{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,3]],"date-time":"2026-06-03T04:39:10Z","timestamp":1780461550374,"version":"3.54.1"},"reference-count":38,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2004,5,26]],"date-time":"2004-05-26T00:00:00Z","timestamp":1085529600000},"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":[[2004,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Extrapyramidal features may occur in spinocerebellar ataxias consistent with neuropathological evidence of nigrostriatal involvement. Recently, striatal dopaminergic neurotransmission was found to be abnormal in the uncommon parkinsonian presentation of spinocerebellar ataxia type 2 (SCA2). We have investigated, therefore, striatal dopamine transporter and D2 receptor function in a series of 9 patients with the more common ataxic presentation of SCA2 using single photon emission computed tomography and \u03b2\u2010CIT as well as IBZM. Age\u2010matched healthy subjects and patients with Parkinson's disease (PD) served as controls. All except 1 SCA2 patient exhibited slowness of limb movements without rigidity or rest tremor. In addition, cervical dystonia was present in 5 and dystonic head tremor in 2 SCA2 patients. Striatocerebellar (S\/C) ratios of \u03b2\u2010CIT binding were significantly reduced in SCA2 patients compared to control subjects, and they were within the range of PD patients. S\/C ratios of IBZM binding were significantly reduced in SCA2 patients compared to control subjects. We conclude that dopaminergic neurotransmission is impaired in the ataxic presentation of SCA2, with a prominent loss of striatal dopamine transporter function. Both slowness of limb movements as well as dystonia in the ataxic SCA2 phenotype may reflect dysfunction not only at cerebellar but also at basal ganglia level. \u00a9 2004 Movement Disorder Society<\/jats:p>","DOI":"10.1002\/mds.20159","type":"journal-article","created":{"date-parts":[[2004,5,26]],"date-time":"2004-05-26T17:01:26Z","timestamp":1085590886000},"page":"1320-1325","source":"Crossref","is-referenced-by-count":33,"title":["Abnormalities of dopaminergic neurotransmission in SCA2: A combined <sup>123<\/sup>I\u2010\u03b2CIT and <sup>123<\/sup>I\u2010IBZM SPECT study"],"prefix":"10.1002","volume":"19","author":[{"given":"Sylvia M.","family":"Boesch","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eveline","family":"Donnemiller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00f6rg","family":"M\u00fcller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Klaus","family":"Seppi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helga","family":"Weirich\u2010Schwaiger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Werner","family":"Poewe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gregor K.","family":"Wenning","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2004,5,26]]},"reference":[{"key":"e_1_2_5_2_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng1196-285"},{"key":"e_1_2_5_3_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng1196-277"},{"key":"e_1_2_5_4_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng1196-269"},{"key":"e_1_2_5_5_2","first-page":"842","article-title":"The prevalence and wide clinical spectrum of the spinocerebellar ataxia type 2 trinucleotide repeat in patients with autosomal dominant cerebellar ataxia","volume":"60","author":"Geschwind DH","year":"1997","journal-title":"Am J Hum Genet"},{"key":"e_1_2_5_6_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.55.6.800"},{"key":"e_1_2_5_7_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/6.5.709"},{"key":"e_1_2_5_8_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/118.6.1573"},{"key":"e_1_2_5_9_2","doi-asserted-by":"publisher","DOI":"10.1007\/s004010050989"},{"key":"e_1_2_5_10_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.10055"},{"key":"e_1_2_5_11_2","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000035625.19871.DC"},{"key":"e_1_2_5_12_2","first-page":"774","article-title":"Confirmation of the SCA\u20102 locus as an alternative locus for dominantly inherited spinocerebellar ataxias and refinement of the candidate region","volume":"54","author":"Lopes\u2010Cendes IE","year":"1994","journal-title":"Am J Hum Genet"},{"key":"e_1_2_5_13_2","doi-asserted-by":"publisher","DOI":"10.1002\/mds.870130311"},{"key":"e_1_2_5_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1071-3581(97)90048-9"},{"key":"e_1_2_5_15_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.57.10.1495"},{"key":"e_1_2_5_16_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.54.2.124"},{"key":"e_1_2_5_17_2","doi-asserted-by":"publisher","DOI":"10.1002\/mds.870070202"},{"key":"e_1_2_5_18_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00839204"},{"key":"e_1_2_5_19_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/121.3.459"},{"issue":"4","key":"e_1_2_5_20_2","first-page":"835","article-title":"Clinical features and natural history of multiple system atrophy","volume":"117","author":"Wenning GK","year":"1994","journal-title":"An analysis of 100 cases. 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