title: [Improvement of action myoclonus in a patient with dentatorubral-pallidoluysian atrophy by piracetam].
abstract: We report a 13-year-old girl with dentatorubal-pallidoluysian atrophy (DRPLA), presenting clinically as progressive myoclonic epilepsy. The action myoclonus, which severely impaired her daily life, was markedly improved by administration of piracetam, a drug reportedly useful for myoclonus of cortical origin. In our case, piracetam effectively suppressed severe subcortical myoclonus of DRPLA, suggesting that the drug may be useful in the treatment of both cortical, and subcortical myoclonus.
authors.affiliation: Tohoku University, School of Medicine, Sendai, Miyagi. mhirose@ped.med.tohoku.ac.jp
authors.forename: Mieko
authors.initials: M
authors.lastname: Hirose
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Yokoyama
authors.forename: Rie
authors.initials: R
authors.lastname: Noguchi
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Haginoya
authors.forename: Masashi
authors.initials: M
authors.lastname: Aoki
authors.forename: Kazuie
authors.initials: K
authors.lastname: Iinuma
mesh_terms: D000293:Adolescent; D002540:Cerebral Cortex; D005260:Female; D006801:Humans; D020191:Myoclonic Epilepsies, Progressive / Q000150:complications* / Q000188:drug therapy* / Q000209:etiology; D018696:Neuroprotective Agents / Q000627:therapeutic use*; D010889:Piracetam / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D018696:Neuroprotective Agents; D010889:Piracetam