Sign Up for a Free Account
  • Updated 01.18.2021
  • Released 10.18.1993
  • Expires For CME 01.18.2024

Juvenile myoclonic epilepsy

Introduction

Overview

In this updated article, the author discusses evidence concerning brain network damage and dysfunction, genetic factors, as well as prognosis and antiepileptic drug treatment in juvenile myoclonic epilepsy.

Key points

• Juvenile myoclonic epilepsy is a form of idiopathic generalized epilepsy, also defined as genetic generalized epilepsy. It is characterized by (a) myoclonic jerks (cardinal symptom) that are most frequent in the early morning and (b) generalized tonic-clonic seizures. Typical absence seizures may also occur, but these are infrequent and short and are often ignored by the patient.

• The differential diagnosis includes other types of genetic generalized epilepsies, juvenile absence epilepsy, and generalized tonic-clonic seizures alone (formerly known as generalized tonic-clonic seizures on awakening).

• Although juvenile myoclonic epilepsy has been considered a long-lasting condition, with frequent seizure relapses after withdrawal of medication, studies have shown that a proportion of patients become seizure-free off medication.

• Sodium valproate is the most effective medicine; however, the high risk of fetal malformations and other side effects limit its use in young women. Lamotrigine, levetiracetam, and brivaracetam are good alternatives, but lamotrigine may exacerbate myoclonus. Benzodiazepines may have an adjunctive role, in particular clobazam or clonazepam.

• Lifestyle advice is an integral part of the treatment of juvenile myoclonic epilepsy. Patients should avoid sleep deprivation and drinking alcohol.

Historical note and terminology

Juvenile myoclonic epilepsy was first reported in France by Herpin (44). The terminology was variable until Janz and his colleagues in Germany reported 47 cases and proposed the name "impulsive petit mal" as a clinically definable epileptic syndrome (49; 48). The syndrome was later called juvenile myoclonic epilepsy (of Janz) in the English-speaking literature (01; 19; 16).

Juvenile myoclonic epilepsy is one of the most common “electroclinical” syndromes within the idiopathic generalized epilepsies (72; 26). There are 4 primary and well‐established epilepsy syndromes within the idiopathic generalized epilepsies: childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy, and generalized tonic-clonic seizures alone (formerly known as generalized tonic-clonic seizures on awakening) (72). There has been a debate as to whether to define this group of syndromes as idiopathic generalized epilepsies or genetic generalized epilepsies as both terms have pros and cons (05; 72). The ILAE Commission on Classification and Terminology decided to keep both terms to define this group of generalized epilepsies (72).

Start a MedLink Neurology trial
to unlock 3 free articles.

  • Nearly 3,000 illustrations, 
including video clips of 
neurologic disorders.

  • Every article is reviewed by our esteemed Editorial Board for accuracy and currency.

  • Full spectrum of 
neurology in 1,200 
comprehensive articles.

Questions or Comment?

MedLink, LLC

10393 San Diego Mission Rd, Suite 120

San Diego, CA 92108-2134

Toll Free (U.S. + Canada): 800-452-2400

US Number: +1-619-640-4660

Support: service@medlink.com

Editor: editor@medlink.com