Department of Neurology, Hospital General Yagüe, Avda Cid 96, 09005, Burgos, Spain.
J Neurol. 2011 Feb;258(2):308-10. doi: 10.1007/s00415-010-5724-z. Epub 2010 Aug 31.
We present single photon emission computed tomography (SPECT) studies using 123IFP-CIT (DAT scan) and 123I-IBZM imaging, performed on four members of a family with genetically proven spinocerebellar ataxia type 3 (SCA-3). DAT scan showed significant asymmetric decreased binding in the striatum in the two members with predominant parkinsonian phenotype, with mild and symmetric decreased binding in the member with the cerebellar phenotype, and normal in the asymptomatic member. The IBZM SPECT studies showed mild and asymmetrical reduction of the striatal dopamine D2 receptor density (parkinsonian members). SCA-3 can present with a levodopa responsive parkinsonism phenotype, and an abnormal DAT scan showing predominant impairment of presynaptic dopamine function.
我们呈现了使用 123IFP-CIT（DAT 扫描）和 123I-IBZM 成像进行的单光子发射计算机断层扫描（SPECT）研究，这些研究对象是一个家族中的四名成员，他们患有遗传性小脑脊髓共济失调 3 型（SCA-3）。DAT 扫描显示，在两名以帕金森病表型为主的患者中，纹状体的结合明显不对称减少，在具有小脑表型的患者中，结合减少轻微且对称，而在无症状的患者中则正常。IBZM SPECT 研究显示，纹状体多巴胺 D2 受体密度轻度且不对称减少（帕金森病成员）。SCA-3 可表现为对左旋多巴反应性帕金森病表型，且 DAT 扫描异常，显示主要的突触前多巴胺功能受损。
