Clinical Neurology · Child Neurology

Pediatric Epilepsy Syndromes

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Pediatric epilepsy syndromes grouped by age of onset — classification, clinical picture, EEG, treatment, and prognosis.

Syndrome	Class	Clinical picture	EEG	Treatment	Prognosis
Neonatal / Infantile period
Benign familial neonatal seizures (BFNS) "fifth-day fits"	Partial / generalized	Onset: first week after birth Clonic or myoclonic seizures AD inheritance — KCNQ2 and KCNQ3 mutation	Focal or multifocal	No treatment needed	Usually stop by 6 wks 16% risk of developing epilepsy
Benign familial infantile seizures (BFIS)	Partial	Focal clonic seizure, eye deviation, cyanosis Seizures often cluster	Occipital-parietal spikes	No treatment needed	Excellent; usually resolves in 1–2 yr
Benign myoclonic epilepsy of infancy	Generalized	Onset: 6 mo–3 yr Brief generalized myoclonic activity	Generalized spike/wave lasting 2–3 s	Valproate, lamotrigine	Remission in most cases
Early myoclonic encephalopathy (EME)	Generalized	Onset: first month of life Starts with erratic myoclonic jerks then simple focal sz then infantile spasms Multiple metabolic causes identified	Burst-suppression that evolves to hypsarrhythmia	AED — intractable	Poor — 50% die in few wks
Early infantile epileptic encephalopathy (EIEE) Ohtahara syndrome	Generalized	Tonic spasms — often hundreds daily 75% have lesions on MRI (often arising from cortical dysplasia or associated with STXBP1 "syntaxin binding protein" gene mutation)	Burst-suppression	Surgical eval	Poor — can evolve into West syndrome or Lennox-Gastaut syndrome
Infantile spasms & West syndrome	Generalized	Onset: infancy Infantile spasms: sudden jackknife-like movement, with flexion of the neck, trunk, limbs, and waist, occurring in clusters West syndrome: infantile spasms + developmental delay + hypsarrhythmia (may be associated with cortical dysplasia) Aicardi syndrome: infantile spasms + agenesis of the corpus callosum + retinal lacunae	Hypsarrhythmia (high-amplitude, chaotic slow waves with multifocal spikes); electrodecrement during spasms	Corticotrophin and vigabatrin	Poor
Severe myoclonic epilepsy in infancy — Dravet syndrome	Focal or generalized	Onset: first year, peak 2–8 mo Focal or GTC seizures; starts initially in setting of fever or vaccination; later, myoclonic and absence seizures Developmental arrest with onset of seizures SCN1A "sodium channel 1A" mutation	Slow background; multifocal or generalized spike-and-wave	Valproate, clobazam, ketogenic diet	Poor
Childhood period
Benign epilepsy with centrotemporal spikes (BECTS) — Rolandic epilepsy	Partial	Onset: school age, 15–25% of epilepsy in children Nocturnal SPS with clonic contractions of upper face and UL, excessive salivation, gurgling or choking sounds	Centrotemporal spikes with normal background	No treatment needed; CBZ if frequent Sz	Resolves by puberty; ⅔ infrequent seizures
Childhood epilepsy with occipital paroxysms — Panayiotopoulos	Partial	Onset: 1–14, average 5 years Nocturnal autonomic seizures of prolonged duration; child is conscious but complains about feeling sick, vomits, turns pale, dilated pupils ± visual seizures, thermoregulatory alterations Later they develop partial or GTCs	Interictal occipital spikes; increase in non-REM sleep	No treatment needed	20% develop ictal syncope w/wo convulsions
Idiopathic childhood occipital epilepsy of Gastaut	Partial	Onset: 3–14, average 8 Episodic blindness or colored luminous discs, visual hallucinations, lasting seconds or minutes Postictal migraine in one-third	Interictal high-amplitude spike-and-wave complexes occurring with the eyes closed	CBZ	50% with positive FH of epilepsy
Acquired epileptic aphasia — Landau-Kleffner syndrome	Partial	Onset: 3–8 years old Focal seizure involving face or arm, usually diagnosed initially as BECTS, then child develops acquired motor & sensory aphasia	Bilateral centrotemporal spikes, increased during sleep	Valproate, lamotrigine & steroids; avoid CBZ & PHT	Variable — many children become permanently aphasic
Continuous spike-wave activity during sleep (CSWS)	—	Onset: around 5 years Starts with partial or generalized seizures then develops epileptic encephalopathy	ESES appears 2–3 years after onset	Valproate, lamotrigine & steroids	Variable
Lennox-Gastaut syndrome	Generalized	Multiple seizure types (tonic, atonic, absence) + mental retardation + slow spike-wave (1.5–2.5 Hz)	Interictal: slow spike-and-wave patterns, ≤2.5 Hz	Valproate for all seizure types Lamotrigine & felbamate for drop attacks	Poor
Myoclonic-astatic epilepsy — Doose syndrome	Generalized	Onset: around 3 years Myoclonic, atonic, tonic, and absence seizures Sz may start in the setting of infection/fever	Slow background, generalized spike-and-wave pattern	Valproate, lamotrigine, levetiracetam, ketogenic diet	Variable — 50% will attain seizure freedom and normal IQ
Childhood absence epilepsy	Generalized	Brief (few seconds) staring episodes — behavioral arrest May include automatisms No postictal state — provoked by hypoglycemia and hyperventilation	3-Hz spike-and-wave pattern	Ethosuximide, valproate, and lamotrigine; D/C'd if seizure free for 1–2 yrs	Excellent — often remits by teenage yrs; should be distinguished from juvenile absence, atypical absence
Adolescent / Adult
Generalized epilepsy with febrile seizures plus — GEFS+	Generalized	Febrile seizures; myoclonic, astatic, tonic-clonic, and absence seizures Strong FHx, variable penetrance AD — SCN1A, SCN1B, SCN2A, and GABRG2 mutations	Normal, generalized or focal spike-and-wave	Variable	Variable presentation within families from benign to catastrophic
AD nocturnal frontal lobe epilepsy (ADNFLE)	Partial	Onset: variable, infants to elderly Brief hypermotor seizures (identical to those from SMA) that tend to cluster & occur mainly during sleep; includes fist clenching, arm throwing, leg cycling, yelling, moaning, sleep walking CHRNA encoding for nicotinic ACh Rc	Frontal sharp waves	Nicotine patch, carbamazepine, clonazepam	Can be misdiagnosed as night terrors or somnambulism
Progressive myoclonic epilepsy	Generalized	Cognitive decline + tonic-clonic, tonic, or myoclonic seizures Associated with: Lafora body disease, Unverricht-Lundborg disease (Baltic myoclonus), neuronal ceroid-lipofuscinosis, MERRF	—	Valproate (1st); lamotrigine & clonazepam (2nd line)	Progressive
Eyelid myoclonia with absences (Jeavons syndrome)	Generalized	Onset: peak around 6 years Triad of: eyelid myoclonia with and without absences; eye closure-induced seizures and EEG paroxysms; and photosensitivity (to flickering light) Eyelid myoclonia: jerking of the eyelids with jerky upward deviation of the eyeballs and the head for few seconds	Brief 3 to 6 Hz generalized poly-spike and wave discharge	Valproate, clonazepam & levetiracetam	—
Juvenile myoclonic epilepsy (JME)	Generalized	Onset: usually teenage (8–24) Myoclonic seizures on awakening GTC – absence seizure Myoclonus can be triggered by reading, talking, photic stimulation	Generalized 4- to 6-Hz polyspike-and-wave discharges in 75% of patients	Valproate, lamotrigine; levetiracetam, topiramate, and zonisamide can be tried	Good but requires lifelong treatment
Epilepsy with GTC seizures on awakening	Generalized	Onset: 2nd decade GTC on awakening; may have absence or myoclonic seizures	—	Similar to JME	—
Idiopathic photosensitive occipital lobe epilepsy	Reflex epilepsy	Onset: during puberty Occipital lobe seizures provoked by visual stimuli (TV, video games); mimic visual aura in migraine	Occipital or generalized spike-and-wave discharges enhanced with eye closure	Avoiding triggers ± valproate	—
Rasmussen encephalitis	Partial	Intractable and progressive focal seizures, hemiparesis, and cognitive regression Slowly progressive cortical atrophy on MRI Antibodies to glutamate receptor 3	—	Immunotherapy; hemispherectomy	Progressive
Autosomal dominant frontal lobe epilepsy	Partial	Hyperkinetic seizures at sleep-wake transition May have aura of fear CHRNA4 and CHRNB2 mutations	Normal or frontal spikes	Carbamazepine	Usually good response to treatment
Familial temporal lobe epilepsy	Partial	Lateral and mesial temporal forms	Temporal spike or sharp waves	Carbamazepine	Usually good response to treatment

Quick associations

Seizures starting with viral illness / fever: Febrile, GEFS+, Dravet, Doose
Seizures associated with spasms: Ohtahara, West, Aicardi

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