{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,25]],"date-time":"2025-10-25T13:55:30Z","timestamp":1761400530660,"version":"build-2065373602"},"reference-count":42,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2007,12,11]],"date-time":"2007-12-11T00:00:00Z","timestamp":1197331200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[2008,2,15]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Spinocerebellar ataxia 6 (SCA6) is a hereditary disease characterized by a trinucleotide repeat expansion in the CACNA1A gene and late\u2010onset bilateral cerebellar atrophy. It is unclear if there is significant pathology outside of the cerebellum. We used transcranial magnetic stimulation to assess sensorimotor cortical circuits and cortical plasticity in 8 SCA6 patients and 8 age\u2010matched controls. Behavioral performance was assessed using a rhythmic tapping task. Neurophysiological measures of SCA6 patients showed a prolonged cortical silent period (CSP) but normal MEP recruitment curve, short\u2010latency afferent inhibition, long\u2010latency afferent inhibition and ipsilateral silent period. Paired\u2010associative stimulation induction also increased motor\u2010evoked potentials normally. SCA6 patients had greater variability with cued rhythmic tapping than normals and deteriorated when the cue was removed; in comparison, normal subjects had similar variability between cued and uncued rhythmic tapping. Analysis using a Wing\u2013Kristofferson timing model indicated that both clock variance and motor delay variance were abnormal. <jats:italic>Conclusion.<\/jats:italic> In SCA6, the circuits for sensorimotor integration and the mechanisms for LTP\u2010like plasticity in the sensorimotor cortex are unimpaired. A prolonged CSP in SCA6 just like in other cerebellar atrophies would suggest that this neurophysiological change typifies cerebellar dysfunction. \u00a9 2007 Movement Disorder Society<\/jats:p>","DOI":"10.1002\/mds.21847","type":"journal-article","created":{"date-parts":[[2007,12,11]],"date-time":"2007-12-11T19:18:40Z","timestamp":1197400720000},"page":"378-385","source":"Crossref","is-referenced-by-count":20,"title":["Prolonged cortical silent period but normal sensorimotor plasticity in spinocerebellar ataxia 6"],"prefix":"10.1002","volume":"23","author":[{"given":"James T.H.","family":"Teo","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Susanne A.","family":"Schneider","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Binith J.","family":"Cheeran","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Miguel","family":"Fernandez\u2010del\u2010Olmo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paola","family":"Giunti","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John C.","family":"Rothwell","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kailash P.","family":"Bhatia","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2007,12,12]]},"reference":[{"key":"e_1_2_7_2_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/6.8.1289"},{"key":"e_1_2_7_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(04)00737-9"},{"key":"e_1_2_7_4_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.49.5.1243"},{"key":"e_1_2_7_5_2","doi-asserted-by":"publisher","DOI":"10.1152\/jn.01293.2006"},{"key":"e_1_2_7_6_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200107053450103"},{"key":"e_1_2_7_7_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.64.1.67"},{"key":"e_1_2_7_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1388-2457(02)00378-4"},{"key":"e_1_2_7_9_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awf023"},{"key":"e_1_2_7_10_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1388-2457(03)00002-6"},{"key":"e_1_2_7_11_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/123.3.572"},{"issue":"3","key":"e_1_2_7_12_2","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1093\/brain\/awh065","article-title":"Does the representation of time depend on the cerebellum? 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