A 14 year old girl with epilepsia partialis continua complicated by palatal myoclonus of focal cortical origin is reported from the Comprehensive Epilepsy Center, Graduate Hospital; and University of Pennsylvania School of Medicine, Philadelphia, PA. Her seizures consisted of staring, impairment of consciousness, and left facial twitching. Later she developed intermittent stuttering speech in association with palatal and lingual movements occurring up to 70 times a day. Several episodes of epilepsia partialis continua, lasting up to two weeks, with noted in the next three years. These were characterized by left facial and pharyngeal movements. They were refractory to medications. The EEG revealed theta and delta activity in the right central region with intermixed spikes, and a SPECT showed a focal area of increased perfusion in the right centrotemporal region. The epilepsia partialis continua remitted after several weeks. [1]

COMMENT. The myoclonus epilepsies of childhood are divided into three major groups: (1) the progressive myoclonus epilepsies which include a number of degenerative and storage diseases; (2) the myoclonus epilepsies symptomatic of non-progressive brain damage usually of prenatal or perinatal origin; (3) the cryptogenic myoclonus epilepsies [2]. More than 15 specific disorders may cause the progressive myoclonus epilepsy syndrome; reviewed by Berkovic SF et al. [3]. The major causes of PME are the Unverricht-Lundborg type, Lafora disease, neuronal ceroid lipofuscinoses, MERRF and sialidoses. Myoclonus is best controlled by valproate and/or clonazepam.