Epileptic negative myoclonus restricted to lower limbs in benign childhood focal epilepsy with vertex spikes.

BACKGROUND AND PURPOSE: The aim was to determine the electroclinical findings in benign childhood focal epilepsy with vertex spikes (BEVS) with epileptic negative myoclonus (ENM) restricted to the lower limbs.

METHODS

The electroencephalogram database of Peking University First Hospital and medical records of patients with BEVS and ENM restricted to the lower limbs were reviewed.

RESULTS

Twenty-seven patients with BEVS had ENM restricted to the lower limbs. Twelve started as ENM restricted to the lower limbs. The age at seizure onset ranged from 1.5 to 4.8 years. During the course, half of the 12 patients developed focal sensorimotor seizures and then were diagnosed as benign childhood epilepsy with centrotemporal spikes (BECTS), with BEVS (four cases) and without BEVS (two cases). Five of them had electrical status epilepticus during sleep (ESES) and met the diagnostic criteria of atypical benign partial epilepsy (ABPE). Fifteen of the 27 patients had ENM restricted to the lower limbs during the course. The age at seizure onset ranged from 1.3 to 9.8 years. All had ESES and were diagnosed as ABPE, 11 as ABPE with BEVS and four as ABPE evolving into BEVS.

CONCLUSIONS

Benign childhood focal epilepsy with vertex spikes (BEVS) might represent a specific epileptic syndrome of the continuum of benign childhood focal epilepsy. ENM restricted to the lower limbs was a special phenomenon in BEVS. BEVS could overlap with BECTS or evolve into BECTS and further into ABPE and vice versa. Ignorance of vertex spikes with associated ENM restricted to the lower limbs might lead to a misunderstanding of BEVS, a specific type of benign childhood focal epilepsy.