Janz Syndrome (Youth Mioclonics), Two Brothers CaseReport and Literature Review

Abstract

Introduction: Juvenile myoclonic epilepsy classified as a generalized genetic epilepsy, It occurs with sudden jerks of the upper limbs and if the lower limbs are affected, with falls and without loss of consciousness. They occur preferably in the first minutes upon waking. They are triggered by sleep deprivation and alcohol intake. The disease follows a non-progressive course.

Clinical Case: A 15-year-old female patient presented to the service due to an absence crisis and an apparent insomnia problem. The semiology of absence crisis is interrogated and in effect, as well as occasional nocturnal myoclonus, electroencephalogram is performed and a 4 Hz slow wave poly tip pattern is found throughout the stroke, especially in photo stimulation (Figure 1, 2).

The patient has an older brother of 17 years who has developed school problems without a previous diagnosis of epilepsy, electroencephalogram is performed and also presents a generalized slow-wave poly tip pattern (Figure 3, 4) treatment with valproate is initiated with good response 

Conclusión: Juvenile myoclonic epilepsy forms at least one tenth of the epileptic syndromes, of a genetic nature with a family presentation that presents with repetitive abrupt movements. Valproic acid is a first-choice treatment and lifestyle changes and their triggers.