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Juvenile myoclonic epilepsy , also known as Janz syndrome, is a fairly common form of generalized epilepsy of presumed genetic origin , and is also known as "Castels and Mendilaharsu Syndrome" in South America, representing 5-10% of all epilepsy cases. This disorder typically first manifests itself between the ages of 12 and 18 with sudden brief involuntary single or multiple episodes of muscle contractions caused by an abnormal excessive or synchronous neuronal activity in the brain. These events typically occur either early in the morning or upon sleep deprivation. JME reported as inherited idiopathic epilepsy syndrome.
Additional clinical presentations include seizures with either a motor or nonmotor generalized onset. The evidence of absence seizure is very rare in the patient with JME. Genetic studies have demonstrated several loci for JME and identified mutations in 4 genes.