A case of Dravet syndrome with focal cortical myoclonus

Abstract Background Dravet syndrome (DS) is a severe epileptic encephalopathy in children dominated by polymorphic seizures. Focal cortical myoclonus indicated on conventional electroencephalogram (EEG) was rarely observed DS. Case presentation The child, boy, thirteen months old, suffered from clonic seizures during bathing at two old. Later he recurrent afebrile or febrile generalized tonic–clonic often developing into status epilepticus. A genetic analysis of the SCN1A gene revealed de novo heterozygous frame shift mutation exon 21(c.3836_c.3837del AT).His myoclonic jerks unilateral arm occurred spontaneously response to movement. spike wave right central-parietal cortex immediately preceded left muscle activity, while activity. onset detected activity 42 ms using jerk-locked back-averaging data. focal not noted when one year Conclusions could be form first life DS, which may broaden types DS and provide some diagnostic clues for