|
|
Juvenile myoclonic epilepsy is a recessively inherited epilepsy, genetic linkage having been established to chromosome 6. A history of epilepsy affecting other family members is frequently elicited (up to 5.5% in first-degree relatives). Neuroimaging typically reveals no abnormalities and EEG frequently reveals generalized spike-wave activity at a frequency of 4-6 hertz.
Clinical manifestations may include tonic-clonic seizures, absence seizures, and myoclonus most frequently involving the arms. Juvenile myoclonic epilepsy is distinct from other myoclonic epilepsies, which are often accompanied by other neurologic abnormalities or progressive symptoms and carry a much worse prognosis.
Valproic acid is the treatment of choice for juvenile myoclonic epilepsy and effectively relieves tonic-clonic and absence seizures in most cases. Treatment should be continued indefinitely in most cases, since risk of relapse upon discontinuation is particularly high in this condition.
| thoban@lumc.edu | Last Updated: August 11, 1996 Created: July 25, 1995 |