We presented a unique case of DS with focal cortical myoclonus on conventional EEG. A visible spike wave from right central-parietal cortex immediately preceded a left myoclonic muscle activity, while a visible spike wave from left central-parietal cortex immediately preceded a right myoclonic muscle activity. The focal myoclonus was noted by physician when he was six months old, and was not noted when thirteen months old from a 24-h video monitoring EEG.
Myoclonic seizures with generalized or multiple spike-waves in DS have been reported to be isolated or grouped in brief bursts of two or three jerks [
6,
7]. Focal myoclonus is muscle jerks resulting from epileptic discharges in the cerebral cortex [
8]. Kobayashi et al. reported a female children with DS with a segmental myoclonus without obvious spike-wave discharge on conventional EEG [
9]. Conventional EEG of our case showed that a left myoclonic muscle activity originated from a spike wave form right central-parietal cortex, while a right myoclonic muscle activity originated from a spike wave form left central-parietal cortex.Our case further verified that focal cortical myoclonus was a seizure pattern of DS. The underlying mechanisms of focal cortical myoclonus in our case may be cortical hyperexcitability because of no obvious structural brain lesions. Other rare forms of seizure of DS with
SCN1A mutation were reported such as photosensitive myoclonic absence seizures, intermittent photic stimulation-induced occipital seizures, musicogenic reflex seizures, and seizures triggered by perineal stimulation or diaper change [
10‐
14]. Although the seizures of DS are intractable, the interictal EEG remains free of epileptic discharges during the first year of life [
15]. The female case with DS with a focal myoclonus, provided by Kobayashi et al., began to present recurrent generalized tonic–clonic seizures at two months of age, and the interictal EEGs were not remarkable [
9]. She presented erratic segmental myoclonus when was admitted at six months, but the disappearance time it was not mentioned [
10]. Our case was also at the age of 2 months when beginning to experience a prolonged hemiconvulsive seizure, but interictal EEG was abnormal during the first year of life. Focal myoclonic of our case with was noted at the age of six months, and not noted at thirteen months old. The two cases suggested that focal cortical myoclonus usually appeared, and may disappear during the first year of life. The limitation of this case report is the small number of cases, so more DS cases are needed to fully grasp the characteristics of focal cortical myoclonus to provide some diagnostic clues for DS.