Myoclonus in childhood.
The term "myoclonus" sounds esoteric, yet it is part of our normal physiology, occurring as a muscle jerk on drowsiness or falling asleep, during rapid eye movement (REM) sleep, and as hiccoughs. Myoclonus is also a developmental feature of the human nervous system, comprising some of the earliest fetal movements. In pathologic settings, myoclonus may be the only neurologic abnormality, as in essential myoclonus, but more often it is one symptom of a larger neurologic problem. The vast etiologic spectrum of symptomatic myoclonus can be bewildering, but defining the underlying problem may provide the opportunity to develop specific therapies. Otherwise, treatment is merely symptomatic. The approach to the patient should be to verify the nature of the movement disorder and establish a specific etiologic diagnosis. A battery of neurophysiologic, neuroradiologic, and other laboratory studies is needed to localize the origin of the myoclonus and identify causative lesions. Drug treatment is largely empiric but must be systematic and aimed at restoring activities of everyday living. Unlike in epilepsies, in myoclonus multiple drugs usually must be combined to attain functional improvement.
Pranzatelli
Michael R
MR
Department of Neurology, National Pediatric Myoclonus Center, Southern Illinois University School of Medicine, Springfield, IL 62702, USA.
D000818:Animals; D000927:Anticonvulsants / Q000627:therapeutic use; D001259:Ataxia / Q000175:diagnosis; D001327:Autoimmune Diseases / Q000175:diagnosis; D002648:Child; D004195:Disease Models, Animal; D004827:Epilepsy / Q000175:diagnosis; D006801:Humans; D009207:Myoclonus / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D020258:Neurotoxicity Syndromes / Q000175:diagnosis
D000927:Anticonvulsants