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ALL / Neurology

Epilepsy in children: syndromes

Epilepsy in children: syndromes

Infantile spasms (West's syndrome)
  • brief spasms beginning in the first few months of life
  • key features:
    • flexion of head, trunk, limbs → extension of arms (Salaam attack); last 1-2 secs, repeat up to 50 times
    • progressive mental handicap
    • EEG: hypsarrhythmia
  • usually secondary to serious neurological abnormality (e.g. tuberous sclerosis, encephalitis, birth asphyxia) or may be idiopathic
  • possible treatments include vigabatrin and steroids
  • has a poor prognosis

Typical (petit mal) absence seizures
  • onset 4-8 yrs
  • duration few-30 secs; no warning, quick recovery; often many per day
  • EEG: 3Hz generalized, symmetrical
  • sodium valproate, ethosuximide
  • good prognosis: 90-95% become seizure free in adolescence

Lennox-Gastaut syndrome
  • may be an extension of infantile spasms
  • onset 1-5 yrs
  • features:
    • atypical absences, falls, jerks
    • 90% moderate-severe mental handicap
    • EEG: slow spike
  • treatment: ketogenic diet may help

Benign rolandic epilepsy
  • most common in childhood, more common in males
  • features: paraesthesia (e.g. unilateral face), usually on waking up

Juvenile myoclonic epilepsy (Janz syndrome)
  • typical onset is in the teenage years, more common in girls
  • features:
    • infrequent generalized seizures, often in morning//following sleep deprivation
    • daytime absences
    • sudden, shock-like myoclonic seizure (these may develop before seizures)
  • treatment: levetiracetam
+PassMedicine Notes
Infantile spasms (West's syndrome)
  • brief spasms beginning in the first few months of life
  • key features:
    • flexion of head, trunk, limbs --> extension of arms (Salaam attack); last 1-2 secs, repeat up to 50 times
    • progressive mental handicap
    • EEG: hypsarrhythmia
  • usually secondary to serious neurological abnormality (e.g. tuberous sclerosis, encephalitis, birth asphyxia) or may be idiopathic
  • possible treatments include vigabatrin and steroids
  • has a poor prognosis
Typical (petit mal) absence seizures
  • onset 4-8 yrs
  • duration few-30 secs; no warning, quick recovery; often many per day
  • EEG: 3Hz generalized, symmetrical
  • sodium valproate, ethosuximide
  • good prognosis: 90-95% become seizure free in adolescence
Lennox-Gastaut syndrome
  • may be an extension of infantile spasms
  • onset 1-5 yrs
  • features:
    • atypical absences, falls, jerks
    • 90% moderate-severe mental handicap
    • EEG: slow spike
  • treatment: ketogenic diet may help
Benign rolandic epilepsy
  • most common in childhood, more common in males
  • features: paraesthesia (e.g. unilateral face), usually on waking up
Juvenile myoclonic epilepsy (Janz syndrome)
  • typical onset is in the teenage years, more common in girls
  • features:
    • infrequent generalized seizures, often in morning//following sleep deprivation
    • daytime absences
    • sudden, shock-like myoclonic seizure (these may develop before seizures)
  • treatment: levetiracetam


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