Parkinson's Disease and Movement Disorders Unit, Neurology Service, Institut Clínic de Neurociències (ICN), Hospital Clínic i Universitari, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Centro de Investigación en Red de Enfermedades Neurodegenerativas (CIBERNED), Villarroel 170, 08036, Barcelona, Catalunya, Spain.
J Neurol. 2011 Dec;258(12):2248-53. doi: 10.1007/s00415-011-6108-8. Epub 2011 Jun 3.
Nigrostriatal involvement is considered an additional feature in the new consensus criteria for the diagnosis of the cerebellar variant of multiple system atrophy (MSA-C). However, so far, only a few studies, which include a relative small number of patients, give support to this criterion. Our objective was to assess nigrostriatal dopaminergic innervation in patients with MSA-C without parkinsonism by use of dopamine transporter single photon emission computed tomography (DAT SPECT). Thirteen patients that fulfilled criteria for possible or probable MSA-C and presented no parkinsonian signs, and 12 age-matched healthy controls underwent ((123)I-2-β-carbomethoxy-3β-(4-iodophenyl)-N-(3-fluoropropyl) nortropane ([(123)I]FP-CIT) SPECT. Patients were also evaluated through the Unified Multiple System Atrophy Rating Scale (UMSARS) and brain magnetic resonance imaging (MRI). The mean duration of the cerebellar syndrome was 3.8 ± 1.7 years. DAT SPECT showed a significant decrease of striatal [(123)I]FP-CIT uptake ratios in patients (p < 0.001). Radiotracer uptake reduction was 21% in the entire striatum, 19% in putamen, and 24% in caudate nuclei. Striatal binding ratios were within the normal range in 3 patients. We did not find correlation between striatal uptake and disease duration, age of patients, UMSARS-II score, and pontine diameter. [(123)I]FP-CIT SPECT shows that most but not all MSA-C patients without parkinsonism have subclinical nigrostriatal dopaminergic denervation which is not related to disease duration, cerebellar dysfunction, or pontine atrophy.
纹状体受累被认为是多系统萎缩（MSA）小脑变异型新诊断共识标准的附加特征。然而，到目前为止，只有少数研究，包括相对较少的患者，支持这一标准。我们的目的是通过使用多巴胺转运蛋白单光子发射计算机断层扫描（DAT SPECT）评估无帕金森病的 MSA-C 患者的黑质纹状体多巴胺能神经支配。13 名符合可能或可能 MSA-C 标准且无帕金森病体征的患者和 12 名年龄匹配的健康对照者接受了（123）I-2-β- carbomethoxy-3β-（4-iodophenyl）-N-（3-fluoropropyl） nortropane([123]I)FP-CIT)SPECT。患者还通过统一多系统萎缩评分量表（UMSARS）和脑磁共振成像（MRI）进行评估。小脑综合征的平均持续时间为 3.8±1.7 年。DAT SPECT 显示患者纹状体[123]I]FP-CIT 摄取比值显著降低（p<0.001）。整个纹状体的放射性示踪剂摄取减少 21%，壳核减少 19%，尾状核减少 24%。3 名患者的纹状体结合率在正常范围内。我们没有发现纹状体摄取与疾病持续时间、患者年龄、UMSARS-II 评分和脑桥直径之间的相关性。[123]I]FP-CIT SPECT 显示，大多数（但不是全部）无帕金森病的 MSA-C 患者存在亚临床黑质纹状体多巴胺能神经支配丧失，与疾病持续时间、小脑功能障碍或脑桥萎缩无关。
