management and prognosis of Lennox-Gastaut syndrome

Last edited 09/2018

An epilepsy syndrome with an age of onset of 3-10 years characterised by multiple seizure types (including atonic, tonic, tonic-clonic and atypical absence seizures), cognitive impairment and specific EEG features of diffuse slow spike and wave (< 2 Hz) as well as paroxysmal fast activity (10 Hz or more) in sleep.

Typically the fits are resistant to treatment, and may require a combination of anti-epileptic drugs.

  • NICE have suggested (2):

    Pharmacological treatment of Lennox-Gastaut syndrome

    First-line treatment in children with Lennox-Gastaut syndrome

    • discuss with, or refer to, a tertiary paediatric epilepsy specialist when a child presents with suspected Lennox-Gastaut syndrome
    • sodium valproate should be offered as first-line treatment to children with Lennox-Gastaut syndrome. Follow the MHRA safety advice on sodium valproate

    Adjunctive treatment in children, young people and adults with Lennox-Gastaut syndrome

    • lamotrigine should be offered as adjunctive treatment to children, young people and adults with Lennox-Gastaut syndrome if first-line treatment with sodium valproate is unsuitable, ineffective or not tolerated. Follow the MHRA safety advice on sodium valproate
    • discuss with a tertiary epilepsy specialist if adjunctive treatment is ineffective or not tolerated. Other AEDs that may be considered by the tertiary epilepsy specialist are rufinamide and topiramate
    • do not offer carbamazepine, gabapentin, oxcarbazepine, pregabalin, tiagabine or vigabatrin
    • felbamate should only be offered in centres providing tertiary epilepsy specialist care and when treatment with all of the AEDs has proved ineffective or not tolerated

Notes:

  • aketogenic diet is often prescribed - observational studies suggest that this may help some children with Lennox-Gastaut syndrome
  • surgery may help e.g. callostomy may reduce atonic drop attacks
  • long-term prognosis, in terms of cognitive function and spontaneous remission, is poor.

Reference:

  1. Drug and Therapeutics Bulletin (2001), 39 (2), 12-16.
  2. NICE (April 2018). Epilepsies: diagnosis and management