Brown P, Kaur P, Sulima M P, Goldfarb L G, Gibbs C J, Gajdusek D C
Laboratory of CNS Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892.
Lancet. 1993 Jan 16;341(8838):127-9. doi: 10.1016/0140-6736(93)90001-w.
The term "prion dementia" has been proposed to replace "spongiform encephalopathy", to accommodate the existence of atypical forms of these "prion protein" (PrP) cerebral amyloidoses that may not show spongiform changes in the brain. We tested brain tissue extracts for the presence of PrP from 46 cases (including 13 familial cases) of non-spongiform dementias with a variety of associated neurological signs, referred to our laboratory for primate transmission studies. None of the cases transmitted disease to primates, and none had PrP detectable by western immunoblots of extracted brain tissue. We conclude that prion dementias are not lurking undetected within the larger landscape of neurodegenerative disorders, and that their clinicopathological limits are, except for a small number of previously reported familial cases, essentially those of spongiform encephalopathy.
有人提议用“朊病毒痴呆症”一词取代“海绵状脑病”，以涵盖这些“朊病毒蛋白”（PrP）脑淀粉样变性的非典型形式的存在，这些非典型形式在大脑中可能不会出现海绵状变化。我们检测了46例（包括13例家族性病例）伴有各种相关神经体征的非海绵状痴呆症患者的脑组织提取物中是否存在PrP，这些病例被转诊至我们实验室进行灵长类动物传播研究。没有一例病例将疾病传播给灵长类动物，并且通过提取脑组织的western免疫印迹法均未检测到PrP。我们得出结论，在更大范围的神经退行性疾病中，不存在未被发现的朊病毒痴呆症，并且除了少数先前报道的家族性病例外，其临床病理界限基本上就是海绵状脑病的界限。
