{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,15]],"date-time":"2026-01-15T08:26:20Z","timestamp":1768465580959,"version":"3.49.0"},"reference-count":10,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2008,1,9]],"date-time":"2008-01-09T00:00:00Z","timestamp":1199836800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"\u201cthe Research Committee for Ataxic Diseases\u201d of the Research on Measures for Intractable Diseases from the Ministry of Health, Welfare and Labor, Japan"},{"name":"Scientific Research from the Ministry of Education, Culture, Sports, Science and Technology of Japan","award":["18790587"],"award-info":[{"award-number":["18790587"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[2008,4,15]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>We describe a patient with Huntington's disease (HD) who showed asymmetrical upper limb amyotrophy as a main manifestation. Chorea and psychiatric symptoms were not prominent. Electromyography revealed generalized active and chronic denervation and fasciculations. A genetic test showed 46 CAG repeats in the <jats:italic>huntingtin<\/jats:italic> gene. Asymmetrical amyotrophy restricted to the upper limb has been reported in some patients with progressive chorea and amyotrophy without acanthocytosis, but genetically proven cases of HD have rarely been reported. It is not known why only a few HD patients show the motor neuronal loss; however, certain as\u2010yet\u2010unidentified genetic factors combined with some environment factors and the underlying cellular dysfunctions by polyglutamine aggregation could be responsible for the motor neuronal loss similar to that in amyotrophic lateral sclerosis. \u00a9 2008 Movement Disorder Society<\/jats:p>","DOI":"10.1002\/mds.21937","type":"journal-article","created":{"date-parts":[[2008,1,9]],"date-time":"2008-01-09T19:16:40Z","timestamp":1199906200000},"page":"748-751","source":"Crossref","is-referenced-by-count":16,"title":["Genetically confirmed Huntington's disease masquerading as motor neuron disease"],"prefix":"10.1002","volume":"23","author":[{"given":"Kazuaki","family":"Kanai","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Satoshi","family":"Kuwabara","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Setsu","family":"Sawai","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Miho","family":"Nakata","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sonoko","family":"Misawa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sagiri","family":"Isose","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shigeki","family":"Hirano","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Naoki","family":"Kawaguchi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kaoru","family":"Katayama","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takamichi","family":"Hattori","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2008,1,9]]},"reference":[{"key":"e_1_2_7_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(03)13304-1"},{"key":"e_1_2_7_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(94)90191-0"},{"key":"e_1_2_7_4_2","doi-asserted-by":"publisher","DOI":"10.1007\/s004010050539"},{"key":"e_1_2_7_5_2","first-page":"63","article-title":"Huntington's chorea and chronic\u2010progressive spinal muscular atrophy","volume":"18","author":"Fotopulos D","year":"1966","journal-title":"Psychiatr Neurol Med Psychol (Leipz)"},{"key":"e_1_2_7_6_2","doi-asserted-by":"publisher","DOI":"10.1007\/s004150050610"},{"key":"e_1_2_7_7_2","doi-asserted-by":"publisher","DOI":"10.1002\/mds.10039"},{"key":"e_1_2_7_8_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-006-0267-z"},{"key":"e_1_2_7_9_2","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.32.11.841"},{"key":"e_1_2_7_10_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awh278"},{"key":"e_1_2_7_11_2","doi-asserted-by":"publisher","DOI":"10.1002\/1531-8257(199903)14:2<320::AID-MDS1018>3.0.CO;2-Z"}],"container-title":["Movement Disorders"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fmds.21937","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fmds.21937","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mds.21937","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/mds.21937","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mds.21937","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,20]],"date-time":"2025-10-20T10:44:57Z","timestamp":1760957097000},"score":1,"resource":{"primary":{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/10.1002\/mds.21937"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2008,1,9]]},"references-count":10,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2008,4,15]]}},"alternative-id":["10.1002\/mds.21937"],"URL":"https:\/\/doi.org\/10.1002\/mds.21937","archive":["Portico"],"relation":{},"ISSN":["0885-3185","1531-8257"],"issn-type":[{"value":"0885-3185","type":"print"},{"value":"1531-8257","type":"electronic"}],"subject":[],"published":{"date-parts":[[2008,1,9]]}}}