial tumor** are the classic long-term epilepsy-associated tumors of the temporal lobe; early complete resection gives the best seizure outcomes. Metastases and high-grade gliomas cause seizures less often. In people with brain tumors who have never had a seizure, prophylactic ASMs are not recommended. When treatment is needed, drugs without enzyme induction (such as levetiracetam) avoid interactions with chemotherapy."
      },
      {
       "type": "h3",
       "text": "Vascular lesions"
      },
      {
       "type": "li",
       "text": "**Cavernous malformations** are highly epileptogenic, through the surrounding hemosiderin; resection including the hemosiderin rim improves seizure outcome. An incidental cavernoma with no seizures needs neither an ASM nor surgery."
      },
      {
       "type": "li",
       "text": "**Arteriovenous malformations** present with seizures in a substantial proportion."
      },
      {
       "type": "li",
       "text": "**Sturge–Weber syndrome** (somatic GNAQ): facial port-wine stain in the V1 area, leptomeningeal angioma, gyriform calcification and hemiatrophy; early focal seizures and stroke-like episodes."
      },
      {
       "type": "h3",
       "text": "After stroke and head injury"
      },
      {
       "type": "li",
       "text": "**Stroke** is the commonest identifiable cause of new epilepsy after 60. Seizures in the first week are acute symptomatic. A seizure after that carries a high recurrence risk, so one late seizure usually justifies treatment. Cortical involvement, hemorrhage, larger and more severe strokes, and early seizures raise the risk."
      },
      {
       "type": "li",
       "text": "**Traumatic brain injury**: penetrating injury, depressed skull fracture, intracranial (especially subdural) hematoma, early seizures and greater severity raise the risk of post-traumatic epilepsy. A week of phenytoin or levetiracetam after severe injury reduces early seizures only."
      },
      {
       "type": "mcq",
       "id": "q-muhcdv",
       "stem": "A 12-year-old has drug-resistant focal seizures. MRI shows blurring of the gray–white junction in the right frontal lobe with a T2 hyperintense band tapering from the cortex toward the lateral ventricle. What is the most likely pathology?",
       "options": [
        "Focal cortical dysplasia type I",
        "Focal cortical dysplasia type IIb",
        "Dysembryoplastic neuroepithelial tumor",
        "Periventricular nodular heterotopia",
        "Hippocampal sclerosis"
       ],
       "answer": 1,
       "explain": "The transmantle sign is characteristic of FCD type IIb (dysmorphic neurons and balloon cells). Type I is often MRI-negative."
      },
      {
       "type": "mcq",
       "id": "q-1d11upl",
       "stem": "A 42-year-old has a 7-mm cortical cavernous malformation found on MRI done for headaches. She has never had a seizure. What is the best management?",
       "options": [
        "Start levetiracetam",
        "Resect the lesion with its hemosiderin rim",
        "No ASM or surgery; follow clinically and explain which symptoms to report",
        "Radiosurgery",
        "Routine EEG every six months"
       ],
       "answer": 2,
       "explain": "An incidental cavernoma without seizures does not warrant prophylactic ASMs or surgery. Resection with the hemosiderin rim is considered when the lesion causes epilepsy or bleeds."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-if3w0i",
         "front": "FCD type IIb",
         "back": "Dysmorphic neurons plus balloon cells; transmantle sign; often somatic mTOR variants."
        },
        {
         "id": "c-bhdto7",
         "front": "FCD type III",
         "back": "Dysplasia adjacent to another lesion: IIIa hippocampal sclerosis, IIIb tumor, IIIc vascular, IIId other early injury."
        },
        {
         "id": "c-1wdc4p9",
         "front": "MOGHE",
         "back": "Mild malformation with oligodendroglial hyperplasia in epilepsy; added in the 2022 FCD update."
        },
        {
         "id": "c-1e0iyts",
         "front": "Most epileptogenic tumors",
         "back": "Low-grade: ganglioglioma and DNET."
        },
        {
         "id": "c-wbhxed",
         "front": "Prophylactic ASM for a brain tumor without seizures",
         "back": "Not recommended."
        },
        {
         "id": "c-1hhkmtg",
         "front": "Cavernoma surgery for epilepsy",
         "back": "Resect with the hemosiderin rim."
        },
        {
         "id": "c-gyxxx4",
         "front": "Sturge–Weber gene",
         "back": "Somatic GNAQ."
        },
        {
         "id": "c-5mi2mk",
         "front": "Seizure after the first week post-stroke",
         "back": "Unprovoked; high recurrence risk; usually treat."
        },
        {
         "id": "c-qqk6gw",
         "front": "Phenytoin for a week after severe head injury",
         "back": "Reduces early seizures only."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "ILAE FCD classification update (2022)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/35706131/"
      },
      {
       "title": "Phenytoin after head injury (Temkin 1990)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/2115976/"
      },
      {
       "title": "ILAE definition of acute symptomatic seizures (2010)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/19732133/"
      }
     ],
     "words": 614,
     "bank": 12
    },
    {
     "id": "immune-infectious-metabolic",
     "n": "8",
     "title": "Immune, infectious and metabolic causes",
     "tags": [
      "seizure-management",
      "ilae-classification"
     ],
     "updated": false,
     "goals": [
      "Distinguish acute symptomatic seizures in autoimmune encephalitis from autoimmune-associated epilepsy",
      "manage neurocysticercosis",
      "recognize the treatable metabolic epilepsies"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "Autoimmune"
      },
      {
       "type": "p",
       "text": "The ILAE separates two situations:"
      },
      {
       "type": "li",
       "text": "**Acute symptomatic seizures secondary to autoimmune encephalitis**: usually with antibodies to neuronal surface proteins (NMDA receptor, LGI1, CASPR2, GABA-B, AMPA receptor). They respond to immunotherapy, and seizures usually stop as the encephalitis settles."
      },
      {
       "type": "li",
       "text": "**Autoimmune-associated epilepsy**: usually with antibodies to intracellular antigens (GAD65, onconeural antibodies such as Hu). T-cell mediated, less responsive to immunotherapy, and more likely to persist."
      },
      {
       "type": "table",
       "head": [
        "Antibody",
        "Clinical signature"
       ],
       "rows": [
        [
         "NMDA receptor",
         "Young women and children; psychiatric onset, seizures, dyskinesias, autonomic instability, hypoventilation; ovarian teratoma; extreme delta brush; can follow herpes simplex encephalitis"
        ],
        [
         "LGI1",
         "Older adults; **faciobrachial dystonic seizures** (very brief, very frequent arm-and-face jerks); hyponatremia; memory decline"
        ],
        [
         "CASPR2",
         "Neuromyotonia, Morvan syndrome, limbic encephalitis"
        ],
        [
         "GABA-B receptor",
         "Severe refractory seizures or status; small-cell lung cancer"
        ],
        [
         "GAD65",
         "Temporal lobe epilepsy, stiff-person spectrum, type 1 diabetes; poor response to immunotherapy"
        ]
       ]
      },
      {
       "type": "p",
       "text": "Faciobrachial dystonic seizures respond far better to immunotherapy than to ASMs, and treating them early can prevent the cognitive decline that follows. First-line immunotherapy is corticosteroids, IVIG or plasma exchange; rituximab or cyclophosphamide is second-line. Always look for a tumor."
      },
      {
       "type": "h3",
       "text": "Infectious"
      },
      {
       "type": "p",
       "text": "**Neurocysticercosis** is a leading cause of acquired epilepsy worldwide. For viable parenchymal cysts, antiparasitic treatment (albendazole, adding praziquantel when there are more than two cysts) is given with corticosteroids and an ASM. Calcified cysts need no antiparasitic treatment. Examine the fundi before antiparasitic drugs. Other causes to remember: tuberculosis, cerebral malaria, HIV and its opportunistic infections, and the sequelae of viral encephalitis."
      },
      {
       "type": "h3",
       "text": "Metabolic and vitamin-responsive"
      },
      {
       "type": "table",
       "head": [
        "Disorder",
        "Clue",
        "Treatment"
       ],
       "rows": [
        [
         "Pyridoxine-dependent epilepsy (ALDH7A1)",
         "Refractory neonatal seizures; raised α-aminoadipic semialdehyde",
         "Pyridoxine for life, with lysine restriction"
        ],
        [
         "PNPO deficiency",
         "As above, with poor response to pyridoxine",
         "Pyridoxal 5′-phosphate"
        ],
        [
         "GLUT1 deficiency (SLC2A1)",
         "Early-onset absence, movement disorder, low CSF glucose relative to blood",
         "Ketogenic diet"
        ],
        [
         "Biotinidase deficiency",
         "Seizures, alopecia, rash, hearing loss",
         "Biotin"
        ],
        [
         "Creatine deficiency (GAMT)",
         "Absent creatine peak on MR spectroscopy",
         "Creatine (with ornithine)"
        ],
        [
         "Cerebral folate deficiency",
         "Low CSF 5-MTHF",
         "Folinic acid"
        ],
        [
         "POLG disease",
         "Refractory focal (often occipital) seizures and epilepsia partialis continua with liver involvement",
         "Avoid valproate"
        ],
        [
         "Nonketotic hyperglycinemia",
         "Neonatal encephalopathy, hiccups, burst-suppression; high CSF-to-plasma glycine",
         "Sodium benzoate and dextromethorphan (supportive)"
        ]
       ]
      },
      {
       "type": "case",
       "id": "case-lgi1",
       "title": "Brief jerks and memory loss at 64",
       "intro": [
        "A 64-year-old man has had dozens of daily episodes, each about two seconds long, in which his left arm stiffens and the left side of his face grimaces. Over two months his memory has declined. His sodium is 128 mmol/L. Routine EEG is normal."
       ],
       "steps": [
        {
         "narrative": [],
         "stem": "What is the most likely diagnosis?",
         "options": [
          "Functional movement disorder",
          "Focal motor seizures from a right frontal tumor",
          "LGI1 antibody encephalitis with faciobrachial dystonic seizures",
          "Creutzfeldt–Jakob disease",
          "Hemifacial spasm"
         ],
         "answer": 2,
         "explain": "Very brief, very frequent arm-and-face dystonic seizures (faciobrachial dystonic seizures), hyponatremia and subacute memory decline in an older man are characteristic of LGI1 antibody encephalitis. The EEG is often normal during them."
        },
        {
         "narrative": [],
         "stem": "Levetiracetam and then lacosamide have had little effect. What is the most important treatment?",
         "options": [
          "Add a third ASM",
          "Immunotherapy, starting with corticosteroids (or IVIG or plasma exchange)",
          "Epilepsy surgery",
          "Vagus nerve stimulation",
          "Ketogenic diet"
         ],
         "answer": 1,
         "explain": "Faciobrachial dystonic seizures respond much better to immunotherapy than to ASMs. Early immunotherapy can prevent progression to the full limbic encephalitis and permanent memory loss."
        }
       ],
       "outro": [
        "This is the classic acute symptomatic pattern of antibody-mediated encephalitis: seizures that ASMs barely touch, and that stop with immunotherapy."
       ]
      },
      {
       "type": "mcq",
       "id": "q-1mg6ts4",
       "stem": "Which antibody is most typical of autoimmune-associated epilepsy that responds poorly to immunotherapy?",
       "options": [
        "NMDA receptor",
        "LGI1",
        "GAD65",
        "CASPR2",
        "AMPA receptor"
       ],
       "answer": 2,
       "explain": "Antibodies to intracellular antigens such as GAD65 indicate a T-cell-mediated process that responds poorly to immunotherapy. Surface-antibody encephalitides usually respond and seizures often resolve."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-146ssq0",
         "front": "Surface versus intracellular antibodies",
         "back": "Surface (NMDAR, LGI1, CASPR2, GABA-B, AMPA): immunotherapy-responsive, seizures usually acute symptomatic. Intracellular (GAD65, Hu): T-cell mediated, poorer response."
        },
        {
         "id": "c-puy918",
         "front": "Faciobrachial dystonic seizures",
         "back": "LGI1 antibodies; respond to immunotherapy more than ASMs."
        },
        {
         "id": "c-1tuzz9b",
         "front": "NMDA receptor encephalitis tumor",
         "back": "Ovarian teratoma."
        },
        {
         "id": "c-hucijj",
         "front": "GABA-B receptor encephalitis tumor",
         "back": "Small-cell lung cancer."
        },
        {
         "id": "c-qalai",
         "front": "Neurocysticercosis with viable cysts",
         "back": "Albendazole (plus praziquantel if more than two cysts) with corticosteroids and an ASM."
        },
        {
         "id": "c-1hh4smp",
         "front": "Pyridoxine-dependent epilepsy gene and marker",
         "back": "ALDH7A1; raised α-aminoadipic semialdehyde."
        },
        {
         "id": "c-6afhjz",
         "front": "GLUT1 deficiency",
         "back": "Low CSF glucose relative to blood; ketogenic diet."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "ILAE: autoimmune encephalitis seizures and autoimmune-associated epilepsy (2020)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/32544279/"
      },
      {
       "title": "IDSA/ASTMH neurocysticercosis guideline (2018)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/29481580/"
      },
      {
       "title": "NSGC guideline on genetic testing (2022)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/36281494/"
      }
     ],
     "words": 616,
     "bank": 12
    }
   ],
   "intro": [
    "A syndrome diagnosis predicts the course, points to the right drugs and away from the wrong ones, and tells the family what to expect. The ILAE 2022 papers organize syndromes by age at onset. For quick lookups, use the [syndrome lookup on the ILAE page](ilae_classification.html#syndromes-tab)."
   ]
  },
  {
   "id": "treatment",
   "n": "7",
   "title": "Medical treatment",
   "short": "Treatment",
   "sections": [
    {
     "id": "treatment-principles",
     "n": "1",
     "title": "Starting, choosing and stopping",
     "tags": [
      "seizure-management"
     ],
     "updated": false,
     "goals": [
      "Choose a first ASM by seizure type, syndrome and person",
      "recognize drug resistance early",
      "counsel on withdrawal after remission"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "How often the first drug works"
      },
      {
       "type": "p",
       "text": "In a long-term cohort of newly treated epilepsy, about half of patients became seizure-free on the first ASM, roughly one in eight more on the second, and only a few percent on each regimen after that. The curve flattens fast. That is the reason to refer for specialist and surgical evaluation after two appropriate drugs have failed, rather than after five."
      },
      {
       "type": "h3",
       "text": "Choosing the first drug"
      },
      {
       "type": "li",
       "text": "**Match the drug to the seizure type and syndrome first.** Broad-spectrum drugs (valproate, lamotrigine, levetiracetam, and others) suit generalized or unclassified epilepsy. Sodium channel blockers suit focal epilepsy but can worsen absence and myoclonic seizures."
      },
      {
       "type": "li",
       "text": "**Then match it to the person**: the possibility of pregnancy, age, psychiatric and cognitive comorbidity, weight, bone health, kidney and liver function, other medicines, and cost."
      },
      {
       "type": "li",
       "text": "**Trial evidence (SANAD II)**: for focal epilepsy, lamotrigine remained the first choice; neither levetiracetam nor zonisamide did better. For generalized and unclassified epilepsy, valproate outperformed levetiracetam, so valproate remains the most effective option where it can be used safely."
      },
      {
       "type": "li",
       "text": "**Ethosuximide** is first choice for childhood absence epilepsy without other seizure types."
      },
      {
       "type": "h3",
       "text": "Dosing and combining"
      },
      {
       "type": "li",
       "text": "Start low, increase slowly, and aim for the lowest effective dose."
      },
      {
       "type": "li",
       "text": "If the first drug fails because of side effects or lack of effect, substituting a second monotherapy and adding a second drug are both reasonable. Combine drugs with different mechanisms; two sodium channel blockers together add side effects (dizziness, diplopia, ataxia)."
      },
      {
       "type": "li",
       "text": "Valproate plus lamotrigine is an effective combination, but valproate roughly doubles lamotrigine levels, so lamotrigine is started lower and titrated more slowly."
      },
      {
       "type": "li",
       "text": "Before calling a drug a failure, check adherence, the dose, and whether the events are epileptic at all."
      },
      {
       "type": "h3",
       "text": "Stopping after remission"
      },
      {
       "type": "p",
       "text": "Withdrawal can be considered after about two years seizure-free. Relapse is more likely with a longer epilepsy before remission, many seizures before control, adolescent onset (juvenile myoclonic epilepsy relapses in most), developmental delay, a structural cause, and epileptiform discharges on the EEG before withdrawal. Most relapses occur within the first year or two, and most patients regain control when the drug is restarted. Taper gradually, and discuss driving during and after the taper."
      },
      {
       "type": "mcq",
       "id": "q-1wxpjbw",
       "stem": "A 30-year-old man with focal epilepsy has seizures despite lamotrigine at a good dose. Levetiracetam was stopped after two weeks at 250 mg twice daily because of irritability. How many adequate trials has he failed?",
       "options": [
        "One",
        "Two",
        "Three",
        "None",
        "It cannot be determined without drug levels"
       ],
       "answer": 0,
       "explain": "Only lamotrigine was an adequate, tolerated trial. Levetiracetam was stopped early at a low dose for side effects and does not count as a failed trial for defining drug resistance."
      },
      {
       "type": "mcq",
       "id": "q-ihki5w",
       "stem": "According to SANAD II, which drug remains the most effective first choice for a man with newly diagnosed generalized epilepsy?",
       "options": [
        "Levetiracetam",
        "Lamotrigine",
        "Valproate",
        "Zonisamide",
        "Carbamazepine"
       ],
       "answer": 2,
       "explain": "In SANAD II, levetiracetam was not non-inferior to valproate for generalized and unclassified epilepsy. Valproate remains first choice where teratogenicity is not a concern."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-1po5up4",
         "front": "Seizure freedom with the first, second and later ASMs",
         "back": "About half with the first, about one in eight more with the second, a few percent with each later regimen."
        },
        {
         "id": "c-1c2oeun",
         "front": "When to refer for surgical evaluation",
         "back": "After two appropriate, adequate ASM trials have failed."
        },
        {
         "id": "c-1b3kfck",
         "front": "SANAD II focal epilepsy",
         "back": "Lamotrigine remains first choice; levetiracetam and zonisamide did no better."
        },
        {
         "id": "c-eew3n5",
         "front": "SANAD II generalized epilepsy",
         "back": "Valproate outperformed levetiracetam."
        },
        {
         "id": "c-oeg19p",
         "front": "Valproate and lamotrigine",
         "back": "Valproate roughly doubles lamotrigine levels; start lamotrigine lower and titrate slowly."
        },
        {
         "id": "c-au1qrc",
         "front": "When to consider withdrawal",
         "back": "After about two years seizure-free."
        },
        {
         "id": "c-12n8838",
         "front": "Syndrome with a very high relapse rate after withdrawal",
         "back": "Juvenile myoclonic epilepsy."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "SANAD II, focal epilepsy (2021)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/33838757/"
      },
      {
       "title": "SANAD II, generalized and unclassifiable epilepsy (2021)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/33838758/"
      },
      {
       "title": "Treatment outcomes over 30 years (Chen 2018)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/29279892/"
      },
      {
       "title": "Early identification of refractory epilepsy (Kwan and Brodie 2000)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/10660394/"
      }
     ],
     "words": 578,
     "bank": 8
    },
    {
     "id": "asm-pharmacology",
     "n": "2",
     "title": "How ASMs are handled by the body",
     "tags": [
      "seizure-management"
     ],
     "updated": false,
     "goals": [
      "Predict interactions from enzyme induction and inhibition",
      "recognize the drugs with unusual kinetics",
      "decide when a level is useful"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "Enzyme inducers"
      },
      {
       "type": "p",
       "text": "Carbamazepine, phenytoin, phenobarbital and primidone are strong inducers. They lower levels of many other drugs: hormonal contraceptives, warfarin and the direct oral anticoagulants, statins, some chemotherapy, antiretrovirals, and transplant immunosuppressants. Weaker or dose-dependent inducers include oxcarbazepine, eslicarbazepine, topiramate (above about 200 mg/day), perampanel (at 12 mg/day), rufinamide, felbamate, cenobamate and clobazam."
      },
      {
       "type": "h3",
       "text": "Enzyme inhibitors"
      },
      {
       "type": "li",
       "text": "**Valproate** inhibits glucuronidation and CYP2C9: lamotrigine levels roughly double, phenobarbital rises, and phenytoin is displaced from albumin."
      },
      {
       "type": "li",
       "text": "**Felbamate, stiripentol and cenobamate** raise levels of several other ASMs; cenobamate raises phenytoin and phenobarbital."
      },
      {
       "type": "li",
       "text": "**Cannabidiol and stiripentol** raise N-desmethylclobazam, the active metabolite of clobazam, causing sedation."
      },
      {
       "type": "h3",
       "text": "Drugs with unusual kinetics"
      },
      {
       "type": "li",
       "text": "**Phenytoin** has saturable (Michaelis–Menten) kinetics: above the saturation point, small dose increases cause large rises in level. It is about 90% protein-bound, so check a free level in low albumin, uremia, late pregnancy, or with valproate."
      },
      {
       "type": "li",
       "text": "**Carbamazepine** induces its own metabolism over the first weeks, so the level falls on a steady dose."
      },
      {
       "type": "li",
       "text": "**Lamotrigine** clearance rises with estrogen and pregnancy and falls with valproate."
      },
      {
       "type": "figure",
       "html": "phenytoin-model",
       "caption": "Phenytoin steady-state level against daily dose, from the Michaelis–Menten relation: level = Km × dose ÷ (Vmax − dose). The starting values reproduce the case below, where 300 mg/day gives 12 mcg/mL and 400 mg/day gives 32. Vmax and Km vary widely between people, so real doses change in small steps with levels.",
       "n": 8
      },
      {
       "type": "h3",
       "text": "Kidney and liver"
      },
      {
       "type": "li",
       "text": "**Mainly renal**: levetiracetam, gabapentin, pregabalin, vigabatrin, eslicarbazepine, and partly lacosamide and topiramate. Reduce doses in kidney failure, and give a supplement after hemodialysis for the dialysable drugs (levetiracetam, gabapentin, pregabalin, lacosamide, topiramate)."
      },
      {
       "type": "li",
       "text": "**Mainly hepatic**: valproate, carbamazepine, phenytoin, phenobarbital, lamotrigine, perampanel, cenobamate, benzodiazepines. In liver failure favor renally cleared drugs and avoid valproate and felbamate."
      },
      {
       "type": "h3",
       "text": "When to check a level"
      },
      {
       "type": "p",
       "text": "Levels help to confirm adherence, investigate toxicity, guide phenytoin dosing, establish a baseline before pregnancy and follow changes during it, manage kidney or liver disease, and sort out interactions. In a stable patient who is seizure-free without side effects, a routine level changes nothing."
      },
      {
       "type": "mcq",
       "id": "q-199l6ax",
       "stem": "A woman on warfarin and atorvastatin is started on carbamazepine. Two weeks later her INR is 1.3 (previously 2.5) and her LDL has risen. What explains this?",
       "options": [
        "Carbamazepine inhibits CYP2C9",
        "Carbamazepine induces hepatic enzymes, lowering warfarin and atorvastatin levels",
        "Carbamazepine displaces warfarin from albumin",
        "She has stopped taking warfarin",
        "Carbamazepine causes vitamin K deficiency"
       ],
       "answer": 1,
       "explain": "Carbamazepine is a strong inducer of CYP3A4 and other enzymes and lowers levels of warfarin, statins, hormonal contraceptives and many other drugs. A non-inducing ASM avoids the problem."
      },
      {
       "type": "mcq",
       "id": "q-kuqgmv",
       "stem": "A man on phenytoin 300 mg daily has a level of 12 mcg/mL. His dose is raised to 400 mg daily. Two weeks later he has nystagmus and ataxia with a level of 32 mcg/mL. Why did the level rise so much?",
       "options": [
        "Phenytoin induces its own metabolism",
        "Phenytoin is renally cleared",
        "Phenytoin metabolism is saturable, so small dose increases can cause large rises in level",
        "The laboratory measured the free level",
        "He has developed hypoalbuminemia"
       ],
       "answer": 2,
       "explain": "Phenytoin follows Michaelis–Menten kinetics. Once metabolism nears saturation, levels rise disproportionately with dose. Increase by small steps at higher doses."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-u1dxki",
         "front": "Strong enzyme-inducing ASMs",
         "back": "Carbamazepine, phenytoin, phenobarbital, primidone."
        },
        {
         "id": "c-1ez8ryi",
         "front": "Valproate interactions",
         "back": "Doubles lamotrigine, raises phenobarbital, displaces phenytoin."
        },
        {
         "id": "c-nkoz6d",
         "front": "Phenytoin kinetics",
         "back": "Saturable (Michaelis–Menten); about 90% protein-bound."
        },
        {
         "id": "c-yn6oxk",
         "front": "When to measure free phenytoin",
         "back": "Low albumin, uremia, late pregnancy, co-therapy with valproate."
        },
        {
         "id": "c-dm5vz3",
         "front": "Carbamazepine autoinduction",
         "back": "Levels fall over the first weeks on a steady dose."
        },
        {
         "id": "c-10043k",
         "front": "Mainly renally cleared ASMs",
         "back": "Levetiracetam, gabapentin, pregabalin, vigabatrin, eslicarbazepine (partly lacosamide, topiramate)."
        },
        {
         "id": "c-1sld877",
         "front": "Supplemental dose after hemodialysis",
         "back": "Levetiracetam, gabapentin, pregabalin, lacosamide, topiramate."
        },
        {
         "id": "c-1gd7z1c",
         "front": "Drugs raising N-desmethylclobazam",
         "back": "Cannabidiol, stiripentol (and cenobamate)."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "SANAD II, focal epilepsy (2021)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/33838757/"
      },
      {
       "title": "Cenobamate in focal epilepsy (2020)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/31734103/"
      }
     ],
     "words": 578,
     "bank": 10
    },
    {
     "id": "asm-profiles",
     "n": "3",
     "title": "ASM profiles and choosing for a patient",
     "tags": [
      "seizure-management"
     ],
     "updated": true,
     "goals": [
      "Recall the spectrum and signature adverse effects of each common ASM",
      "choose a drug for a specific patient"
     ],
     "blocks": [
      {
       "type": "table",
       "head": [
        "Drug",
        "Main action",
        "Spectrum",
        "Know it for"
       ],
       "rows": [
        [
         "Carbamazepine",
         "Sodium channel",
         "Focal",
         "Strong inducer; hyponatremia; HLA-B*15:02 and SJS; worsens absence and myoclonus"
        ],
        [
         "Oxcarbazepine",
         "Sodium channel",
         "Focal",
         "More hyponatremia than carbamazepine; weaker inducer"
        ],
        [
         "Eslicarbazepine",
         "Sodium channel",
         "Focal",
         "Once daily; hyponatremia"
        ],
        [
         "Phenytoin",
         "Sodium channel",
         "Focal, status",
         "Saturable kinetics; gum hypertrophy, coarse features, neuropathy, cerebellar atrophy"
        ],
        [
         "Lamotrigine",
         "Sodium channel and more",
         "Broad",
         "Rash with fast titration or valproate; lowered by estrogen; can worsen myoclonus"
        ],
        [
         "Lacosamide",
         "Slow sodium inactivation",
         "Focal",
         "PR prolongation; IV available; few interactions"
        ],
        [
         "Valproate",
         "Several",
         "Broad",
         "Most effective for generalized epilepsy; teratogenic; liver, pancreas, platelets, ammonia, weight"
        ],
        [
         "Levetiracetam",
         "SV2A",
         "Broad",
         "Few interactions; IV; irritability, depression"
        ],
        [
         "Brivaracetam",
         "SV2A",
         "Focal",
         "Fewer behavioral effects than levetiracetam"
        ],
        [
         "Topiramate",
         "Several",
         "Broad",
         "Word-finding difficulty, stones, acidosis, glaucoma, weight loss; teratogenic"
        ],
        [
         "Zonisamide",
         "Several",
         "Broad",
         "Sulfonamide; stones, oligohidrosis, weight loss"
        ],
        [
         "Ethosuximide",
         "T-type calcium",
         "Absence only",
         "First choice in childhood absence epilepsy"
        ],
        [
         "Clobazam",
         "GABA-A",
         "Broad",
         "Lennox–Gastaut; tolerance; raised by cannabidiol and stiripentol"
        ],
        [
         "Phenobarbital",
         "GABA-A",
         "Broad",
         "Neonatal first-line; sedation; strong inducer; connective tissue effects"
        ],
        [
         "Gabapentin, pregabalin",
         "α2δ",
         "Focal (weak)",
         "Renal; weight gain; can worsen myoclonus"
        ],
        [
         "Vigabatrin",
         "GABA transaminase",
         "Spasms, focal",
         "Permanent peripheral visual field loss"
        ],
        [
         "Perampanel",
         "AMPA",
         "Broad",
         "Boxed warning for aggression and other psychiatric effects; long half-life"
        ],
        [
         "Cenobamate",
         "Sodium current and GABA-A",
         "Focal",
         "High seizure-freedom rates; slow titration to prevent DRESS; shortens QT"
        ],
        [
         "Rufinamide",
         "Sodium channel",
         "Lennox–Gastaut",
         "Shortens QT; avoid in familial short QT"
        ],
        [
         "Felbamate",
         "NMDA and GABA-A",
         "Broad (refractory)",
         "Aplastic anemia and liver failure"
        ],
        [
         "Cannabidiol",
         "Several",
         "Dravet, Lennox–Gastaut, TSC",
         "Raised liver enzymes (especially with valproate); raises clobazam metabolite"
        ],
        [
         "Fenfluramine",
         "Serotonergic, sigma-1",
         "Dravet, Lennox–Gastaut",
         "Echocardiographic monitoring for valve disease and pulmonary hypertension"
        ],
        [
         "Stiripentol",
         "GABA-A, enzyme inhibition",
         "Dravet",
         "Used with clobazam; raises clobazam metabolite"
        ],
        [
         "Everolimus",
         "mTOR inhibitor",
         "TSC focal seizures",
         "Targets the cause in tuberous sclerosis"
        ],
        [
         "Ganaxolone",
         "Neurosteroid GABA-A",
         "CDKL5 deficiency",
         "Approved for CDKL5 deficiency disorder"
        ]
       ]
      },
      {
       "type": "figure",
       "html": "asm-targets",
       "caption": "Where the drugs in the table act. Choose a target to see its drugs, or a drug to see its target.",
       "n": 9
      },
      {
       "type": "calc",
       "name": "asm-chooser"
      },
      {
       "type": "p",
       "updated": true,
       "label": "Ezogabine",
       "text": "Ezogabine (retigabine), a potassium channel (Kv7) opener, was withdrawn worldwide in 2017 because of retinal pigment changes and blue skin discoloration. Newer Kv7 openers are in trials."
      },
      {
       "type": "mcq",
       "id": "q-1m9shrg",
       "stem": "A 68-year-old man with new focal epilepsy after a stroke takes apixaban, atorvastatin and sertraline. Which ASM is the most appropriate first choice?",
       "options": [
        "Carbamazepine",
        "Phenytoin",
        "Phenobarbital",
        "Levetiracetam or lamotrigine",
        "Valproate"
       ],
       "answer": 3,
       "explain": "In older adults, lamotrigine and levetiracetam are well tolerated and lack enzyme induction. Carbamazepine, phenytoin and phenobarbital lower apixaban and statin levels and harm bone health; phenytoin also has difficult kinetics."
      },
      {
       "type": "mcq",
       "id": "q-18lyrvs",
       "stem": "Which ASM requires baseline and periodic perimetry because of permanent peripheral visual field loss?",
       "options": [
        "Topiramate",
        "Ethosuximide",
        "Vigabatrin",
        "Lacosamide",
        "Zonisamide"
       ],
       "answer": 2,
       "explain": "Vigabatrin causes irreversible concentric peripheral visual field constriction related to dose and duration. Topiramate's eye risk is acute angle-closure glaucoma, which is reversible if treated promptly."
      },
      {
       "type": "match",
       "id": "match-asm-effects",
       "prompt": "Match each ASM to its signature adverse effect",
       "pairs": [
        [
         "Vigabatrin",
         "Permanent peripheral visual field loss"
        ],
        [
         "Felbamate",
         "Aplastic anemia and liver failure"
        ],
        [
         "Topiramate",
         "Acute angle-closure glaucoma and kidney stones"
        ],
        [
         "Lacosamide",
         "PR interval prolongation"
        ],
        [
         "Rufinamide",
         "QT shortening"
        ],
        [
         "Perampanel",
         "Aggression and psychiatric effects"
        ],
        [
         "Oxcarbazepine",
         "Hyponatremia"
        ]
       ]
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-1vybybi",
         "front": "Ethosuximide spectrum",
         "back": "Absence seizures only."
        },
        {
         "id": "c-319orr",
         "front": "Lamotrigine rash risk factors",
         "back": "Fast titration, valproate co-therapy, children."
        },
        {
         "id": "c-1mdnx1f",
         "front": "Cenobamate DRESS prevention",
         "back": "Slow titration from a low starting dose."
        },
        {
         "id": "c-1zjlwx",
         "front": "Drugs contraindicated in familial short QT",
         "back": "Rufinamide and cenobamate."
        },
        {
         "id": "c-a1gdbl",
         "front": "Fenfluramine monitoring",
         "back": "Echocardiography for valve disease and pulmonary hypertension."
        },
        {
         "id": "c-d6fwrc",
         "front": "Perampanel boxed warning",
         "back": "Serious psychiatric and behavioral reactions, including aggression."
        },
        {
         "id": "c-1ct9v58",
         "front": "Everolimus in epilepsy",
         "back": "Refractory focal seizures in tuberous sclerosis."
        },
        {
         "id": "c-i2t8ab",
         "front": "Ezogabine",
         "back": "Kv7 opener withdrawn in 2017 (retinal pigment changes, blue skin discoloration)."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "Cenobamate in focal epilepsy (2020)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/31734103/"
      },
      {
       "title": "Fenfluramine in Dravet syndrome (2019)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/31862249/"
      },
      {
       "title": "SANAD II, focal epilepsy (2021)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/33838757/"
      }
     ],
     "words": 563,
     "bank": 12
    },
    {
     "id": "asm-safety",
     "n": "4",
     "title": "Serious adverse effects and pharmacogenomics",
     "tags": [
      "seizure-management"
     ],
     "updated": false,
     "goals": [
      "Screen for HLA risk alleles before aromatic ASMs",
      "recognize the idiosyncratic reactions that need the drug stopped",
      "know which ASMs can aggravate seizures"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "Pharmacogenomics"
      },
      {
       "type": "li",
       "text": "**HLA-B*15:02** (common in people of Han Chinese and Southeast Asian ancestry) greatly increases the risk of Stevens–Johnson syndrome and toxic epidermal necrolysis with carbamazepine; test before starting it in at-risk ancestry. Phenytoin, fosphenytoin and oxcarbazepine carry a smaller association."
      },
      {
       "type": "li",
       "text": "**HLA-A*31:01** (found in European, Japanese and Korean populations) is associated with carbamazepine hypersensitivity, including DRESS and milder rashes."
      },
      {
       "type": "li",
       "text": "**CYP2C9 poor metabolizers** accumulate phenytoin and have more toxicity and severe skin reactions."
      },
      {
       "type": "h3",
       "text": "Reactions that mean stopping the drug"
      },
      {
       "type": "li",
       "text": "**Severe cutaneous reactions** (SJS/TEN, DRESS): mostly in the first two months, most often with aromatic drugs (carbamazepine, oxcarbazepine, phenytoin, phenobarbital, lamotrigine). Cross-reactivity among aromatic drugs is common."
      },
      {
       "type": "li",
       "text": "**Valproate**: hepatotoxicity (highest risk in children under two on several drugs, and in mitochondrial disease such as POLG), pancreatitis, and hyperammonemic encephalopathy, even with normal liver tests."
      },
      {
       "type": "li",
       "text": "**Felbamate**: aplastic anemia and liver failure."
      },
      {
       "type": "li",
       "text": "**Carbamazepine**: agranulocytosis and aplastic anemia (rare); mild leukopenia is common and usually harmless."
      },
      {
       "type": "li",
       "text": "**Topiramate**: acute angle-closure glaucoma, usually in the first month."
      },
      {
       "type": "h3",
       "text": "Seizure aggravation"
      },
      {
       "type": "li",
       "text": "Sodium channel blockers (carbamazepine, oxcarbazepine, phenytoin, sometimes lamotrigine) can worsen absence and myoclonic seizures and Dravet syndrome."
      },
      {
       "type": "li",
       "text": "Vigabatrin and tiagabine can worsen absence seizures."
      },
      {
       "type": "li",
       "text": "Gabapentin and pregabalin can worsen myoclonus."
      },
      {
       "type": "li",
       "text": "Benzodiazepines can trigger tonic status in Lennox–Gastaut syndrome."
      },
      {
       "type": "li",
       "text": "Toxic levels of phenytoin or carbamazepine can themselves cause seizures."
      },
      {
       "type": "h3",
       "text": "Mood, suicidality and bone"
      },
      {
       "type": "p",
       "text": "All ASMs carry an FDA warning about suicidal thoughts and behavior, based on pooled trial data. Screen for depression and ask about suicidal thinking. Levetiracetam, perampanel, topiramate and phenobarbital are the drugs most often linked to mood problems; lamotrigine can stabilize mood. Enzyme inducers and valproate reduce bone density; supplement vitamin D and calcium in long-term treatment."
      },
      {
       "type": "mcq",
       "id": "q-hjuwkz",
       "stem": "A 29-year-old man of Han Chinese ancestry has trigeminal neuralgia and focal seizures. Before starting carbamazepine, which test is recommended?",
       "options": [
        "HLA-A*31:01",
        "HLA-B*15:02",
        "CYP2D6 genotyping",
        "TPMT activity",
        "G6PD level"
       ],
       "answer": 1,
       "explain": "HLA-B*15:02, common in Han Chinese and Southeast Asian populations, strongly predicts carbamazepine-induced SJS/TEN. Screening is recommended before starting carbamazepine in at-risk ancestry."
      },
      {
       "type": "mcq",
       "id": "q-vukae0",
       "stem": "A 23-year-old on valproate and topiramate becomes confused and lethargic. Liver tests are normal. What test is most likely to reveal the cause?",
       "options": [
        "Serum sodium",
        "Plasma ammonia",
        "Serum amylase",
        "Platelet count",
        "Creatine kinase"
       ],
       "answer": 1,
       "explain": "Valproate can cause hyperammonemic encephalopathy with normal liver tests, and topiramate increases the risk. Check ammonia; carnitine supplementation is sometimes used."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-10pswc7",
         "front": "HLA-B*15:02",
         "back": "Carbamazepine SJS/TEN risk; Han Chinese and Southeast Asian ancestry; test before starting."
        },
        {
         "id": "c-18gosgh",
         "front": "HLA-A*31:01",
         "back": "Carbamazepine hypersensitivity (DRESS, rash) in European, Japanese and Korean populations."
        },
        {
         "id": "c-15yyxac",
         "front": "CYP2C9 poor metabolizers",
         "back": "Phenytoin accumulation and toxicity."
        },
        {
         "id": "c-wcb129",
         "front": "When severe skin reactions appear",
         "back": "Mostly within the first two months."
        },
        {
         "id": "c-r84akx",
         "front": "Valproate hyperammonemia",
         "back": "Encephalopathy even with normal liver tests; more likely with topiramate."
        },
        {
         "id": "c-1otd22f",
         "front": "Highest risk of valproate hepatotoxicity",
         "back": "Children under two on polytherapy; mitochondrial disease (POLG)."
        },
        {
         "id": "c-vc36ne",
         "front": "Benzodiazepines in Lennox–Gastaut",
         "back": "Can provoke tonic status."
        },
        {
         "id": "c-erpabh",
         "front": "FDA ASM class warning",
         "back": "Suicidal thoughts and behavior."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "SANAD II, focal epilepsy (2021)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/33838757/"
      },
      {
       "title": "NSGC guideline on genetic testing (2022)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/36281494/"
      },
      {
       "title": "Screening for depression in epilepsy, NDDI-E (2006)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/16632310/"
      }
     ],
     "words": 456,
     "bank": 11
    },
    {
     "id": "women",
     "n": "5",
     "title": "Women with epilepsy",
     "tags": [
      "seizure-management"
     ],
     "updated": true,
     "goals": [
      "Plan contraception around enzyme induction",
      "choose ASMs before and during pregnancy",
      "manage levels, delivery, breastfeeding and catamenial seizures"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "Contraception"
      },
      {
       "type": "li",
       "text": "Enzyme-inducing ASMs make combined pills, progestogen-only pills, implants and patches less reliable. Intrauterine devices (copper or levonorgestrel) are unaffected and are usually the best option."
      },
      {
       "type": "li",
       "text": "Lamotrigine is the two-way exception. It does not weaken contraception, but estrogen roughly halves lamotrigine levels. Seizures can break through during active-pill weeks, and toxicity can appear in the pill-free week."
      },
      {
       "type": "h3",
       "text": "Pregnancy"
      },
      {
       "type": "p",
       "updated": true,
       "label": "AAN/AES/SMFM 2024",
       "text": "The guideline says to consider lamotrigine, levetiracetam or oxcarbazepine for people with epilepsy who could become pregnant, to avoid valproate because of malformations, neural tube defects and effects on neurodevelopment (including autism and lower IQ), and to avoid valproate and topiramate to reduce the risk of babies born small for gestational age."
      },
      {
       "type": "p",
       "text": "Major malformation rates on monotherapy in the EURAP registry:"
      },
      {
       "type": "table",
       "head": [
        "ASM",
        "Major malformations"
       ],
       "rows": [
        [
         "Valproate",
         "10.3% (dose-dependent)"
        ],
        [
         "Phenobarbital",
         "6.5%"
        ],
        [
         "Phenytoin",
         "6.4%"
        ],
        [
         "Carbamazepine",
         "5.5%"
        ],
        [
         "Topiramate",
         "3.9%"
        ],
        [
         "Oxcarbazepine",
         "3.0%"
        ],
        [
         "Lamotrigine",
         "2.9%"
        ],
        [
         "Levetiracetam",
         "2.8%"
        ]
       ]
      },
      {
       "type": "figure",
       "html": "mcm-risk",
       "caption": "The EURAP rates with 95% intervals computed from the counts (for example, 142 malformations in 1,381 pregnancies on valproate and 17 in 599 on levetiracetam).",
       "n": 10
      },
      {
       "type": "li",
       "text": "**Folic acid**: at least 0.4 mg daily before conception and through pregnancy for anyone on an ASM, to reduce neural tube defects and possibly improve neurodevelopment."
      },
      {
       "type": "li",
       "text": "**Levels**: clearance rises in pregnancy, most of all for lamotrigine and levetiracetam. Take a baseline level before conception, monitor through pregnancy, and reduce the dose again over the first weeks after delivery to avoid toxicity."
      },
      {
       "type": "li",
       "text": "**Seizure control before pregnancy** predicts control during it. Convulsive seizures are the main danger to mother and baby; do not stop an effective ASM abruptly because of pregnancy."
      },
      {
       "type": "li",
       "text": "**Breastfeeding** is encouraged. Watch babies exposed to phenobarbital, primidone or benzodiazepines for sedation and poor feeding."
      },
      {
       "type": "p",
       "updated": true,
       "label": "Valproate precautions",
       "text": "European regulators run a pregnancy prevention program for valproate and, since 2023, for topiramate. In 2024 the EMA also advised precautions for men taking valproate around conception because of a possible neurodevelopmental risk to their children."
      },
      {
       "type": "h3",
       "text": "Catamenial epilepsy"
      },
      {
       "type": "p",
       "text": "Seizures that at least double in a particular phase of the cycle:"
      },
      {
       "type": "li",
       "text": "**C1, perimenstrual**: falling progesterone and its neurosteroid metabolite allopregnanolone (a GABA-A modulator). The commonest pattern."
      },
      {
       "type": "li",
       "text": "**C2, periovulatory**: the estrogen surge, unopposed."
      },
      {
       "type": "li",
       "text": "**C3, luteal**: an inadequate luteal phase in anovulatory cycles."
      },
      {
       "type": "p",
       "text": "Cyclic clobazam or acetazolamide around the vulnerable days are used. A large trial of progesterone was negative overall, with benefit only in women with a marked perimenstrual increase."
      },
      {
       "type": "case",
       "id": "case-pregnancy",
       "title": "Planning a pregnancy on valproate",
       "intro": [
        "A 27-year-old with juvenile myoclonic epilepsy has been seizure-free for three years on valproate 1,500 mg daily. She tells you she would like to become pregnant next year."
       ],
       "steps": [
        {
         "narrative": [],
         "stem": "What is the most important message about her current medication?",
         "options": [
          "Valproate is safe at this dose",
          "Valproate carries the highest risk of malformations and neurodevelopmental harm, and switching should be planned before conception",
          "She should stop valproate immediately",
          "All ASMs carry the same risk",
          "High-dose folic acid removes valproate's risk"
         ],
         "answer": 1,
         "explain": "Valproate has the highest rate of major malformations (about 10% overall, higher at larger doses) and lowers IQ and increases autism risk in exposed children. Folic acid does not neutralize this. Stopping abruptly risks convulsive seizures; the switch should be planned."
        },
        {
         "narrative": [],
         "stem": "Which drug is the most reasonable replacement for her syndrome?",
         "options": [
          "Carbamazepine",
          "Topiramate",
          "Levetiracetam (or lamotrigine)",
          "Phenytoin",
          "Gabapentin"
         ],
         "answer": 2,
         "explain": "Levetiracetam and lamotrigine have the lowest malformation rates and work in juvenile myoclonic epilepsy (lamotrigine can sometimes worsen myoclonus). Carbamazepine and phenytoin worsen myoclonus; topiramate has a higher risk of small-for-gestational-age babies and malformations."
        },
        {
         "narrative": [
          "She switches to levetiracetam and remains seizure-free. She becomes pregnant six months later."
         ],
         "stem": "What should happen with her levetiracetam during pregnancy?",
         "options": [
          "Nothing; levels do not change in pregnancy",
          "Monitor levels against her pre-pregnancy baseline and increase the dose as clearance rises",
          "Stop it in the first trimester to protect the fetus",
          "Switch back to valproate after the first trimester",
          "Halve the dose in the third trimester"
         ],
         "answer": 1,
         "explain": "Levetiracetam clearance rises substantially in pregnancy, as lamotrigine's does. Compare levels with the preconception baseline, adjust the dose, and reduce it again after delivery."
        }
       ],
       "outro": [
        "Every person with epilepsy who could become pregnant needs a plan: effective contraception, the lowest-risk effective drug before conception, folic acid, and a monitoring schedule."
       ]
      },
      {
       "type": "mcq",
       "id": "q-4h2ois",
       "stem": "A woman stable for years on lamotrigine has breakthrough seizures during the active weeks of a newly started combined oral contraceptive. Why?",
       "options": [
        "Lamotrigine induces metabolism of the contraceptive",
        "Ethinylestradiol induces lamotrigine glucuronidation, roughly halving levels",
        "Progestogens lower seizure threshold",
        "The contraceptive displaces lamotrigine from albumin",
        "Lamotrigine interacts only with progestogen-only pills"
       ],
       "answer": 1,
       "explain": "Estrogen roughly halves lamotrigine levels. Levels recover in the pill-free week, sometimes causing toxicity. An intrauterine device avoids the interaction."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-1w6odfm",
         "front": "Best contraception with enzyme-inducing ASMs",
         "back": "Intrauterine device (copper or levonorgestrel)."
        },
        {
         "id": "c-lgk4ai",
         "front": "Estrogen and lamotrigine",
         "back": "Estrogen roughly halves lamotrigine levels."
        },
        {
         "id": "c-8os0hn",
         "front": "Lowest malformation rates in EURAP",
         "back": "Levetiracetam 2.8%, lamotrigine 2.9%, oxcarbazepine 3.0%."
        },
        {
         "id": "c-1u38cwd",
         "front": "Valproate major malformation rate",
         "back": "10.3%, dose-dependent."
        },
        {
         "id": "c-17pgjx0",
         "front": "Folic acid on an ASM",
         "back": "At least 0.4 mg daily before conception and through pregnancy."
        },
        {
         "id": "c-1qbqacs",
         "front": "ASMs whose clearance rises most in pregnancy",
         "back": "Lamotrigine and levetiracetam."
        },
        {
         "id": "c-3gx2yr",
         "front": "After delivery",
         "back": "Reduce doses back toward pre-pregnancy levels over the first weeks."
        },
        {
         "id": "c-1y7qgfo",
         "front": "C1 catamenial pattern",
         "back": "Perimenstrual; withdrawal of progesterone and allopregnanolone."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "AAN/AES/SMFM guideline on ASMs in pregnancy (2024)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/38748979/"
      },
      {
       "title": "EURAP registry, eight ASMs (2018)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/29680205/"
      }
     ],
     "words": 731,
     "bank": 12
    },
    {
     "id": "special-populations",
     "n": "6",
     "title": "Older adults and other special groups",
     "tags": [
      "seizure-management"
     ],
     "updated": false,
     "goals": [
      "Adjust ASM choice for older adults, organ failure and psychiatric comorbidity",
      "avoid the interactions that matter most"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "Older adults"
      },
      {
       "type": "p",
       "text": "New-onset epilepsy is commonest after 60, most often from cerebrovascular disease; neurodegeneration and tumors follow. Seizures may present as confusion or falls. Start low and go slow. Lamotrigine and levetiracetam are well tolerated. Avoid strong inducers (bone loss, lipids, and interactions with anticoagulants and statins) and phenytoin. Watch for hyponatremia with oxcarbazepine and carbamazepine, and for cognitive effects with topiramate and phenobarbital."
      },
      {
       "type": "h3",
       "text": "Anticoagulants, transplant and HIV medicines"
      },
      {
       "type": "p",
       "text": "Strong inducers (carbamazepine, phenytoin, phenobarbital) substantially lower direct oral anticoagulant, tacrolimus, cyclosporine and many antiretroviral levels. Prefer drugs without induction."
      },
      {
       "type": "h3",
       "text": "Kidney and liver failure"
      },
      {
       "type": "p",
       "text": "Reduce renally cleared drugs as filtration falls and supplement after dialysis. In liver failure, prefer renally cleared drugs; avoid valproate and felbamate."
      },
      {
       "type": "h3",
       "text": "Psychiatric comorbidity"
      },
      {
       "type": "p",
       "text": "Depression affects about a third of people with epilepsy and is the strongest driver of poor quality of life. Screen with a validated tool such as the NDDI-E. SSRIs (sertraline, escitalopram) are safe at usual doses. Bupropion lowers seizure threshold in a dose-dependent way, as do clomipramine and maprotiline; avoid them. Choose ASMs with mood in mind."
      },
      {
       "type": "mcq",
       "id": "q-178np7j",
       "stem": "A 45-year-old kidney transplant recipient on tacrolimus develops focal seizures. Which ASM is most likely to cause rejection by lowering tacrolimus levels?",
       "options": [
        "Levetiracetam",
        "Lacosamide",
        "Carbamazepine",
        "Gabapentin",
        "Brivaracetam"
       ],
       "answer": 2,
       "explain": "Carbamazepine induces CYP3A4 and lowers tacrolimus levels. Levetiracetam and lacosamide have few interactions and are usually preferred after transplantation, with dosing adjusted to kidney function."
      },
      {
       "type": "mcq",
       "id": "q-xmssrp",
       "stem": "Which antidepressant should be avoided in a patient with poorly controlled epilepsy because of dose-dependent seizure risk?",
       "options": [
        "Sertraline",
        "Escitalopram",
        "Bupropion",
        "Mirtazapine",
        "Fluoxetine"
       ],
       "answer": 2,
       "explain": "Bupropion lowers seizure threshold in a dose-dependent way. SSRIs are safe at therapeutic doses and are first-line for depression in epilepsy."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-hg6ozh",
         "front": "Commonest cause of new epilepsy after 60",
         "back": "Cerebrovascular disease."
        },
        {
         "id": "c-1xm1058",
         "front": "Preferred ASMs in older adults",
         "back": "Lamotrigine and levetiracetam."
        },
        {
         "id": "c-8pvyxy",
         "front": "Strong inducers and DOACs",
         "back": "Carbamazepine, phenytoin and phenobarbital lower DOAC levels; avoid the combination."
        },
        {
         "id": "c-1db6b2v",
         "front": "ASM choice after organ transplant",
         "back": "A non-inducing drug such as levetiracetam or lacosamide."
        },
        {
         "id": "c-oiwg31",
         "front": "Depression screening tool in epilepsy",
         "back": "NDDI-E."
        },
        {
         "id": "c-bsud8l",
         "front": "Antidepressants to avoid",
         "back": "Bupropion, clomipramine, maprotiline."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "Screening for depression in epilepsy, NDDI-E (2006)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/16632310/"
      },
      {
       "title": "SANAD II, focal epilepsy (2021)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/33838757/"
      }
     ],
     "words": 325,
     "bank": 7
    },
    {
     "id": "status-epilepticus",
     "n": "7",
     "title": "Status epilepticus",
     "tags": [
      "seizure-management",
      "acns-terminology"
     ],
     "updated": false,
     "goals": [
      "Apply the ILAE 2015 time points",
      "run the staged treatment on time and at full dose",
      "manage refractory and super-refractory status and recognize NORSE"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "Definitions (ILAE 2015)"
      },
      {
       "type": "p",
       "text": "Status epilepticus is a seizure that fails to stop by itself or seizures that recur without recovery. Two time points guide action: **t1**, when treatment should start, and **t2**, after which long-term consequences become likely."
      },
      {
       "type": "table",
       "head": [
        "Type",
        "t1 (treat)",
        "t2 (lasting harm likely)"
       ],
       "rows": [
        [
         "Tonic–clonic",
         "5 minutes",
         "30 minutes"
        ],
        [
         "Focal with impaired consciousness",
         "10 minutes",
         "Over 60 minutes"
        ],
        [
         "Absence",
         "10–15 minutes",
         "Unknown"
        ]
       ]
      },
      {
       "type": "p",
       "text": "The classification has four axes: semiology, etiology, EEG correlates and age."
      },
      {
       "type": "h3",
       "text": "Staged treatment"
      },
      {
       "type": "li",
       "text": "**Stabilize** (first 5 minutes): airway, breathing, circulation, oxygen, ECG, glucose, IV access, blood tests. If glucose is low, give thiamine and glucose."
      },
      {
       "type": "li",
       "text": "**First line** (about 5–20 minutes): a benzodiazepine at full dose. IM midazolam 10 mg (for over 40 kg), IV lorazepam 0.1 mg/kg (maximum 4 mg, may repeat once), or IV diazepam 0.15–0.2 mg/kg (maximum 10 mg, may repeat once). Under-dosing is the commonest error. In RAMPART, IM midazolam before hospital was at least as effective as IV lorazepam."
      },
      {
       "type": "li",
       "text": "**Second line** (about 20–40 minutes): levetiracetam 60 mg/kg (max 4,500 mg), fosphenytoin 20 mg PE/kg (max 1,500 mg PE), or valproate 40 mg/kg (max 3,000 mg). In ESETT the three stopped seizures with improved responsiveness in a similar proportion (about half), so choose on patient factors. Phenobarbital is an alternative."
      },
      {
       "type": "li",
       "text": "**Third line** (about 40–60 minutes): anesthetic infusion with continuous EEG: midazolam, propofol, or pentobarbital, and ketamine in some centers."
      },
      {
       "type": "figure",
       "html": "se-timeline",
       "caption": "The ILAE time points and the treatment stages on one clock. Treatment should start at t1; after t2, lasting harm becomes likely.",
       "n": 11
      },
      {
       "type": "h3",
       "text": "Refractory and beyond"
      },
      {
       "type": "li",
       "text": "**Refractory** status continues despite a benzodiazepine and one second-line drug."
      },
      {
       "type": "li",
       "text": "**Super-refractory** status continues or recurs 24 hours or more after anesthesia is started, including on weaning."
      },
      {
       "type": "li",
       "text": "**NORSE** is refractory status in someone without active epilepsy and without a clear acute cause; **FIRES** is NORSE after a febrile illness. Search for autoimmune, infectious and genetic causes, and start immunotherapy early when no other cause is found. The ketogenic diet and anakinra are options."
      },
      {
       "type": "li",
       "text": "**Propofol infusion syndrome** (acidosis, rhabdomyolysis, arrhythmia) is more likely with high doses for long periods and in children."
      },
      {
       "type": "case",
       "id": "case-status",
       "title": "Convulsions that do not stop",
       "intro": [
        "A 44-year-old with known epilepsy is brought to the emergency department still convulsing, 12 minutes after onset. Paramedics gave no medication. Weight 80 kg; IV access is in place."
       ],
       "steps": [
        {
         "narrative": [],
         "stem": "What is the first treatment?",
         "options": [
          "Levetiracetam 60 mg/kg IV",
          "Lorazepam 4 mg IV, repeated once if needed",
          "Lorazepam 1 mg IV",
          "Propofol infusion",
          "Wait for the glucose result"
         ],
         "answer": 1,
         "explain": "Give a full dose of benzodiazepine: lorazepam 0.1 mg/kg, maximum 4 mg per dose, which can be repeated once. Under-dosing is the commonest error. Check glucose, but do not delay the benzodiazepine for it."
        },
        {
         "narrative": [
          "The convulsions continue five minutes after the second lorazepam dose. He has long QT on his last ECG and chronic hepatitis."
         ],
         "stem": "Which second-line drug fits best?",
         "options": [
          "Levetiracetam 60 mg/kg (maximum 4,500 mg)",
          "Valproate 40 mg/kg",
          "Fosphenytoin 20 mg PE/kg infused rapidly",
          "A third lorazepam dose",
          "Oral lacosamide"
         ],
         "answer": 0,
         "explain": "ESETT found levetiracetam, fosphenytoin and valproate similarly effective, so choose on patient factors. Valproate is unsuitable in liver disease, and fosphenytoin carries arrhythmia and hypotension risk with cardiac conduction problems."
        },
        {
         "narrative": [
          "Twenty minutes later he is no longer convulsing but remains unresponsive, with subtle eyelid twitching."
         ],
         "stem": "What is the next step?",
         "options": [
          "Assume postictal state and observe for several hours",
          "Obtain EEG urgently to look for nonconvulsive status",
          "Discharge once he wakes",
          "Give flumazenil",
          "Obtain an MRI first"
         ],
         "answer": 1,
         "explain": "After convulsive status, persistent unresponsiveness should prompt urgent EEG, since nonconvulsive seizures commonly continue and need treatment."
        },
        {
         "narrative": [
          "EEG shows ongoing right hemispheric seizures. A second second-line drug fails, and he is intubated."
         ],
         "stem": "How is this treated?",
         "options": [
          "Add oral clobazam and wait 24 hours",
          "Anesthetic infusion (midazolam or propofol) titrated to stop seizures, with continuous EEG",
          "Stop all sedation to allow neurological examination",
          "Give a fourth benzodiazepine bolus",
          "Ketogenic diet only"
         ],
         "answer": 1,
         "explain": "Refractory status needs anesthetic infusion titrated to seizure suppression on continuous EEG, while the cause is sought and maintenance ASMs are optimized."
        }
       ],
       "outro": [
        "Time and dose: full-dose benzodiazepine by 5 minutes, a second-line drug by about 20, and EEG whenever consciousness does not return."
       ]
      },
      {
       "type": "mcq",
       "id": "q-jkge1s",
       "stem": "In the ESETT trial of benzodiazepine-refractory status epilepticus, what was the main finding?",
       "options": [
        "Fosphenytoin was superior to levetiracetam",
        "Valproate was superior to both other drugs",
        "Levetiracetam, fosphenytoin and valproate had similar efficacy, each stopping seizures in about half of patients",
        "Levetiracetam caused more hypotension",
        "None of the drugs was better than placebo"
       ],
       "answer": 2,
       "explain": "The three drugs were equally effective (success in about 45–47%), with similar adverse event rates. Choose among them on patient factors."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-1n2gwcq",
         "front": "t1 and t2 for tonic–clonic status",
         "back": "5 and 30 minutes."
        },
        {
         "id": "c-xu7e0y",
         "front": "t1 and t2 for focal impaired-consciousness status",
         "back": "10 minutes and over 60 minutes."
        },
        {
         "id": "c-tlx02",
         "front": "IM midazolam dose in status (over 40 kg)",
         "back": "10 mg."
        },
        {
         "id": "c-145jarn",
         "front": "IV lorazepam dose in status",
         "back": "0.1 mg/kg, maximum 4 mg, may repeat once."
        },
        {
         "id": "c-giu1ju",
         "front": "Second-line doses (ESETT)",
         "back": "Levetiracetam 60 mg/kg (max 4,500 mg); fosphenytoin 20 mg PE/kg (max 1,500 mg PE); valproate 40 mg/kg (max 3,000 mg)."
        },
        {
         "id": "c-lhf95m",
         "front": "Refractory status",
         "back": "Continues after a benzodiazepine and one second-line drug."
        },
        {
         "id": "c-1azcd5w",
         "front": "Super-refractory status",
         "back": "Continues or recurs 24 hours or more after starting anesthesia."
        },
        {
         "id": "c-1fovjdo",
         "front": "NORSE",
         "back": "New refractory status without active epilepsy or a clear acute cause; FIRES is the post-febrile form."
        }
       ]
      },
      {
       "type": "note",
       "label": "Try it",
       "text": "The [status epilepticus protocol](seizure_management.html#status-epilepticus) sets these steps against the clock."
      }
     ],
     "sources": [
      {
       "title": "ILAE definition and classification of status epilepticus (2015)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/26336950/"
      },
      {
       "title": "AES guideline on convulsive status epilepticus (2016)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/26900382/"
      },
      {
       "title": "ESETT trial (2019)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/31774955/"
      },
      {
       "title": "RAMPART trial (2012)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/22335736/"
      },
      {
       "title": "NORSE and FIRES consensus definitions (2018)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/29399791/"
      }
     ],
     "words": 761,
     "bank": 14
    },
    {
     "id": "diet-and-other",
     "n": "8",
     "title": "Dietary therapy and other treatments",
     "tags": [
      "seizure-management"
     ],
     "updated": false,
     "goals": [
      "Know the dietary options, their indications and contraindications",
      "use rescue medicines and immunotherapy appropriately"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "The diets"
      },
      {
       "type": "table",
       "head": [
        "Diet",
        "Composition",
        "Notes"
       ],
       "rows": [
        [
         "Classic ketogenic",
         "4:1 or 3:1 fat to protein-plus-carbohydrate by weight",
         "Most restrictive; weighed meals"
        ],
        [
         "Modified Atkins",
         "Carbohydrate limited to about 10–20 g/day, fat encouraged",
         "No weighing; suits adolescents and adults"
        ],
        [
         "Medium-chain triglyceride",
         "MCT oil produces ketones efficiently, allowing more carbohydrate",
         "Gastrointestinal side effects"
        ],
        [
         "Low glycemic index treatment",
         "Carbohydrates limited and restricted to a low glycemic index",
         "Least restrictive"
        ]
       ]
      },
      {
       "type": "h3",
       "text": "When to use it"
      },
      {
       "type": "li",
       "text": "**The treatment of choice** for GLUT1 deficiency and pyruvate dehydrogenase deficiency, where ketones supply the brain with fuel around the block."
      },
      {
       "type": "li",
       "text": "**Strong evidence** in infantile epileptic spasms syndrome, Dravet syndrome, epilepsy with myoclonic–atonic seizures, tuberous sclerosis, FIRES and super-refractory status. Consider it after two ASMs have failed."
      },
      {
       "type": "li",
       "text": "**Contraindications**: disorders of fat oxidation and ketone use: primary carnitine deficiency, carnitine palmitoyltransferase I or II deficiency, carnitine translocase deficiency, β-oxidation defects, pyruvate carboxylase deficiency and porphyria. Screen before starting."
      },
      {
       "type": "li",
       "text": "Give it about three months before judging it. Watch for acidosis, hypoglycemia, kidney stones (citrate helps), constipation, dyslipidemia, poor growth and reduced bone density."
      },
      {
       "type": "h3",
       "text": "Rescue medicines"
      },
      {
       "type": "p",
       "text": "For clusters or prolonged seizures at home: intranasal midazolam, intranasal or rectal diazepam, or buccal midazolam, with a written seizure action plan."
      },
      {
       "type": "h3",
       "text": "Immunotherapy and hormones"
      },
      {
       "type": "p",
       "text": "Corticosteroids, IVIG, plasma exchange, rituximab and cyclophosphamide for autoimmune epilepsy and encephalitis; ACTH or high-dose prednisolone for infantile spasms; steroids in spike-wave activation in sleep."
      },
      {
       "type": "mcq",
       "id": "q-1n80j4g",
       "stem": "A child is being considered for the ketogenic diet. Which condition must be excluded first because the diet could be fatal?",
       "options": [
        "GLUT1 deficiency",
        "Pyruvate dehydrogenase deficiency",
        "Carnitine palmitoyltransferase II deficiency",
        "Tuberous sclerosis",
        "Dravet syndrome"
       ],
       "answer": 2,
       "explain": "Fat oxidation defects, including CPT I and II deficiency, carnitine deficiency and translocase deficiency, make the diet dangerous. GLUT1 and pyruvate dehydrogenase deficiency are its strongest indications."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-wvuc6g",
         "front": "Ketogenic diet treatment of choice",
         "back": "GLUT1 deficiency and pyruvate dehydrogenase deficiency."
        },
        {
         "id": "c-6y3xa4",
         "front": "Ketogenic diet contraindications",
         "back": "Fat oxidation defects (carnitine deficiency, CPT I/II, translocase, β-oxidation), pyruvate carboxylase deficiency, porphyria."
        },
        {
         "id": "c-1ku18j5",
         "front": "Classic ketogenic ratio",
         "back": "4:1 or 3:1 fat to protein plus carbohydrate."
        },
        {
         "id": "c-6tebrf",
         "front": "Modified Atkins carbohydrate limit",
         "back": "About 10–20 g per day."
        },
        {
         "id": "c-rmx5p2",
         "front": "How long to try the diet",
         "back": "About three months."
        },
        {
         "id": "c-1y7mnet",
         "front": "Home rescue options",
         "back": "Intranasal midazolam, intranasal or rectal diazepam, buccal midazolam."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "International Ketogenic Diet Study Group consensus (2018)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/29881797/"
      },
      {
       "title": "NORSE and FIRES consensus definitions (2018)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/29399791/"
      }
     ],
     "words": 348,
     "bank": 6
    }
   ],
   "intro": [
    "Choosing, dosing, combining and stopping antiseizure medications (ASMs), treating women and other special groups, and stopping status epilepticus. For bedside dosing tables, see the [Seizure Management guide](seizure_management.html)."
   ]
  },
  {
   "id": "surgery",
   "n": "8",
   "title": "Epilepsy surgery and devices",
   "short": "Surgery",
   "sections": [
    {
     "id": "presurgical-evaluation",
     "n": "1",
     "title": "Who to refer, and the presurgical zones",
     "tags": [
      "epilepsy-surgery"
     ],
     "updated": false,
     "goals": [
      "Recognize who should be referred for surgical evaluation, and when",
      "name the six cortical zones and the test that defines each",
      "explain why agreement between tests matters more than any single test"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "Refer early"
      },
      {
       "type": "p",
       "text": "Once two appropriate, tolerated ASMs have failed, the chance that the next drug will stop the seizures is small. That is the point to refer to a comprehensive epilepsy center, not after five or six drugs. Earlier referral also makes sense when the diagnosis is in doubt, when MRI shows a lesion that fits the seizures, or when a child's development is being harmed by frequent seizures."
      },
      {
       "type": "p",
       "text": "Two randomized trials settled the question for temporal lobe epilepsy:"
      },
      {
       "type": "li",
       "text": "**Surgery versus continued medication (Wiebe 2001)**: at one year, 58% of the surgical group were free of seizures that impair awareness, against 8% of the medical group."
      },
      {
       "type": "li",
       "text": "**Early surgery (ERSET 2012)**: in patients within two years of failing a second drug, 11 of 15 treated surgically were seizure-free in the second year, against none of 23 treated medically."
      },
      {
       "type": "li",
       "text": "**Children (Dwivedi 2017)**: in a trial of 116 children and adolescents, 77% were seizure-free at one year after surgery against 7% on medication alone."
      },
      {
       "type": "p",
       "text": "Despite this, adults often reach surgery after about two decades of epilepsy, by which time psychosocial and cognitive harm is harder to undo."
      },
      {
       "type": "h3",
       "text": "The six zones"
      },
      {
       "type": "p",
       "text": "Surgery aims to remove or disconnect the **epileptogenic zone**: the cortex whose removal stops the seizures. No test measures it directly. It is inferred from six other zones, each defined by a different test, and it is only proven when the patient is seizure-free after surgery."
      },
      {
       "type": "table",
       "head": [
        "Zone",
        "What it is",
        "Main test"
       ],
       "rows": [
        [
         "Symptomatogenic zone",
         "Cortex that produces the signs of the seizure",
         "Semiology on video"
        ],
        [
         "Irritative zone",
         "Cortex that produces interictal spikes",
         "Interictal EEG, MEG"
        ],
        [
         "Seizure-onset zone",
         "Where recorded seizures begin",
         "Ictal EEG (scalp or intracranial), ictal SPECT"
        ],
        [
         "Epileptogenic lesion",
         "The structural abnormality responsible",
         "MRI"
        ],
        [
         "Functional deficit zone",
         "Cortex that works abnormally between seizures",
         "Examination, neuropsychology, FDG-PET, focal slowing"
        ],
        [
         "Eloquent cortex",
         "Cortex needed for language, movement or vision",
         "Stimulation mapping, fMRI, Wada test, MEG"
        ]
       ]
      },
      {
       "type": "figure",
       "html": "presurgical-zones",
       "caption": "The six zones in a left temporal example, each drawn on its own against the epileptogenic zone (dashed). They overlap it, but rarely match it in size or position.",
       "n": 12
      },
      {
       "type": "h3",
       "text": "Concordance decides the plan"
      },
      {
       "type": "p",
       "text": "When semiology, EEG, MRI and the functional tests all point to the same region, the chance of seizure freedom is high, and the team may operate without intracranial EEG. Discordant results are the main reason to record intracranially, or to choose a palliative option instead. These decisions are made together at a multidisciplinary conference."
      },
      {
       "type": "note",
       "label": "Board pearl",
       "text": "A seizure can be silent until it spreads. The first sign then comes from the symptomatogenic zone, which can be some distance from where the seizure begins."
      },
      {
       "type": "mcq",
       "id": "q-3g1zeo",
       "stem": "Which zone can be confirmed only by the result of surgery?",
       "options": [
        "Irritative zone",
        "Seizure-onset zone",
        "Epileptogenic zone",
        "Functional deficit zone",
        "Symptomatogenic zone"
       ],
       "answer": 2,
       "explain": "The epileptogenic zone is defined by what happens after it is removed: if the patient is seizure-free, it was included in the resection. Every other zone has a test that defines it before surgery."
      },
      {
       "type": "mcq",
       "id": "q-s6gs3z",
       "stem": "A 26-year-old's seizures begin with clonic jerking of the right hand. Intracranial EEG shows that onset is in the left premotor cortex, and the hand jerking appears only when the discharge reaches primary motor cortex. What does the primary motor cortex represent here?",
       "options": [
        "Seizure-onset zone",
        "Symptomatogenic zone",
        "Irritative zone",
        "Epileptogenic lesion",
        "Functional deficit zone"
       ],
       "answer": 1,
       "explain": "The motor cortex produces the first sign but is reached by spread. Resecting it would cause a deficit without stopping the seizures, which start in premotor cortex."
      },
      {
       "type": "mcq",
       "id": "q-p6o6i8",
       "stem": "A 38-year-old has had focal impaired awareness seizures for 20 years. They have tried levetiracetam, lamotrigine and lacosamide at adequate doses. MRI shows left hippocampal sclerosis. What is the best next step?",
       "options": [
        "Add a fourth ASM and review in six months",
        "Refer to a comprehensive epilepsy center for presurgical evaluation",
        "Refer only if seizures become more frequent",
        "Implant a vagus nerve stimulator",
        "Start the ketogenic diet"
       ],
       "answer": 1,
       "explain": "After two adequate ASM trials, this is drug-resistant epilepsy, and hippocampal sclerosis responds well to surgery. Waiting for more drug trials only delays the most effective treatment."
      },
      {
       "type": "match",
       "id": "match-zones",
       "prompt": "Match each zone to the test that best defines it",
       "pairs": [
        [
         "Symptomatogenic zone",
         "Video analysis of the seizure"
        ],
        [
         "Irritative zone",
         "Interictal EEG or MEG"
        ],
        [
         "Seizure-onset zone",
         "Ictal EEG"
        ],
        [
         "Epileptogenic lesion",
         "MRI"
        ],
        [
         "Functional deficit zone",
         "FDG-PET and neuropsychology"
        ],
        [
         "Eloquent cortex",
         "Electrical stimulation mapping"
        ]
       ]
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-1kekcdt",
         "front": "Epileptogenic zone",
         "back": "The cortex whose removal or disconnection stops the seizures. Inferred from the other zones, proven only by outcome."
        },
        {
         "id": "c-1j83d02",
         "front": "Symptomatogenic zone",
         "back": "Cortex that produces the ictal signs; can be distant from the onset when a seizure is silent until it spreads."
        },
        {
         "id": "c-1n0q5u4",
         "front": "Irritative zone",
         "back": "Cortex that produces interictal spikes; usually larger than the epileptogenic zone."
        },
        {
         "id": "c-v6dk2w",
         "front": "Functional deficit zone",
         "back": "Cortex working abnormally between seizures; shown by FDG-PET, neuropsychology and focal slowing."
        },
        {
         "id": "c-1yr272m",
         "front": "Wiebe 2001 trial",
         "back": "Temporal lobe epilepsy: 58% free of seizures impairing awareness after surgery versus 8% on medication at one year."
        },
        {
         "id": "c-264v93",
         "front": "ERSET 2012 trial",
         "back": "Early surgery: 11 of 15 seizure-free in year two, against none of 23 on medication."
        },
        {
         "id": "c-ywffdq",
         "front": "When to refer for presurgical evaluation",
         "back": "After two appropriate, tolerated ASMs have failed."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "Presurgical evaluation of epilepsy (Rosenow and Lüders 2001)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/11522572/"
      },
      {
       "title": "Randomized trial of surgery for temporal lobe epilepsy (Wiebe 2001)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/11484687/"
      },
      {
       "title": "Early surgical therapy for drug-resistant temporal lobe epilepsy (ERSET, 2012)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/22396514/"
      },
      {
       "title": "Surgery for drug-resistant epilepsy in children (Dwivedi 2017)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/29069568/"
      },
      {
       "title": "Definition of drug-resistant epilepsy (ILAE 2010)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/19889013/"
      }
     ],
     "words": 820,
     "bank": 8
    },
    {
     "id": "epilepsy-imaging",
     "n": "2",
     "title": "Imaging and functional testing",
     "tags": [
      "epilepsy-surgery"
     ],
     "updated": false,
     "goals": [
      "Order an epilepsy-protocol MRI and know what it looks for",
      "recognize hippocampal sclerosis and focal cortical dysplasia",
      "know what PET, SPECT, MEG, fMRI, the Wada test and neuropsychology each add"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "MRI: the most important test"
      },
      {
       "type": "p",
       "text": "A routine brain MRI misses many epileptogenic lesions. The ILAE's recommended protocol (HARNESS-MRI) has three core sequences:"
      },
      {
       "type": "li",
       "text": "A 3D T1-weighted volume with 1 mm isotropic voxels"
      },
      {
       "type": "li",
       "text": "A 3D FLAIR volume"
      },
      {
       "type": "li",
       "text": "High-resolution coronal T2, cut perpendicular to the long axis of the hippocampus"
      },
      {
       "type": "p",
       "text": "Have the scan read, or re-read, by someone experienced in epilepsy imaging, with the clinical and EEG findings in hand. A dedicated protocol reviewed by an expert finds lesions that routine scans and reports miss. Add susceptibility-weighted imaging to look for cavernomas and calcification."
      },
      {
       "type": "h3",
       "text": "Hippocampal sclerosis"
      },
      {
       "type": "li",
       "text": "**On MRI**: a small hippocampus with high T2 and FLAIR signal and loss of its internal structure. Supporting signs include a shrunken fornix and mammillary body on the same side, a larger temporal horn, and blurring of the temporal pole."
      },
      {
       "type": "li",
       "text": "**ILAE types**: type 1 (neuron loss in CA1 and CA4) is the commonest and has the best seizure outcome after surgery. Type 2 affects mainly CA1 and type 3 mainly CA4. \"Gliosis only, no hippocampal sclerosis\" is a separate category."
      },
      {
       "type": "h3",
       "text": "Focal cortical dysplasia"
      },
      {
       "type": "p",
       "text": "The 2022 ILAE classification keeps the three main types and adds genetic findings to an integrated diagnosis."
      },
      {
       "type": "li",
       "text": "**Type I**: abnormal layering of the cortex. It is often invisible on MRI."
      },
      {
       "type": "li",
       "text": "**Type II**: dysmorphic neurons, without (IIa) or with (IIb) balloon cells. On MRI it shows thick cortex, a blurred gray–white junction and, in IIb, a **transmantle sign**: a funnel of FLAIR signal tapering from the cortex toward the ventricle. Type II lesions are often small and sit at the **bottom of a sulcus**. Somatic variants in the mTOR pathway are a common cause."
      },
      {
       "type": "li",
       "text": "**Type III**: dysplasia next to another main lesion: hippocampal sclerosis (IIIa), a tumor (IIIb), a vascular malformation (IIIc), or another lesion acquired early in life (IIId)."
      },
      {
       "type": "li",
       "text": "**Added in 2022**: mild malformation of cortical development (mMCD), and MOGHE (mild malformation of cortical development with oligodendroglial hyperplasia in epilepsy)."
      },
      {
       "type": "h3",
       "text": "Functional tests"
      },
      {
       "type": "li",
       "text": "**FDG-PET**: interictal hypometabolism helps lateralize temporal lobe epilepsy, especially when the MRI is normal. The hypometabolic area is wider than the epileptogenic zone."
      },
      {
       "type": "li",
       "text": "**Ictal SPECT**: tracer injected within seconds of seizure onset shows hyperperfusion at the onset. Subtracting an interictal scan and fusing the result with MRI (SISCOM) sharpens the picture. A late injection shows where the seizure spread, not where it began."
      },
      {
       "type": "li",
       "text": "**MEG**: localizes the sources of interictal spikes. It is most useful in neocortical and MRI-negative epilepsy, and it can also map eloquent cortex."
      },
      {
       "type": "li",
       "text": "**fMRI**: lateralizes language. It can replace the Wada test in many patients with temporal lobe epilepsy, and fMRI of verbal memory or language helps predict memory decline after surgery."
      },
      {
       "type": "li",
       "text": "**Wada test**: an intracarotid barbiturate (or similar drug) briefly anesthetizes one hemisphere while language and memory are tested. It is now reserved mainly for patients whose fMRI is atypical or unreliable."
      },
      {
       "type": "li",
       "text": "**Neuropsychology**: sets a baseline and helps lateralize. Poor verbal memory suggests dominant (usually left) temporal dysfunction; the nondominant pattern is less reliable."
      },
      {
       "type": "h3",
       "text": "Who is most at risk of memory decline"
      },
      {
       "type": "p",
       "text": "Verbal memory is most likely to fall after surgery on the dominant temporal lobe in someone whose memory is still good before surgery. The risk is higher still when the hippocampus looks normal on MRI, the epilepsy began later in life, or fMRI shows memory activation on the side of surgery. Tissue that still works has more to lose."
      },
      {
       "type": "mcq",
       "id": "q-10kgs41",
       "stem": "In a patient with MRI-negative temporal lobe epilepsy, ictal SPECT was injected 70 seconds after clinical onset. It shows hyperperfusion in the right frontal lobe. How should this be interpreted?",
       "options": [
        "The right frontal lobe is the seizure-onset zone",
        "The finding may show spread rather than onset because the injection was late",
        "Ictal SPECT cannot be used in temporal lobe epilepsy",
        "The scan should be read as interictal",
        "Hyperperfusion always marks the functional deficit zone"
       ],
       "answer": 1,
       "explain": "Blood flow follows the seizure as it spreads. The earlier the injection after onset, the more likely the hyperperfusion marks where the seizure began."
      },
      {
       "type": "mcq",
       "id": "q-1466gjl",
       "stem": "Which candidate for anterior temporal lobectomy is at highest risk of verbal memory decline?",
       "options": [
        "Right-sided resection, severe hippocampal sclerosis, poor visual memory",
        "Left-sided resection, severe hippocampal sclerosis, poor verbal memory",
        "Left-sided resection, normal-looking hippocampus, strong verbal memory",
        "Right-sided resection, normal MRI, strong verbal memory",
        "Left-sided resection in a right-hemisphere-dominant patient with poor memory"
       ],
       "answer": 2,
       "explain": "The risk is highest when the dominant hippocampus still works well: normal on MRI and with good baseline verbal memory. Removing an already sclerotic hippocampus costs less."
      },
      {
       "type": "mcq",
       "id": "q-o0rqaa",
       "stem": "A 19-year-old has drug-resistant focal motor seizures. MRI shows a small area of FLAIR signal tapering from the depth of a frontal sulcus toward the lateral ventricle. What is the most likely diagnosis?",
       "options": [
        "Hippocampal sclerosis",
        "Cavernous malformation",
        "Focal cortical dysplasia type IIb",
        "Dysembryoplastic neuroepithelial tumor",
        "Polymicrogyria"
       ],
       "answer": 2,
       "explain": "The transmantle sign is typical of type IIb dysplasia, which often sits at the bottom of a sulcus. Complete resection gives a good chance of seizure freedom."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-wtb839",
         "front": "HARNESS-MRI core sequences",
         "back": "3D T1 (1 mm isotropic), 3D FLAIR, and high-resolution coronal T2 perpendicular to the hippocampus."
        },
        {
         "id": "c-s9pmcb",
         "front": "Hippocampal sclerosis on MRI",
         "back": "Small hippocampus, high T2 and FLAIR signal, loss of internal structure."
        },
        {
         "id": "c-u7gvjj",
         "front": "Hippocampal sclerosis type with the best surgical outcome",
         "back": "ILAE type 1 (CA1 and CA4 neuron loss), also the commonest."
        },
        {
         "id": "c-88u2ob",
         "front": "Transmantle sign",
         "back": "A funnel of FLAIR signal from cortex toward the ventricle; focal cortical dysplasia type IIb."
        },
        {
         "id": "c-kgfqew",
         "front": "Bottom-of-sulcus dysplasia",
         "back": "A small type II focal cortical dysplasia at the depth of a sulcus; easy to miss on a routine MRI."
        },
        {
         "id": "c-vqgkic",
         "front": "FDG-PET in epilepsy",
         "back": "Interictal hypometabolism; lateralizes but overestimates the epileptogenic zone."
        },
        {
         "id": "c-1br3bgn",
         "front": "Why ictal SPECT timing matters",
         "back": "Late injection shows spread, not onset."
        },
        {
         "id": "c-1jaiv4y",
         "front": "Highest risk of verbal memory decline",
         "back": "Dominant temporal resection with good baseline memory and a normal-looking hippocampus."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "HARNESS-MRI protocol (ILAE Neuroimaging Task Force 2019)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/31135062/"
      },
      {
       "title": "ILAE classification of hippocampal sclerosis (2013)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/23692496/"
      },
      {
       "title": "ILAE classification of focal cortical dysplasia, 2022 update",
       "url": "https://pubmed.ncbi.nlm.nih.gov/35706131/"
      },
      {
       "title": "AAN guideline: fMRI in presurgical evaluation (2017)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/28077494/"
      },
      {
       "title": "Presurgical evaluation of epilepsy (Rosenow and Lüders 2001)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/11522572/"
      }
     ],
     "words": 934,
     "bank": 12
    },
    {
     "id": "invasive-eeg",
     "n": "3",
     "title": "Intracranial EEG",
     "tags": [
      "epilepsy-surgery",
      "eeg-fundamentals"
     ],
     "updated": false,
     "goals": [
      "Decide when intracranial EEG is needed",
      "compare stereo-EEG with subdural grids",
      "interpret intracranial onsets and stimulation mapping with their limits in mind"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "When to record intracranially"
      },
      {
       "type": "li",
       "text": "The noninvasive tests disagree, or the MRI is normal."
      },
      {
       "type": "li",
       "text": "The likely onset lies next to eloquent cortex that needs mapping."
      },
      {
       "type": "li",
       "text": "Seizures may start independently in both temporal lobes."
      },
      {
       "type": "li",
       "text": "Two possible sources exist, such as a lesion plus hippocampal sclerosis."
      },
      {
       "type": "p",
       "text": "Intracranial electrodes record only the tissue they touch. The plan must start from a clear hypothesis about where seizures begin and spread, built from the noninvasive data. Otherwise the recording shows the seizure arriving from somewhere unsampled."
      },
      {
       "type": "h3",
       "text": "Stereo-EEG or subdural electrodes"
      },
      {
       "type": "table",
       "head": [
        "",
        "Stereo-EEG (depth electrodes)",
        "Subdural grids and strips"
       ],
       "rows": [
        [
         "Placement",
         "Through small drill holes",
         "Through a craniotomy"
        ],
        [
         "Reaches",
         "Deep structures, sulcal depths, insula, both hemispheres",
         "The surface of the convexity"
        ],
        [
         "Mapping",
         "Samples points in 3D",
         "Continuous surface coverage for functional mapping"
        ],
        [
         "Risk",
         "Lower: small risk of bleeding and infection",
         "Higher: bleeding, infection, CSF leak, brain swelling"
        ]
       ]
      },
      {
       "type": "p",
       "text": "Stereo-EEG has become the preferred method in many centers. Through the same electrodes, radiofrequency thermocoagulation can treat a small onset zone."
      },
      {
       "type": "h3",
       "text": "Reading the onset"
      },
      {
       "type": "li",
       "text": "**Low-voltage fast activity** (beta or gamma range) is the commonest intracranial onset pattern, and it points to a nearby onset."
      },
      {
       "type": "li",
       "text": "**A focal series of rhythmic spikes** before the fast activity is typical of mesial temporal onset."
      },
      {
       "type": "li",
       "text": "**If the first change on intracranial EEG is already a well-formed, slower rhythm**, or it appears on the edge contacts, suspect that the true onset lies outside the sampled area."
      },
      {
       "type": "li",
       "text": "**High-frequency oscillations** (ripples at 80–250 Hz and fast ripples above 250 Hz) are markers of epileptogenic tissue. Using them to guide the extent of resection has not yet been shown to improve outcome."
      },
      {
       "type": "h3",
       "text": "Stimulation mapping"
      },
      {
       "type": "p",
       "text": "Brief trains of electrical stimulation between adjacent contacts identify motor, sensory, visual and language cortex. Speech arrest or naming failure marks cortex to spare. Stimulation can also trigger afterdischarges or a habitual seizure; a habitual seizure supports the proposed onset zone."
      },
      {
       "type": "note",
       "label": "Board pearl",
       "text": "A normal intracranial EEG in the sampled area does not exclude an onset elsewhere. Coverage is always the limit."
      },
      {
       "type": "mcq",
       "id": "q-8q9nxq",
       "stem": "A patient with MRI-negative epilepsy has seizures that suggest either insular or bilateral mesial temporal onset. Which invasive approach samples these areas best?",
       "options": [
        "Stereo-EEG with depth electrodes",
        "A large left frontotemporal subdural grid",
        "Bilateral foramen ovale electrodes alone",
        "Repeated scalp video-EEG",
        "Intraoperative electrocorticography during resection"
       ],
       "answer": 0,
       "explain": "Depth electrodes reach deep structures such as the insula and both hippocampi through small drill holes, which a surface grid cannot do."
      },
      {
       "type": "mcq",
       "id": "q-msbvzc",
       "stem": "On stereo-EEG, a patient's seizures begin with well-formed 6 Hz rhythmic activity that appears first on the deepest contact of an electrode aimed at the edge of the hypothesized zone. What is the most important concern?",
       "options": [
        "The electrode has failed",
        "The true onset may lie outside the sampled region",
        "This confirms a mesial temporal onset",
        "The seizure is nonepileptic",
        "The patient needs an extra dose of their ASM"
       ],
       "answer": 1,
       "explain": "A well-formed slower rhythm at the start, especially on edge contacts, suggests the seizure began elsewhere and spread to the electrode. Low-voltage fast activity at a contact points to a nearby onset."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-1l3p9pp",
         "front": "Why record intracranially",
         "back": "Discordant or MRI-negative workup, onset near eloquent cortex, possible bitemporal onset, or dual pathology."
        },
        {
         "id": "c-1wzqo38",
         "front": "Stereo-EEG strength",
         "back": "Samples deep structures, sulcal depths, insula and both hemispheres through small drill holes."
        },
        {
         "id": "c-1ussgic",
         "front": "Subdural grid strength",
         "back": "Continuous surface coverage for mapping eloquent cortex on the convexity."
        },
        {
         "id": "c-1f1h1e1",
         "front": "Commonest intracranial onset pattern",
         "back": "Low-voltage fast activity."
        },
        {
         "id": "c-10d9bnq",
         "front": "Clue that the onset is outside the sampled area",
         "back": "Well-formed, slower rhythm at onset, or first changes on edge contacts."
        },
        {
         "id": "c-13dpj66",
         "front": "High-frequency oscillations",
         "back": "Ripples 80–250 Hz, fast ripples above 250 Hz; markers of epileptogenic tissue, not yet proven to guide resection."
        }
       ]
      },
      {
       "type": "note",
       "label": "Try it",
       "text": "See the mesial temporal structures and stereo-EEG targets in the [Epilepsy Brain Atlas](eeg_atlas.html)."
      }
     ],
     "sources": [
      {
       "title": "French guidelines on stereo-EEG (Isnard 2018)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/29277357/"
      },
      {
       "title": "Presurgical evaluation of epilepsy (Rosenow and Lüders 2001)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/11522572/"
      }
     ],
     "words": 599,
     "bank": 8
    },
    {
     "id": "surgical-options",
     "n": "4",
     "title": "Resection, ablation and disconnection",
     "tags": [
      "epilepsy-surgery"
     ],
     "updated": false,
     "goals": [
      "Match the procedure to the cause",
      "quote expected outcomes and complications",
      "score outcome with the Engel and ILAE scales"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "Procedures"
      },
      {
       "type": "li",
       "text": "**Anterior temporal lobectomy**: the standard operation for mesial temporal lobe epilepsy with hippocampal sclerosis. In reviews, about two-thirds of patients become free of disabling seizures."
      },
      {
       "type": "li",
       "text": "**Selective amygdalohippocampectomy**: removes the mesial structures and spares the lateral temporal cortex. Seizure outcome is similar, with small differences in cognitive outcome."
      },
      {
       "type": "li",
       "text": "**Laser interstitial thermal therapy (LITT)**: MRI-guided ablation through a single burr hole. Seizure freedom after mesial temporal ablation is somewhat lower than after open surgery, but naming and recognition are better preserved and recovery is quicker. It suits small deep targets such as a **hypothalamic hamartoma**."
      },
      {
       "type": "li",
       "text": "**Lesionectomy**: for tumors that cause epilepsy (ganglioglioma, dysembryoplastic neuroepithelial tumor) and cavernomas. Complete removal predicts success; for a cavernoma, include the hemosiderin rim."
      },
      {
       "type": "li",
       "text": "**Hemispherotomy**: disconnects one hemisphere. Used for Rasmussen encephalitis, hemimegalencephaly, large perinatal stroke and Sturge–Weber syndrome. Seizure freedom rates are high. It causes, or completes, a contralateral hemiparesis and hemianopia, so it suits those who already have them."
      },
      {
       "type": "li",
       "text": "**Corpus callosotomy**: a palliative operation for drop attacks, as in Lennox–Gastaut syndrome."
      },
      {
       "type": "h3",
       "text": "What predicts a good result"
      },
      {
       "type": "p",
       "text": "Good predictors are a clear lesion on MRI (especially hippocampal sclerosis or a low-grade tumor), agreement between tests, complete removal, and a shorter duration of epilepsy. Results are poorer when the MRI is normal, the onset is outside the temporal lobe, or both hemispheres are involved. Outcomes in children are similar to those in adults."
      },
      {
       "type": "h3",
       "text": "Complications"
      },
      {
       "type": "li",
       "text": "**Death**: perioperative mortality is about 0.1–0.5%."
      },
      {
       "type": "li",
       "text": "**Vision**: the commonest neurological complication of temporal resection is a contralateral superior quadrantanopia (\"pie in the sky\"), from damage to Meyer's loop."
      },
      {
       "type": "li",
       "text": "**Language and memory**: naming difficulty and verbal memory decline, after surgery on the dominant temporal lobe."
      },
      {
       "type": "li",
       "text": "**Mood**: new or worse depression or anxiety, most often in the first months."
      },
      {
       "type": "li",
       "text": "**Rarer**: hemiparesis (anterior choroidal artery territory) and diplopia (fourth cranial nerve)."
      },
      {
       "type": "h3",
       "text": "Scoring the outcome"
      },
      {
       "type": "table",
       "head": [
        "Engel class",
        "Meaning"
       ],
       "rows": [
        [
         "I",
         "Free of disabling seizures (IA: completely seizure-free since surgery; IB: auras only)"
        ],
        [
         "II",
         "Rare disabling seizures"
        ],
        [
         "III",
         "Worthwhile improvement"
        ],
        [
         "IV",
         "No worthwhile improvement"
        ]
       ]
      },
      {
       "type": "table",
       "head": [
        "ILAE class",
        "Meaning"
       ],
       "rows": [
        [
         "1",
         "Completely seizure-free, no auras"
        ],
        [
         "2",
         "Auras only"
        ],
        [
         "3",
         "1 to 3 seizure days a year"
        ],
        [
         "4",
         "4 seizure days a year, up to a 50% reduction from baseline"
        ],
        [
         "5",
         "Less than 50% reduction, up to a 100% increase"
        ],
        [
         "6",
         "More than a 100% increase"
        ]
       ]
      },
      {
       "type": "note",
       "label": "Board pearl",
       "text": "The ILAE scale separates \"auras only\" (class 2) from complete freedom (class 1). Engel groups both under class I."
      },
      {
       "type": "case",
       "id": "case-mtle",
       "title": "Mesial temporal lobe epilepsy",
       "intro": [
        "A 32-year-old right-handed accountant has had seizures since age 14 that begin with a rising epigastric feeling. Then comes staring with lip-smacking and fumbling of the left hand, while the right arm stiffens in a twisted posture. She has tried three ASMs at adequate doses and still has two seizures a month."
       ],
       "steps": [
        {
         "narrative": [],
         "stem": "Which side does the semiology point to?",
         "options": [
          "Left temporal",
          "Right temporal",
          "Left frontal",
          "It cannot be lateralized",
          "Bilateral"
         ],
         "answer": 0,
         "explain": "Dystonic posturing of one arm is contralateral to the onset (the right arm here, so a left-sided onset). Hand automatisms usually occur on the same side as the onset (the left hand here)."
        },
        {
         "narrative": [
          "MRI shows left hippocampal sclerosis. Video-EEG records five typical seizures with left temporal rhythmic theta, and interictal spikes are seen only over the left anterior temporal region. Neuropsychology shows impaired verbal memory, and fMRI shows left language dominance."
         ],
         "stem": "What is the best next step?",
         "options": [
          "Stereo-EEG of both temporal lobes before any decision",
          "Present at the surgical conference and offer left temporal surgery",
          "Add a fourth ASM",
          "Vagus nerve stimulation",
          "Repeat MRI in a year"
         ],
         "answer": 1,
         "explain": "Semiology, EEG, MRI and neuropsychology agree. With this concordance, intracranial EEG adds little, and surgery gives the best chance of seizure freedom."
        },
        {
         "narrative": [
          "She chooses an open anterior temporal lobectomy rather than laser ablation."
         ],
         "stem": "Which new deficit is she most likely to notice?",
         "options": [
          "Right hemiparesis",
          "A right upper quadrant visual field defect",
          "Left upper quadrant visual field defect",
          "Dense global amnesia",
          "Right facial numbness"
         ],
         "answer": 1,
         "explain": "Temporal resection interrupts Meyer's loop, causing a contralateral superior quadrantanopia. It is often not noticed by the patient but matters for driving assessments."
        },
        {
         "narrative": [
          "Two years later she has had only three brief episodes of her old rising epigastric feeling, with no loss of awareness."
         ],
         "stem": "How is her outcome scored?",
         "options": [
          "Engel IA, ILAE class 1",
          "Engel IB, ILAE class 2",
          "Engel II, ILAE class 3",
          "Engel III, ILAE class 4",
          "Engel IV, ILAE class 5"
         ],
         "answer": 1,
         "explain": "Auras only since surgery is Engel IB and ILAE class 2. It counts as a good outcome and is common after temporal lobectomy."
        }
       ],
       "outro": [
        "Concordant semiology, EEG, MRI and neuropsychology mean a high chance of seizure freedom without invasive monitoring. Discuss vision, memory and mood before surgery, not after."
       ]
      },
      {
       "type": "match",
       "id": "match-procedures",
       "prompt": "Match each procedure to a classic indication",
       "pairs": [
        [
         "Anterior temporal lobectomy",
         "Hippocampal sclerosis"
        ],
        [
         "Laser interstitial thermal therapy",
         "Hypothalamic hamartoma with gelastic seizures"
        ],
        [
         "Hemispherotomy",
         "Rasmussen encephalitis"
        ],
        [
         "Corpus callosotomy",
         "Drop attacks in Lennox–Gastaut syndrome"
        ],
        [
         "Lesionectomy including the hemosiderin rim",
         "Cavernous malformation"
        ]
       ]
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-1tag5tb",
         "front": "Anterior temporal lobectomy outcome",
         "back": "About two-thirds free of disabling seizures."
        },
        {
         "id": "c-1bigex5",
         "front": "LITT versus open temporal surgery",
         "back": "Somewhat lower seizure freedom, but better naming and recognition and quicker recovery."
        },
        {
         "id": "c-1pgoswn",
         "front": "Hemispherotomy indications",
         "back": "Rasmussen encephalitis, hemimegalencephaly, large perinatal stroke, Sturge–Weber syndrome."
        },
        {
         "id": "c-3cykyh",
         "front": "Corpus callosotomy",
         "back": "Palliative surgery for drop attacks."
        },
        {
         "id": "c-13vfzpa",
         "front": "Pie in the sky",
         "back": "Contralateral superior quadrantanopia from Meyer's loop damage in temporal resection."
        },
        {
         "id": "c-1iz8loh",
         "front": "Engel IB",
         "back": "Auras only since surgery."
        },
        {
         "id": "c-1jbdk5k",
         "front": "ILAE class 2",
         "back": "Auras only."
        },
        {
         "id": "c-1jld5uj",
         "front": "ILAE class 3",
         "back": "1 to 3 seizure days a year."
        },
        {
         "id": "c-16xi70c",
         "front": "Perioperative mortality of epilepsy surgery",
         "back": "About 0.1–0.5%."
        },
        {
         "id": "c-2f92j0",
         "front": "Poorer surgical outcome",
         "back": "Normal MRI, extratemporal onset, bilateral involvement, incomplete resection."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "Resective epilepsy surgery for drug-resistant focal epilepsy (Jobst and Cascino 2015)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/25602999/"
      },
      {
       "title": "ILAE classification of outcome after epilepsy surgery (Wieser 2001)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/11240604/"
      },
      {
       "title": "Randomized trial of surgery for temporal lobe epilepsy (Wiebe 2001)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/11484687/"
      },
      {
       "title": "Surgery for drug-resistant epilepsy in children (Dwivedi 2017)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/29069568/"
      }
     ],
     "words": 808,
     "bank": 9
    },
    {
     "id": "neuromodulation",
     "n": "5",
     "title": "Neuromodulation",
     "tags": [
      "epilepsy-surgery"
     ],
     "updated": false,
     "goals": [
      "Choose between vagus nerve, responsive and deep brain stimulation",
      "know the pivotal trial results and side effects",
      "set realistic expectations"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "What devices do"
      },
      {
       "type": "p",
       "text": "Devices are for people who are not candidates for resection, or who prefer not to have it. They rarely make anyone seizure-free, but the benefit tends to grow over years of stimulation."
      },
      {
       "type": "figure",
       "html": "neuromod-devices",
       "caption": "Where each device sits, from the front: the patient's left is on the right of the drawing. Choose a device for its parts, its target and when it stimulates.",
       "n": 13
      },
      {
       "type": "table",
       "head": [
        "",
        "Vagus nerve stimulation (VNS)",
        "Responsive neurostimulation (RNS)",
        "Deep brain stimulation (DBS)"
       ],
       "rows": [
        [
         "Target",
         "Left cervical vagus nerve",
         "One or two seizure foci",
         "Anterior nucleus of the thalamus"
        ],
        [
         "How",
         "Scheduled stimulation, plus a magnet to trigger extra stimulation",
         "Detects seizure patterns and stimulates in response",
         "Scheduled stimulation"
        ],
        [
         "Pivotal trial",
         "28% vs 15% seizure reduction at 3 months",
         "38% vs 17% reduction in the blinded phase",
         "40% vs 15% reduction in the blinded phase"
        ],
        [
         "Suits",
         "Labeled for focal seizures from age 4; also used for generalized epilepsies such as Lennox–Gastaut syndrome; no intracranial surgery",
         "Adults with one or two well-defined foci that cannot be removed, such as both hippocampi or eloquent cortex",
         "Adults with focal epilepsy after three or more failed ASMs, often with no single resectable focus"
        ],
        [
         "Side effects",
         "Hoarseness, cough, throat discomfort, breathlessness during stimulation; can worsen sleep apnea",
         "Implant-site infection, bleeding",
         "Paresthesias, implant-site infection; depression and memory complaints were more common in the trial"
        ]
       ]
      },
      {
       "type": "figure",
       "html": "neuromod-hardware",
       "caption": "What each device looks like, and how it shows on a plain film: the hardware drawn to scale from published dimensions, beside a frontal or lateral radiograph of a typical placement. The numbers match the list under each picture.",
       "n": 14
      },
      {
       "type": "h3",
       "text": "Practical points"
      },
      {
       "type": "li",
       "text": "**VNS is placed on the left** because the right vagus nerve carries more fibers to the sinoatrial node, so right-sided stimulation risks bradycardia."
      },
      {
       "type": "li",
       "text": "**Some VNS models detect a sudden rise in heart rate** (ictal tachycardia) and stimulate automatically."
      },
      {
       "type": "li",
       "text": "**RNS monitors the brain continuously** and stores detection counts and short electrocorticography clips. These show how often seizures happen and at what time of day, and whether bilateral temporal seizures really come from both sides."
      },
      {
       "type": "li",
       "text": "**In all three trials, benefit kept rising** in the open-label years that followed: a median 75% reduction at 9 years with RNS, and 75% at 7 years with DBS."
      },
      {
       "type": "figure",
       "html": "neuromod-trials",
       "caption": "Seizure reduction in the blinded phases of the pivotal trials, and in long-term open-label follow-up. Open-label results have no control arm and include medication changes.",
       "n": 15
      },
      {
       "type": "li",
       "text": "**Stimulation of the centromedian thalamus** is being studied for generalized epilepsies such as Lennox–Gastaut syndrome."
      },
      {
       "type": "mcq",
       "id": "q-19eu0w8",
       "stem": "A 35-year-old has drug-resistant focal impaired awareness seizures. Intracranial EEG shows independent onsets from both hippocampi, and memory testing shows that each side still supports memory. What is the best option?",
       "options": [
        "Left anterior temporal lobectomy",
        "Bilateral anterior temporal lobectomy",
        "Responsive neurostimulation with a lead in each hippocampus",
        "Corpus callosotomy",
        "Hemispherotomy"
       ],
       "answer": 2,
       "explain": "Removing both hippocampi would cause severe amnesia. Responsive neurostimulation treats both foci without removing tissue, and its recordings show which side seizures come from over time."
      },
      {
       "type": "mcq",
       "id": "q-1pzhy3x",
       "stem": "Why is the vagus nerve stimulator placed on the left?",
       "options": [
        "The left vagus nerve is larger",
        "The right vagus nerve carries more fibers to the sinoatrial node, so right-sided stimulation risks bradycardia",
        "The left side is closer to the brainstem",
        "The right vagus nerve does not project to the nucleus tractus solitarius",
        "The left vagus nerve does not supply the larynx"
       ],
       "answer": 1,
       "explain": "The right vagus nerve provides most of the parasympathetic supply to the sinoatrial node. Left-sided stimulation avoids most cardiac effects; hoarseness still occurs because fibers of the left recurrent laryngeal nerve run in the vagus trunk under the electrode."
      },
      {
       "type": "mcq",
       "id": "q-1jnqwpt",
       "stem": "A 42-year-old with drug-resistant focal epilepsy and a history of severe depression is considering a device. Which trial finding is most relevant to this patient's counseling?",
       "options": [
        "VNS worsened depression in its pivotal trial",
        "Anterior thalamic DBS was associated with more reports of depression and memory problems",
        "RNS was associated with a high rate of suicide",
        "All three devices improve depression",
        "Devices have no psychiatric effects"
       ],
       "answer": 1,
       "explain": "In the SANTE trial, depression and memory complaints were more common with active stimulation. VNS, by contrast, is also approved for treatment-resistant depression."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-19oo3rl",
         "front": "VNS target and side",
         "back": "Left cervical vagus nerve."
        },
        {
         "id": "c-2l52ff",
         "front": "VNS side effects",
         "back": "Hoarseness, cough, throat discomfort and breathlessness during stimulation; can worsen sleep apnea."
        },
        {
         "id": "c-27cx1k",
         "front": "VNS magnet",
         "back": "Triggers an extra stimulation train to shorten or abort a seizure."
        },
        {
         "id": "c-1dn5fg6",
         "front": "RNS",
         "back": "Detects seizure patterns at one or two foci and stimulates in response; also records long-term ECoG."
        },
        {
         "id": "c-rqxq9r",
         "front": "RNS best fit",
         "back": "Two independent mesial temporal onsets, or a focus in eloquent cortex."
        },
        {
         "id": "c-19rkq69",
         "front": "DBS target in epilepsy",
         "back": "Anterior nucleus of the thalamus (SANTE trial)."
        },
        {
         "id": "c-162wzew",
         "front": "SANTE side effects to counsel on",
         "back": "Depression and memory complaints."
        },
        {
         "id": "c-134i9av",
         "front": "Devices over time",
         "back": "Benefit grows over years of stimulation; seizure freedom is uncommon."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "VNS pivotal trial (Handforth 1998)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/9674777/"
      },
      {
       "title": "Responsive neurostimulation at 9 years (Nair 2020)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/32690786/"
      },
      {
       "title": "SANTE at 7 and 10 years (Salanova 2021)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/33830503/"
      },
      {
       "title": "Responsive cortical stimulation pivotal trial (Morrell 2011)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/21917777/"
      },
      {
       "title": "Anterior thalamic stimulation: SANTE (Fisher 2010)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/20331461/"
      }
     ],
     "words": 766,
     "bank": 9
    }
   ],
   "intro": [
    "About a third of people with epilepsy keep having seizures despite medication. For many with focal epilepsy, surgery gives the best chance of seizure freedom, yet it is underused and usually comes late. This part follows a patient from referral, through the presurgical workup, to resection, ablation or a device."
   ]
  },
  {
   "id": "living",
   "n": "9",
   "title": "Living with epilepsy",
   "short": "Living with epilepsy",
   "sections": [
    {
     "id": "sudep-mortality",
     "n": "1",
     "title": "SUDEP and mortality",
     "tags": [
      "seizure-management"
     ],
     "updated": false,
     "goals": [
      "Define SUDEP and give its incidence",
      "name the strongest risk factor and what lowers risk",
      "counsel patients and families in plain numbers"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "Why people with epilepsy die early"
      },
      {
       "type": "p",
       "text": "Mortality in epilepsy is about two to three times that of the general population. In a Finnish cohort of children followed for 40 years, a quarter had died. Over half of those deaths were related to epilepsy: sudden unexpected death, seizures and drowning. Causes of excess death:"
      },
      {
       "type": "li",
       "text": "The underlying disease (tumor, stroke, neurodegenerative disease)"
      },
      {
       "type": "li",
       "text": "Sudden unexpected death in epilepsy (SUDEP)"
      },
      {
       "type": "li",
       "text": "Status epilepticus"
      },
      {
       "type": "li",
       "text": "Accidents, especially drowning"
      },
      {
       "type": "li",
       "text": "Suicide"
      },
      {
       "type": "li",
       "text": "Aspiration pneumonia"
      },
      {
       "type": "h3",
       "text": "Definition"
      },
      {
       "type": "p",
       "text": "SUDEP is a sudden, unexpected death in a person with epilepsy, witnessed or not, that is not caused by trauma, drowning or documented status epilepticus. It occurs with or without evidence of a seizure. Autopsy finds no toxic or structural cause of death."
      },
      {
       "type": "li",
       "text": "**Definite SUDEP**: an autopsy was done."
      },
      {
       "type": "li",
       "text": "**Probable SUDEP**: no autopsy was done."
      },
      {
       "type": "li",
       "text": "**SUDEP plus**: another condition, such as long QT syndrome, could have contributed."
      },
      {
       "type": "li",
       "text": "**Near-SUDEP**: resuscitation succeeded and the person survived more than an hour."
      },
      {
       "type": "li",
       "text": "**Not SUDEP**: any evidence of submersion in water excludes it, and so does status epilepticus lasting 30 minutes or more."
      },
      {
       "type": "h3",
       "text": "How common, and who is at risk"
      },
      {
       "type": "li",
       "text": "**Incidence**: about 1 in 4,500 children with epilepsy each year, and about 1 in 1,000 adults."
      },
      {
       "type": "li",
       "text": "**The major risk factor is generalized tonic–clonic seizures**, and risk rises with their frequency. Three or more a year carries a risk many times higher than none."
      },
      {
       "type": "li",
       "text": "**Other risk factors**: seizures during sleep, sleeping alone or unsupervised, young age at onset, long duration, a structural cause, and male sex. Polytherapy is linked to risk, largely because it marks severe epilepsy; the priority is fewer tonic–clonic seizures, not fewer drugs."
      },
      {
       "type": "figure",
       "html": "sudep-counsel",
       "caption": "The AAN and AES counseling numbers, as a picture a patient can keep: about 1 in 1,000 adults and 1 in 4,500 children with epilepsy each year.",
       "n": 16
      },
      {
       "type": "h3",
       "text": "What happens"
      },
      {
       "type": "p",
       "text": "Most SUDEP happens at night, often with the person found face down. Monitoring-unit cases (MORTEMUS) showed a consistent sequence after a generalized tonic–clonic seizure: rapid breathing, then, within minutes, failure of breathing and heart function. The terminal apnea came before the final cardiac arrest."
      },
      {
       "type": "h3",
       "text": "What lowers risk"
      },
      {
       "type": "li",
       "text": "**Seizure freedom, above all freedom from tonic–clonic seizures.** Treat actively and refer for surgery when appropriate."
      },
      {
       "type": "li",
       "text": "Take medicines reliably and avoid sleep deprivation and alcohol excess."
      },
      {
       "type": "li",
       "text": "**Nighttime supervision or a listening device** may help. Someone who can reposition the person and stimulate breathing may interrupt the sequence."
      },
      {
       "type": "li",
       "text": "**Wearable seizure detectors**: the ILAE and IFCN recommend clinically validated devices to detect tonic–clonic seizures, especially in people who sleep unsupervised, where an alarm can bring rapid help."
      },
      {
       "type": "note",
       "label": "Board pearl",
       "text": "Tell every patient, early and in plain numbers. The AAN and AES guideline advises telling adults that SUDEP affects about 1 in 1,000 adults with epilepsy each year, and that seizure freedom, especially from tonic–clonic seizures, strongly lowers the risk."
      },
      {
       "type": "mcq",
       "id": "q-10znrbq",
       "stem": "Which factor is most strongly associated with SUDEP?",
       "options": [
        "Number of ASMs taken",
        "Frequency of generalized tonic–clonic seizures",
        "Frequency of focal aware seizures",
        "Female sex",
        "Interictal spike frequency on EEG"
       ],
       "answer": 1,
       "explain": "Generalized (including focal to bilateral) tonic–clonic seizures are the major risk factor, and risk rises with how often they occur. The link with polytherapy largely reflects more severe epilepsy."
      },
      {
       "type": "mcq",
       "id": "q-1llneil",
       "stem": "The parents of a 7-year-old recently diagnosed with epilepsy ask about the risk of sudden death. What is the best answer?",
       "options": [
        "\"It is too rare to be worth discussing.\"",
        "\"About 1 in 100 children with epilepsy die suddenly each year.\"",
        "\"About 1 in 4,500 children with epilepsy each year; controlling seizures, especially convulsive ones, lowers the risk.\"",
        "\"The risk is the same as for any child.\"",
        "\"It only happens in people who do not take their medicine.\""
       ],
       "answer": 2,
       "explain": "The AAN and AES guideline advises giving the actual figure (1 in 4,500 children a year) and linking it to what families can do. Most families want to be told."
      },
      {
       "type": "mcq",
       "id": "q-1184gcz",
       "stem": "A 45-year-old is found dead in bed, face down, with a bitten tongue. Autopsy shows no anatomic or toxicologic cause. There is a history of drug-resistant focal epilepsy with nocturnal tonic–clonic seizures. How is this classified?",
       "options": [
        "Definite SUDEP",
        "Probable SUDEP",
        "Possible SUDEP",
        "Near-SUDEP",
        "Death from status epilepticus"
       ],
       "answer": 0,
       "explain": "A sudden, unexpected death in someone with epilepsy, with an autopsy showing no other cause, is definite SUDEP. Without an autopsy it would be probable SUDEP."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-137c9ni",
         "front": "SUDEP incidence in children",
         "back": "About 1 in 4,500 a year (0.22 per 1,000 patient-years)."
        },
        {
         "id": "c-1lxkb00",
         "front": "SUDEP incidence in adults",
         "back": "About 1 in 1,000 a year (1.2 per 1,000 patient-years)."
        },
        {
         "id": "c-1rop1v1",
         "front": "Major SUDEP risk factor",
         "back": "Frequency of generalized tonic–clonic seizures."
        },
        {
         "id": "c-enmkid",
         "front": "Definite versus probable SUDEP",
         "back": "Definite has an autopsy that finds no other cause; probable has no autopsy."
        },
        {
         "id": "c-aae5k3",
         "front": "Near-SUDEP",
         "back": "Cardiorespiratory arrest reversed by resuscitation, with survival for more than an hour."
        },
        {
         "id": "c-4c3p7m",
         "front": "MORTEMUS sequence",
         "back": "Tonic–clonic seizure, rapid breathing, then within minutes failure of breathing and heart function; terminal apnea before cardiac arrest."
        },
        {
         "id": "c-2q088e",
         "front": "Wearable seizure detectors",
         "back": "Recommended for tonic–clonic seizures, especially in people who sleep unsupervised."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "AAN and AES guideline: SUDEP incidence and risk factors (2017)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/28438841/"
      },
      {
       "title": "Unified SUDEP definitions (Nashef 2012)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/22191982/"
      },
      {
       "title": "Combined analysis of SUDEP risk factors (Hesdorffer 2011)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/21671925/"
      },
      {
       "title": "MORTEMUS (Ryvlin 2013)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/24012372/"
      },
      {
       "title": "Long-term mortality in childhood-onset epilepsy (Sillanpää and Shinnar 2010)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/21175314/"
      },
      {
       "title": "ILAE and IFCN guideline on wearable seizure detection (2021)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/33666944/"
      }
     ],
     "words": 796,
     "bank": 7
    },
    {
     "id": "comorbidities",
     "n": "2",
     "title": "Mood, behavior and cognition",
     "tags": [
      "seizure-management"
     ],
     "updated": false,
     "goals": [
      "Screen for depression, anxiety and suicidal thoughts",
      "treat depression safely alongside ASMs",
      "recognize peri-ictal psychiatric syndromes and the psychiatric effects of ASMs"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "How common"
      },
      {
       "type": "p",
       "text": "Depression and anxiety are the commonest psychiatric conditions in epilepsy, each affecting roughly one in four to one in three people. The relationship runs both ways: depression before the first seizure raises the risk of later epilepsy, and epilepsy raises the risk of depression. Depression predicts a worse quality of life more strongly than seizure frequency does, and suicide is more common than in the general population."
      },
      {
       "type": "h3",
       "text": "Screen"
      },
      {
       "type": "li",
       "text": "**In adults**: the Neurological Disorders Depression Inventory for Epilepsy (NDDI-E) is a six-item questionnaire designed so that ASM side effects are not mistaken for depression. A score above 15 suggests major depression."
      },
      {
       "type": "li",
       "text": "**In children and adolescents**: the ILAE recommends screening everyone for anxiety and depression, with closer watch from age 12."
      },
      {
       "type": "li",
       "text": "**Suicidal thoughts**: the FDA warning applies to all ASMs. It is a reason to ask about suicidal thoughts at every visit, not a reason to withhold treatment."
      },
      {
       "type": "h3",
       "text": "Treat"
      },
      {
       "type": "li",
       "text": "**Mild depression**: psychological treatment, such as cognitive behavioral therapy, comes first."
      },
      {
       "type": "li",
       "text": "**Moderate or severe depression**: SSRIs are the first-choice drugs, and at usual doses they do not meaningfully worsen seizures. Venlafaxine is a reasonable switch if an SSRI fails."
      },
      {
       "type": "li",
       "text": "**Duration**: continue for at least six months after remission, or nine months if there have been earlier episodes."
      },
      {
       "type": "li",
       "text": "**Avoid**: bupropion (especially immediate-release or at high doses), clomipramine, maprotiline and amoxapine lower the seizure threshold. Tricyclics are dangerous in overdose."
      },
      {
       "type": "li",
       "text": "**Check interactions**: enzyme-inducing ASMs lower levels of many antidepressants and antipsychotics. Fluoxetine and fluvoxamine can raise phenytoin levels."
      },
      {
       "type": "h3",
       "text": "Psychiatric effects of ASMs"
      },
      {
       "type": "li",
       "text": "**Can worsen mood or behavior**: levetiracetam (irritability, low mood, rarely psychosis), perampanel (aggression; boxed warning), topiramate (low mood, slowed thinking), zonisamide, and phenobarbital (depression in adults, hyperactivity in children)."
      },
      {
       "type": "li",
       "text": "**Can help mood**: lamotrigine, valproate and carbamazepine."
      },
      {
       "type": "li",
       "text": "**Personal risk matters**: a history of psychiatric illness raises the chance of behavioral side effects from levetiracetam and perampanel."
      },
      {
       "type": "h3",
       "text": "Psychiatric syndromes tied to seizures"
      },
      {
       "type": "li",
       "text": "**Postictal psychosis**: follows a cluster of seizures after a clear interval of hours to a few days. It lasts days, often recurs, and is more common with seizures arising from both hemispheres. Treat with short-term antipsychotics and prevent the clusters."
      },
      {
       "type": "li",
       "text": "**Alternative psychosis (\"forced normalization\")**: psychosis or mood change that appears when seizures stop abruptly and the EEG improves, for example after a new, effective ASM."
      },
      {
       "type": "li",
       "text": "**Interictal psychosis**: a chronic, schizophrenia-like illness in long-standing epilepsy."
      },
      {
       "type": "li",
       "text": "**Functional seizures**: these can coexist with epilepsy, so recording the typical event is essential before changing treatment."
      },
      {
       "type": "h3",
       "text": "Children and cognition"
      },
      {
       "type": "p",
       "text": "ADHD, autism and learning disability are common in childhood epilepsy. Methylphenidate is effective for ADHD in children with epilepsy and does not meaningfully worsen well-controlled seizures. Memory and word-finding complaints in adults have several causes: the epilepsy itself, ASMs (topiramate especially), depression and poor sleep. Treat each one."
      },
      {
       "type": "mcq",
       "id": "q-1oxoyag",
       "stem": "A 34-year-old with focal epilepsy scores 18 on the NDDI-E. The patient denies suicidal intent and has moderate depressive symptoms. Seizures are controlled on lamotrigine. What is the best treatment?",
       "options": [
        "Avoid all antidepressants because they provoke seizures",
        "Start an SSRI such as sertraline, and offer cognitive behavioral therapy",
        "Start bupropion",
        "Switch lamotrigine to levetiracetam",
        "Start clomipramine"
       ],
       "answer": 1,
       "explain": "SSRIs are first-choice drugs for moderate depression in epilepsy and do not meaningfully worsen seizures at usual doses. Cognitive behavioral therapy adds benefit. Bupropion and clomipramine lower the seizure threshold, and levetiracetam may worsen mood."
      },
      {
       "type": "mcq",
       "id": "q-p2jd6l",
       "stem": "Two days after a cluster of four focal to bilateral tonic–clonic seizures, a 40-year-old who had recovered to normal becomes agitated, paranoid and hears voices. What is the most likely diagnosis?",
       "options": [
        "Postictal psychosis",
        "Nonconvulsive status epilepticus",
        "New schizophrenia",
        "Alternative psychosis",
        "Levetiracetam toxicity"
       ],
       "answer": 0,
       "explain": "Postictal psychosis follows a seizure cluster after a clear interval of hours to days, then lasts days. EEG helps to exclude nonconvulsive status when the picture is unclear."
      },
      {
       "type": "match",
       "id": "match-psych-asm",
       "prompt": "Match each ASM to its psychiatric profile",
       "pairs": [
        [
         "Levetiracetam",
         "Irritability and low mood, especially with a psychiatric history"
        ],
        [
         "Perampanel",
         "Aggression and hostility; boxed warning"
        ],
        [
         "Lamotrigine",
         "Mood-stabilizing; used in bipolar depression"
        ],
        [
         "Topiramate",
         "Low mood and slowed thinking and word-finding"
        ],
        [
         "Phenobarbital",
         "Depression in adults, hyperactivity in children"
        ]
       ]
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-12emn0u",
         "front": "NDDI-E",
         "back": "Six-item depression screen for epilepsy; above 15 suggests major depression."
        },
        {
         "id": "c-tc1yqx",
         "front": "First-line drug for depression in epilepsy",
         "back": "An SSRI."
        },
        {
         "id": "c-tdxn3q",
         "front": "Antidepressants that lower the seizure threshold",
         "back": "Bupropion, clomipramine, maprotiline, amoxapine."
        },
        {
         "id": "c-609yrl",
         "front": "Duration of antidepressant treatment",
         "back": "At least six months after remission; nine if there were earlier episodes."
        },
        {
         "id": "c-1y47v4t",
         "front": "Postictal psychosis",
         "back": "After a seizure cluster and a clear interval of hours to days; lasts days; often recurs."
        },
        {
         "id": "c-10zt9pp",
         "front": "Alternative psychosis",
         "back": "Psychosis when seizures stop abruptly and the EEG improves (\"forced normalization\")."
        },
        {
         "id": "c-1gvt5tb",
         "front": "ADHD in children with epilepsy",
         "back": "Methylphenidate is effective and does not meaningfully worsen controlled seizures."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "NDDI-E validation (Gilliam 2006)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/16632310/"
      },
      {
       "title": "ILAE recommendations: treating depression in adults with epilepsy (2022)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/34866176/"
      },
      {
       "title": "ILAE recommendations: anxiety and depression in children with epilepsy (2024)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/39320421/"
      }
     ],
     "words": 747,
     "bank": 11
    },
    {
     "id": "safety-driving-law",
     "n": "3",
     "title": "Driving, safety, work and school",
     "tags": [
      "seizure-management"
     ],
     "updated": false,
     "goals": [
      "Counsel on driving using the seizure-free interval and local law",
      "give practical safety and first-aid advice",
      "know the protections available at work and school"
     ],
     "blocks": [
      {
       "type": "h3",
       "text": "Driving"
      },
      {
       "type": "p",
       "text": "In the United States, each state sets its own rules. Most require a seizure-free interval of three to twelve months before driving, and many allow exceptions:"
      },
      {
       "type": "li",
       "text": "Seizures only during sleep, in an established pattern"
      },
      {
       "type": "li",
       "text": "Auras that never impair awareness or control"
      },
      {
       "type": "li",
       "text": "A seizure after a doctor-directed medication change, once the previous regimen is restored"
      },
      {
       "type": "li",
       "text": "Provoked seizures whose cause has resolved"
      },
      {
       "type": "p",
       "text": "Most states rely on the driver to report. A few require physicians to report. Commercial driving across state lines has much stricter federal standards. Many clinicians also advise against driving during an ASM taper and for some months afterward."
      },
      {
       "type": "p",
       "text": "The evidence behind the seizure-free interval: in a case-control study, a seizure-free interval of 12 months or more lowered the odds of a seizure-related crash by 93%. A reliable warning aura also lowered the odds. Over half of the drivers who crashed were driving illegally, before their required seizure-free interval had passed, and a fifth had missed an ASM dose just before."
      },
      {
       "type": "note",
       "label": "Board pearl",
       "text": "Counsel every patient about driving, record that you did so, and check your own state's rules. The law is not the same everywhere."
      },
      {
       "type": "h3",
       "text": "Everyday safety"
      },
      {
       "type": "li",
       "text": "**Water**: take showers rather than baths, and never swim alone. Drowning is a leading cause of accidental death in epilepsy, often in a bathtub."
      },
      {
       "type": "li",
       "text": "**Heights, fire and tools**: avoid unprotected heights. Use the back burners of the stove and a microwave where possible. Use power tools with guards and cutoffs."
      },
      {
       "type": "li",
       "text": "**Common triggers**: missed doses, sleep deprivation and alcohol excess are the commonest causes of breakthrough seizures."
      },
      {
       "type": "li",
       "text": "**Photosensitive epilepsy**: sit farther from the screen, play in a well-lit room, avoid playing when tired, and cover one eye if flashing starts."
      },
      {
       "type": "h3",
       "text": "Seizure first aid"
      },
      {
       "type": "li",
       "text": "Stay with the person, time the seizure, and move hard objects away."
      },
      {
       "type": "li",
       "text": "After the convulsion, turn the person onto one side."
      },
      {
       "type": "li",
       "text": "Put nothing in the mouth, and do not hold the person down."
      },
      {
       "type": "li",
       "text": "**Give rescue medication**, as the seizure action plan directs, for a prolonged seizure or a cluster. Options include intranasal midazolam or diazepam, buccal midazolam and rectal diazepam."
      },
      {
       "type": "li",
       "text": "**Call emergency services** if a convulsion lasts 5 minutes or longer, or seizures repeat without recovery in between. Also call if the person is injured, pregnant or having trouble breathing, if the seizure happened in water, or if it is a first seizure."
      },
      {
       "type": "h3",
       "text": "Work and school"
      },
      {
       "type": "p",
       "text": "Under the Americans with Disabilities Act, epilepsy counts as a disability. Employers must make reasonable adjustments and cannot ask about health conditions before a job offer. At school, a written seizure action plan, and a 504 plan or individualized education program, secure rescue medication, supervision and learning support."
      },
      {
       "type": "case",
       "id": "case-driving",
       "title": "Back behind the wheel",
       "intro": [
        "A 24-year-old delivery driver has juvenile myoclonic epilepsy. The last generalized tonic–clonic seizure was 14 months ago, after a night without sleep. The patient has occasional morning myoclonic jerks, usually after a late night, and takes levetiracetam, occasionally missing evening doses."
       ],
       "steps": [
        {
         "narrative": [],
         "stem": "What is the most important first step in advising about driving?",
         "options": [
          "Say that nobody with epilepsy may drive",
          "Check the state's rules, discuss the seizure-free interval and triggers, and record the discussion",
          "Say that driving is fine because the last seizure was more than a year ago",
          "Report the patient to the licensing authority in every case",
          "Order an EEG and base the advice only on that"
         ],
         "answer": 1,
         "explain": "Driving rules vary by state and by the kind of license. Advice should cover the interval, the risk factors (sleep loss, missed doses) and how to reduce them, and the discussion should be documented."
        },
        {
         "narrative": [
          "The patient asks about driving a delivery truck across state lines."
         ],
         "stem": "What should the patient be told?",
         "options": [
          "The same rules apply as for a private car",
          "Interstate commercial driving has much stricter federal standards",
          "Commercial driving is allowed once seizure-free for three months",
          "Only the employer decides",
          "Epilepsy has no effect on commercial licensing"
         ],
         "answer": 1,
         "explain": "Interstate commercial vehicles fall under federal medical standards that are much stricter than state rules for private cars. The patient needs to check them before planning a career around interstate driving."
        },
        {
         "narrative": [
          "The patient wants to know how to lower the chance of another seizure."
         ],
         "stem": "Which advice matters most?",
         "options": [
          "Stop levetiracetam once two years seizure-free",
          "Regular sleep, taking every dose (a pill organizer or phone alarm), and limiting alcohol",
          "Avoid all screens",
          "Take an extra dose before driving",
          "Switch to carbamazepine"
         ],
         "answer": 1,
         "explain": "Sleep deprivation, missed doses and alcohol are the main triggers in juvenile myoclonic epilepsy, which usually needs lifelong treatment. Carbamazepine can worsen myoclonus."
        }
       ],
       "outro": [
        "Driving advice has three parts: the local law, the person's own risk and how to lower it, and a written record of the discussion."
       ]
      },
      {
       "type": "mcq",
       "id": "q-1t2b4wa",
       "stem": "Which situation most often permits driving before the usual seizure-free interval in many US states?",
       "options": [
        "Any focal seizure with impaired awareness",
        "A seizure caused by a doctor-directed medication change, once the previous regimen is restored",
        "Seizures that happen only while driving",
        "A patient who has stopped taking medication",
        "Seizures with a short postictal phase"
       ],
       "answer": 1,
       "explain": "Many states allow exceptions for seizures after a supervised medication change, seizures only during sleep, auras that never impair awareness, and provoked seizures whose cause has resolved."
      },
      {
       "type": "mcq",
       "id": "q-1ittl34",
       "stem": "A person has a convulsive seizure in a shopping center. It has lasted 3 minutes when you arrive. What should you do?",
       "options": [
        "Put something between the teeth to protect the tongue",
        "Hold the arms and legs still",
        "Time it, protect the head, move hard objects away, and call emergency services if it reaches 5 minutes",
        "Pour water on the face",
        "Leave to find a doctor"
       ],
       "answer": 2,
       "explain": "Time the seizure and keep the person safe. Nothing goes in the mouth, and restraint causes injury. Call emergency services at 5 minutes or if seizures repeat."
      },
      {
       "type": "cards",
       "items": [
        {
         "id": "c-1sy5sw4",
         "front": "Typical US seizure-free interval for driving",
         "back": "Three to twelve months, depending on the state."
        },
        {
         "id": "c-3rxvsf",
         "front": "Common driving exceptions",
         "back": "Sleep-only seizures, auras without impaired awareness, a seizure after a doctor-directed medication change, provoked seizures."
        },
        {
         "id": "c-b4lkft",
         "front": "Seizure-free for at least 12 months and crash risk",
         "back": "93% lower odds of a seizure-related crash in one case-control study."
        },
        {
         "id": "c-1lv6rrh",
         "front": "Bath or shower",
         "back": "Shower; drowning is a leading accidental death in epilepsy."
        },
        {
         "id": "c-k7os5d",
         "front": "When to call emergency services",
         "back": "Convulsion lasting 5 minutes or more, repeated seizures without recovery, injury, pregnancy, breathing trouble, a seizure in water, or a first seizure."
        },
        {
         "id": "c-ilax2d",
         "front": "Commonest causes of breakthrough seizures",
         "back": "Missed doses, sleep deprivation, alcohol."
        },
        {
         "id": "c-i2dvyr",
         "front": "ADA and epilepsy",
         "back": "Epilepsy counts as a disability; employers must make reasonable adjustments."
        }
       ]
      }
     ],
     "sources": [
      {
       "title": "Risk factors for seizure-related motor vehicle crashes (Krauss 1999)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/10227613/"
      },
      {
       "title": "AAN and AES guideline: SUDEP incidence and risk factors (2017)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/28438841/"
      },
      {
       "title": "ILAE and IFCN guideline on wearable seizure detection (2021)",
       "url": "https://pubmed.ncbi.nlm.nih.gov/33666944/"
      }
     ],
     "words": 924,
     "bank": 10
    }
   ],
   "intro": [
    "Epilepsy care does not end with seizure control. This part covers what kills people with epilepsy, the psychiatric and cognitive conditions that travel with it, and the practical questions raised at almost every clinic visit: driving, safety, work and school."
   ]
  }
 ]
}