Dravet Syndrome and Severe Myoclonic Epilepsy of Infancy (SMEI) - Short Review

Abstract

Dravet syndrome is a rare and genetic type of severe form of epilepsy characterized by episodes of prolonged, uncontrollable and progressive seizures that begin in the first year of life. It is also called myoclonic epilepsy of infancy, a rare type of epilepsy. It caused by mutations in the SCN1A gene and inherited from the parents in an autosomal dominant manner. Clinically features seen as frequent, prolong and refractory seizure, usually begins at first year of life and  seizures that last longer than five to 30 minutes or longer. Sudden unexpected death in epilepsy (SUDEP) is more chance in this type of epilepsy. Fifteen to 20% common cause of  death in sleep and also Intellectual  functions(IQ)  of child  may be deviated. It can be diagnosed by different various investigations as EEG, MRI, history, physical examination, clinical observation, genetic testing and so on but conform diagnosis may be very late.