Lennox-Gastaut syndrome

Last revised by Rohit Sharma on 24 May 2022

Lennox-Gastaut syndrome is an epileptic encephalopathy characterized by refractory childhood-onset epilepsy accompanied by intellectual disability and psychiatric/behavioral disturbance.

Lennox-Gastaut syndrome is thought to represent 1-2% of all patients with epilepsy 6. The condition most commonly manifests between the 2nd and 6th years of life, in some cases developing from earlier onset West syndrome or Ohtahara syndrome 6.

Lennox-Gastaut syndrome is characterized by frequent drug-refractory seizures (up to 200 times an hour 4) of multiple types (e.g. tonic seizures, atypical absence seizures, tonic-clonic seizures) 6. Affected patients also commonly have intellectual disability and associated developmental delay and psychiatric/behavioral manifestations 6.

An interictal electroencephalogram (EEG) often shows a slow spike and wave pattern or generalized paroxysmal fast activity 6.

The cause of Lennox-Gastaut syndrome is generally known in up to 75% of patients 6. The most common cause is a structural brain abnormality, but it is postulated that genetic causes may become increasingly recognized 6.

MRI brain should be obtained to evaluate for an underlying structural etiology 6.

Management is with antiseizure medications, usually requiring multiple agents in combination 6. Palliative surgical approaches are often considered, such as vagal nerve stimulator placement or corpus callosotomy 6.

It is named after William G Lennox (1884-1960), a US neurologist, and Henri Gastaut (1915-1995)a French neurologist 4,5.

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