Snow B J, Bhatt M, Martin W R, Li D, Calne D B
Belzberg Laboratory of Clinical Neuroscience, Department of Radiology, University of British Columbia, Vancouver, Canada.
J Neurol Neurosurg Psychiatry. 1991 Jan;54(1):12-7. doi: 10.1136/jnnp.54.1.12.
Movement disorders, including Parkinsonism, are prominent features of neurological Wilson's disease (WD). This suggests there may be dysfunction of the nigrostriatal dopaminergic pathway. To explore this possibility, five patients were studied using positron emission tomography (PET) with 18F-6-fluorodopa (6FD), and magnetic resonance imaging (MRI). We calculated striatal 6FD uptake rate constants by a graphical method and compared the results with those of 18 normal subjects. It was found that four patients with symptoms all had abnormally low 6FD uptake, and the one asymptomatic patient had normal uptake. PET evidence for nigrostriatal dopaminergic dysfunction was present even after many years of penicillamine treatment. It is concluded that the nigrostriatal dopaminergic pathway is involved in neurological WD.
运动障碍，包括帕金森症，是神经型威尔逊病（WD）的显著特征。这表明黑质纹状体多巴胺能通路可能存在功能障碍。为探究这种可能性，我们使用正电子发射断层扫描（PET）结合18F - 6 - 氟多巴（6FD）以及磁共振成像（MRI）对5名患者进行了研究。我们通过图形法计算纹状体6FD摄取速率常数，并将结果与18名正常受试者的结果进行比较。结果发现，4名有症状的患者6FD摄取均异常降低，而1名无症状患者的摄取正常。即使经过多年青霉胺治疗，仍有PET证据表明存在黑质纹状体多巴胺能功能障碍。结论是黑质纹状体多巴胺能通路参与了神经型WD。
