management and prognosis of juvenile myoclonic epilepsy

Last edited 09/2018 and last reviewed 05/2021

Spontaneous remission before puberty occurs in fewer than 20% of children with juvenile myoclonic epilepsy. Also drug withdrawal is often unsuccessful even after 2 or more years of seizure-freedom, with at least 70% of patients relapsing. Often lifelong treatment will be required to ensure seizure-freedom.

Pharmacological treatment of childhood absence epilepsy, juvenile absence epilepsy or other absence epilepsy syndromes

First-line treatment in children, young people and adults with childhood absence epilepsy, juvenile absence epilepsy or other absence epilepsy syndromes

  • ethosuximide or sodium valproate should be offered as first-line treatment to children, young people and adults with absence syndromes. If there is a high risk of GTC seizures, offer sodium valproate first, unless it is unsuitable. Follow the MHRA safety advice on sodium valproate
  • lamotrigine should be offered if ethosuximide and sodium valproate are unsuitable, ineffective or not tolerated. Follow the MHRA safety advice on sodium valproate

Adjunctive treatment in children, young people and adults with childhood absence epilepsy, juvenile absence epilepsy or other absence epilepsy syndromes

  • if two first-line AEDs are ineffective in children, young people and adults with absence epilepsy syndromes, consider a combination of two of these three AEDs as adjunctive treatment: ethosuximide, lamotrigine or sodium valproate. Follow the MHRA safety advice on sodium valproate
  • if adjunctive treatment is ineffective or not tolerated, discuss with, or refer to, a tertiary epilepsy specialist and consider clobazam, clonazepam, levetiracetam, topiramate or zonisamide
  • do not offer carbamazepine, gabapentin, oxcarbazepine, phenytoin, pregabalin, tiagabine or vigabatrin

Reference:

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