The ILAE Classification provides a framework for classifying seizures, epilepsies, and epilepsy syndromes. This page presents an overview of these classifications to help with the understanding and diagnosis of epilepsy.
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Epilepsy Syndromes with Onset in Neonates and Infants
Self‑Limited Neonatal Epilepsy (SeLNE)
Focal seizures starting between days 2 and 7 with normal development. Associated with KCNQ2KCNQ2 mutations affect potassium channels and are the most common cause of self-limited neonatal epilepsy. Most cases follow autosomal dominant inheritance. mutations.
Infantile Spasms
Clusters of spasms with chaotic EEG and developmental regression. Peak onset 4-7 months.
Early Infantile Epileptic Encephalopathy (EIEE)
Severe neonatal encephalopathy with tonic seizures and suppression-burst on EEG. Also known as Ohtahara syndromeNamed after Dr. Shunsuke Ohtahara who first described it in 1976. Often evolves into West syndrome (infantile spasms) after 3-6 months..
Epilepsy of Infancy with Migrating Focal Seizures (EIMFS)
Multifocal, migrating focal seizures with onset before 6 months. Associated with KCNT1KCNT1 encodes a sodium-activated potassium channel. Mutations lead to gain of function and increased excitability. Other genes involved include SCN1A, SCN2A, and KCNQ2. mutations.
Dravet Syndrome
Begins with prolonged, often fever-triggered seizures in the first year. Associated with SCN1ASCN1A gene provides instructions for making sodium channel subunits. Mutations lead to impaired function of inhibitory interneurons, causing hyperexcitability. 90% of mutations occur de novo. mutations (80% of cases).
Self‑Limited Focal Epilepsies of Childhood
Self‑Limited Epilepsy with Centrotemporal Spikes (SeLECTS)
Focal centrotemporal seizures during sleep with remission by adolescence. Formerly called BECTSBenign Epilepsy with Centrotemporal Spikes (BECTS) or Rolandic Epilepsy. Name changed to Self-Limited Epilepsy with Centrotemporal Spikes in the 2017 ILAE classification to avoid the term "benign" as cognitive and behavioral issues can occur..
Self-Limited Epilepsy with Autonomic Seizures (SeLEAS)
Onset in early childhood of focal autonomic seizures that are often prolonged. Formerly known as Panayiotopoulos syndromeNamed after Dr. Chrysostomos P. Panayiotopoulos. Key features include ictal vomiting, pallor, and eye deviation. Despite often prolonged seizures (>30 minutes in 40% of cases), prognosis is excellent..
Childhood Occipital Visual Epilepsy (COVE)
Focal visual seizures with occipital EEG findings and favorable prognosis. Formerly called Gastaut typeNamed after Henri Gastaut. Distinguished from migraine by brief duration of visual symptoms (seconds to minutes), stereotypical appearance, and potential evolution to other seizure types. of childhood occipital epilepsy.
Photosensitive Occipital Lobe Epilepsy (POLE)
Focal visual seizures triggered by light with characteristic occipital EEG patterns. Strong female predominanceFemale predominance is a common feature of photosensitive epilepsies, possibly related to hormonal influences on neural excitability..
Generalized Epilepsies of Childhood
Childhood Absence Epilepsy (CAE)
Brief staring spells with classic 3 Hz spike‑and‑wave EEG activity. Peak onset 5-7 years with multiple daily episodesChildren may have dozens to hundreds of absence seizures per day, each lasting 5-30 seconds. Often misinterpreted as daydreaming or inattention. Can be provoked by hyperventilation in about 90% of patients..
Epilepsy with Eyelid Myoclonia (EEM)
Eyelid myoclonia, with or without absences, induced by eye closure and photic stimulation. Also called Jeavons syndromeNamed after Peter Jeavons who first described it in 1977. Features the triad of eyelid myoclonia with or without absences, eye-closure sensitivity, and photosensitivity. Often resistant to medications..
Epilepsy with Myoclonic Atonic Seizures (EMAtS)
Myoclonic and atonic seizures causing drop attacks with generalized EEG findings. Previously called Doose syndromeNamed after Hermann Doose who described it in 1970. Characterized by diverse seizure types, with myoclonic-atonic seizures being the hallmark. Children often have normal development before seizure onset..
Epilepsy with Myoclonic Absence (EMA)
Very rare childhood epilepsy syndrome with daily myoclonic absence seizures. Features rhythmic 3 Hz jerkingDuring seizures, there is rhythmic myoclonic jerking at 3 Hz, often involving the shoulders and arms, with concomitant impaired awareness. Seizures typically last 10-60 seconds and may cause progressive drop of arms. with impaired consciousness.
Idiopathic Generalized Epilepsies (IGE)
Juvenile Absence Epilepsy (JAE)
Brief impaired awareness episodes with >3 Hz spike‑and‑wave EEG. Distinguished from CAE by later onset (10-16 years)Unlike CAE, JAE features less frequent absences but >90% develop generalized tonic-clonic seizures. Absences are typically less frequent than in CAE but may be longer in duration. and frequent GTCs.
Juvenile Myoclonic Epilepsy (JME)
Adolescent-onset epilepsy with myoclonic jerks and generalized polyspike‑and‑wave EEG. Prominent morning jerksMyoclonic jerks typically occur within 1-2 hours after awakening and can be triggered by sleep deprivation, alcohol, and stress. JME has a strong genetic component and usually requires lifelong treatment. with high photosensitivity.
Epilepsy with Generalized Tonic–Clonic Seizures Alone (GTCA)
Isolated generalized tonic–clonic seizures with normal interictal EEG in up to 50% of cases. Often sleep-relatedGTCA seizures frequently occur shortly after awakening (within the first 2 hours) or during relaxation periods. About 80% of patients respond well to appropriate anti-seizure medications..
Epilepsy Syndromes with Onset at a Variable Age
Sleep-Related Hypermotor Epilepsy (SHE)
Brief hyperkinetic seizures during sleep with focal frontal EEG abnormalities. Formerly called NFLENocturnal Frontal Lobe Epilepsy was renamed to Sleep-Related Hypermotor Epilepsy in 2017 because: 1) seizures can occur during daytime sleep, 2) the origin isn't always frontal, and 3) the semiology is more accurately described as hyperkinetic rather than just nocturnal..
Familial Mesial Temporal Lobe Epilepsy (FMTLE)
Focal temporal seizures with a positive family history. Features psychic and autonomic aurasCommon aura types include déjà vu, jamais vu, epigastric rising sensations, and fear. Unlike sporadic MTLE, FMTLE is typically not associated with hippocampal sclerosis and has a better prognosis with many achieving seizure freedom on medication., often well-controlled.
Familial Focal Epilepsy with Variable Foci (FFEVF)
Focal seizures with variable localization among family members. Associated with DEPDC5DEPDC5 gene mutations account for about 12-37% of FFEVF cases. This gene is part of the mTOR pathway regulation, and mutations can lead to cortical malformations including focal cortical dysplasia. mutations.
Epilepsy with Auditory Features (EAF)
Focal seizures with auditory auras and possible temporal EEG abnormalities. Familial form associated with LGI1LGI1 (Leucine-rich Glioma Inactivated 1) gene mutations are found in about 50% of familial cases, known as Autosomal Dominant Lateral Temporal Lobe Epilepsy (ADLTLE), but are rare in sporadic cases. mutations.
Autoimmune Epilepsies
Various syndromes with autoimmune etiology, including anti-NMDARAnti-NMDA receptor encephalitis typically affects young women and features psychiatric symptoms, memory deficits, seizures, and movement disorders. It's often responsive to immunotherapy, with 80% of patients achieving good outcomes., anti-LGI1, and GAD65 antibody-associated epilepsies.
Progressive Myoclonus Epilepsies (PME)
Severe myoclonic seizures with progressive neurological decline. Includes Unverricht-Lundborg diseaseCaused by mutations in the CSTB gene encoding cystatin B. The most common PME in Europe, especially Finland. Characterized by stimulus-sensitive myoclonus and tonic-clonic seizures with onset at 6-16 years, followed by progressive neurological deterioration., Lafora disease, and others.
Rasmussen Syndrome (RS)
Intractable seizures with progressive hemiplegia and unilateral EEG abnormalities. Features epilepsia partialis continuaEpilepsia partialis continua is a form of ongoing focal motor seizure activity that may continue for extended periods (days to years). In Rasmussen syndrome, it frequently affects the face and hand. Hemispherectomy may be required for seizure control. and unilateral atrophy.
Additional Resources
- Introduction to the Epilepsy Syndrome Papers (Epilepsia, 2022)
- Methodology for Classification and Definition of Epilepsy Syndromes (Epilepsia, 2022)
- ILAE Position Statement: Epilepsy Syndromes with Onset at a Variable Age (Epilepsia, 2022)
- ILAE Position Statement: Epilepsy Syndromes with Onset in Childhood (Epilepsia, 2022)
- ILAE Position Statement: Epilepsy Syndromes with Onset in Neonates and Infants (Epilepsia, 2022)
- ILAE Position Statement: Idiopathic Generalized Epilepsy Syndromes (Epilepsia, 2022)