Lennox–Gastaut syndrome

 

Presentation

Severe form of early onset (preschool age) epilepsy with multiple seizure types, including absences, stiffening (tonic) and drop attacks (atonic seizures).

LGS is usually symptomatic, (brain malformation, genetic cause or after brain injury); and sometimes develops after West syndrome or Ohtahara syndrome.

Disordered development before onset of seizures is commonly noted.

The epileptic ‘encephalopathy’ results in further deterioration of cognitive and behavioural issues.

Seizures are often drug-resistant and persist into adulthood.

 

If myoclonic seizures are prominent consider Doose syndrome.

 

Investigations

 – Interictal EEG shows widespread “slow spike and slow wave” (2–2.5 Hz) discharges.

 – Sleep EEG shows fast (10 – 12Hz) discharges.

 – Neuroimaging & metabolic/ genetic tests considered for underlying aetiology.

 

Management

Seizures are difficult to control with medications, often treated initially with Valproate or Clobazam. Polytherapy may include Lamotrigine, Levetiracetam, Rufinamide, or Topiramate.

Rescue medication may be offered for prolonged or cluster seizures.

Further management may include ketogenic diet, vagus nerve stimulation or surgery (Corpus callosotomy) under tertiary neurologist.

Provide input from therapists and educational support.