Lennox-Gastaut syndrome

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

Epidemiology

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

Clinical presentation

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.

Pathology

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.

Radiographic features

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

Treatment and prognosis

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.

History and etymology

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