{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T09:58:14Z","timestamp":1792058294262,"version":"4.4.0"},"reference-count":21,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2004,10,12]],"date-time":"2004-10-12T00:00:00Z","timestamp":1097539200000},"content-version":"vor","delay-in-days":3937,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[1994,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>We describe three patients with Huntington's disease, from two families, in whom myoclonus was the predominant clinical feature. The diagnosis was confirmed at autopsy in two cases and by DNA analysis in all three. These patients all presented before the age of 30 years and were the offspring of affected fathers. Neurophysiological studies documented generalised and multifocal action myoclonus of cortical origin that was strikingly stimulus sensitive, without enlargement of the cortical somatosensory evoked potential. The myoclonus improved with piracetam therapy in one patient and a combination of sodium valproate and clonazepam in the other two. Cortical reflex myoclonus is a rare but disabling component of the complex movement disorder of Huntington's disease, which may lead to substantial diagnostic difficulties.<\/jats:p>","DOI":"10.1002\/mds.870090609","type":"journal-article","created":{"date-parts":[[2004,12,31]],"date-time":"2004-12-31T11:38:19Z","timestamp":1104493099000},"page":"633-641","source":"Crossref","is-referenced-by-count":60,"title":["Cortical myoclonus in huntington's disease"],"prefix":"10.1002","volume":"9","author":[{"given":"P. D.","family":"Thompson","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K. P.","family":"Bhatia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"Brown","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. B.","family":"Davis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Pires","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N. P.","family":"Quinn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"Luthert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Honovar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. D.","family":"O'Brien","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C. D.","family":"Marsden","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. E.","family":"Harding","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2004,10,12]]},"reference":[{"key":"e_1_2_1_2_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.17.6.587"},{"key":"e_1_2_1_3_2","first-page":"144","article-title":"Rigidity as a form of Huntington's disease","volume":"28","author":"Barrows HS","year":"1963","journal-title":"Bull Los Angeles Neurol Soc"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1963.00460090050005"},{"key":"e_1_2_1_5_2","first-page":"353","article-title":"Etude anatomoclinique d'une forme rigide et myoclonique de maladie de Huntington infantile","volume":"124","author":"Fau R","year":"1971","journal-title":"Rev Neurol"},{"key":"e_1_2_1_6_2","first-page":"82","article-title":"Intention myoclonus in Huntington's disease","volume":"41","author":"Novom S","year":"1976","journal-title":"Bull Los Angeles Neurol Soc"},{"key":"e_1_2_1_7_2","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1177\/155005948001100202","article-title":"Myoclonic and major seizures in early adult Huntington's chorea case report and electroclinical findings","volume":"11","author":"Kereshi S","year":"1980","journal-title":"Clin Electroencephalogr"},{"key":"e_1_2_1_8_2","first-page":"189","article-title":"Myoclonus and Huntington's chorea description of a case","volume":"3","author":"Previdi P","year":"1980","journal-title":"Ital J Neurol Sci"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410290217"},{"key":"e_1_2_1_10_2","doi-asserted-by":"publisher","DOI":"10.1002\/mds.870080216"},{"key":"e_1_2_1_11_2","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1016\/0092-8674(93)90585-E","article-title":"Huntington's Disease Collaborative Group. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes","volume":"72","year":"1993","journal-title":"Cell"},{"key":"e_1_2_1_12_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/2.6.635"},{"key":"e_1_2_1_13_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng0893-387"},{"key":"e_1_2_1_14_2","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.25.9.589"},{"key":"e_1_2_1_15_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.44.9.829"},{"key":"e_1_2_1_16_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.29.8.1107"},{"key":"e_1_2_1_17_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410340213"},{"key":"e_1_2_1_18_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/114.5.2333"},{"key":"e_1_2_1_19_2","doi-asserted-by":"publisher","DOI":"10.1016\/0168-5597(90)90055-I"},{"key":"e_1_2_1_20_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/116.1.87"},{"key":"e_1_2_1_21_2","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(93)90315-P"},{"key":"e_1_2_1_22_2","unstructured":"ThompsonPD DayBL RothwellJC et al. The myoclonus in corticobasal degeneration evidence for two forms of cortical reflex myoclonus.Brain(in press)."}],"container-title":["Movement Disorders"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fmds.870090609","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mds.870090609","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,20]],"date-time":"2025-10-20T14:07:56Z","timestamp":1760969276000},"score":1,"resource":{"primary":{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/10.1002\/mds.870090609"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1994,1]]},"references-count":21,"journal-issue":{"issue":"6","published-print":{"date-parts":[[1994,1]]}},"alternative-id":["10.1002\/mds.870090609"],"URL":"https:\/\/doi.org\/10.1002\/mds.870090609","archive":["Portico"],"relation":{},"ISSN":["0885-3185","1531-8257"],"issn-type":[{"value":"0885-3185","type":"print"},{"value":"1531-8257","type":"electronic"}],"subject":[],"published":{"date-parts":[[1994,1]]}}}