ILAE: Epilepsy Syndromes (Childhood)

• Table outlining various epilepsy syndromes, their clinical overviews, seizure characteristics, EEG findings, and treatment approaches.

Self-limited Epilepsy with Centrotemporal Spikes (SeLECTS)

• Onset occurs in early school years, involving focal clonic/tonic activity, often affecting the mouth/tongue.
• Seizures manifest with dysarthria, sialorrhea, dysphasia, typically unilaterally and often nocturnally, remitting by puberty.
• The person can also experience mouth movements and focal to bilateral tonic clonic seizures.
• EEG shows high-amplitude centrotemporal spikes, activated during drowsiness/sleep, possibly with bilateral sharp waves and a normal background
• Treatment is often not necessary; if needed, short-term ASM like carbamazepine can be considered.

Self-limited Epilepsy with Autonomic Seizures (SeLEAS)

• Onset is in early childhood, marked by focal seizures accompanied by autonomic symptoms.
• Seizures involve retching, vomiting, pallor, and abdominal discomfort; normal development; remits in 1-3 years.
• EEG shows high-amplitude spikes, either focal or multifocal, prolonged and enhanced during sleep, variable location.
• EEG spikes are mostly enhanced during sleep, variable location
• Observation is suitable for mild cases; ASM is used for frequent/prolonged seizures.

Childhood Occipital Visual Epilepsy (COVE)

• Visual seizures occur in late childhood, indicated by occipital EEG findings, more persistent than SeLECTS/SeLEAS.
• Focal aware seizures are characterized by visual hallucinations, sometimes leading to tonic-clonic seizures.
• EEG shows occipital spikes; photic sensitivity may be present.
• ASMs like valproate or levetiracetam should be used; avoid triggering factors in susceptible patients.

Photosensitive Occipital Lobe Epilepsy (POLE)

• Triggered by visual stimuli, specifically light, with occipital onset and photosensitivity.
• Focal visual seizures, often incited by flickering lights or patterns.
• EEG shows occipital spikes with generalized discharges during photic stimulation.
• Avoid triggers and use valproate or levetiracetam if necessary.

Epilepsy with Myoclonic-Atonic Seizures (EMAS)

• Onset occurs between 2-5 years, with generalized seizures including myoclonic and atonic features.
• These seizures can also be absence seizures
• Additional symptoms involve developmental impairment, GTCS, absence, and focal seizures may occur
• The EEG often shows generalized spike/polyspike-and-wave discharges.
• Valproate, ethosuximide, and levetiracetam work; ketogenic diet may also be an option.

Lennox-Gastaut Syndrome (LGS)

• A severe developmental and epileptic encephalopathy (DEE) that starts in early childhood, characterized by multiple seizure types, cognitive impairment, and poor prognosis.
• Involves tonic, atonic, atypical absence, GTCS, and focal seizures.
• Slow spike-and-wave patterns during wakefulness and and fast rhythms during sleep.
• Use valproate, lamotrigine, rufinamide, cannabidiol, or a ketogenic diet.

Epileptic Encephalopathy with SWAS (EE-SWAS)

• Marked regression in language/cognition with continuous spike-and-wave pattern during sleep.
• Various focal or generalized seizures occur, including epileptic spasms.
• EEG shows continuous spike-and-wave during >85% of non-REM sleep.
• Steroids, benzodiazepines, and valproate used; surgery in selected cases.

Febrile Infection-Related Epilepsy Syndrome (FIRES)

• Happens acutely after a febrile illness, leading to status epilepticus, causes refractory epilepsy.
• There is an explosive onset of focal/multifocal seizures with status epilepticus.
• A EEG shows diffuse slowing with multifocal discharges.
• Consider a ketogenic diet, immunotherapy, cannabinoids, and ASMs; has poor prognosis.

Hemiconvulsion-Hemiplegia-Epilepsy Syndrome (HHE)

• Manifests as post-febrile hemiconvulsions with persistent hemiplegia, followed by chronic focal epilepsy.
• Prolonged focal seizures lead to hemiplegia, later focal seizures.
• EEG indicates unilateral slowing and epileptiform discharges.
• Use ASM, hemicorticectomy in severe cases.