Jendroska K, Hoffmann O, Schelosky L, Lees A J, Poewe W, Daniel S E
Department of Neurology, Universitätsklinikum Rudolf Virchow, Berlin, Germany.
J Neurol Neurosurg Psychiatry. 1994 Oct;57(10):1249-51. doi: 10.1136/jnnp.57.10.1249.
Movement disorders presenting with parkinsonism may share histopathological features with Creutzfeldt-Jakob disease, a spongiform encephalopathy caused by the accumulation of pathological prion protein in brain. To investigate a possible aetiological link between these conditions and Creutzfeldt-Jakob disease, histoblot immunostaining for pathological prion protein was carried out in 90 cases including idiopathic Parkinson's disease, multiple system atrophy, diffuse Lewy body disease, Steele-Richardson-Olszewski syndrome, corticobasal degeneration, and Pick's disease. Pathological prion protein was identified in four controls with Creutzfeldt-Jakob disease but not in any of the other diseases examined. The findings suggest that an aetiological role for prions in these movement disorders is unlikely. Histoblotting provides a useful method for screening large areas of tissue for the presence of pathological prion protein and may be helpful in the differential diagnosis of difficult cases.
表现为帕金森症的运动障碍可能与克雅氏病具有共同的组织病理学特征，克雅氏病是一种由病理性朊蛋白在大脑中积聚引起的海绵状脑病。为了研究这些病症与克雅氏病之间可能的病因联系，对90例患者进行了病理性朊蛋白的组织印迹免疫染色，这些患者包括特发性帕金森病、多系统萎缩、弥漫性路易体病、斯蒂尔-理查森-奥尔谢夫斯基综合征、皮质基底节变性和皮克病。在4例克雅氏病对照中鉴定出病理性朊蛋白，但在所检查的其他疾病中均未发现。这些发现表明，朊病毒在这些运动障碍中不太可能起到病因作用。组织印迹法为在大面积组织中筛查病理性朊蛋白的存在提供了一种有用的方法，可能有助于疑难病例的鉴别诊断。
