Barcikowska M, Kwiecinski H, Liberski P P, Kowalski J, Brown P, Gajdusek D C
Department of Neuropathology, Medical Research Center, Polish Academy of Sciences, Warsaw.
Histopathology. 1995 May;26(5):445-50. doi: 10.1111/j.1365-2559.1995.tb00252.x.
Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker syndrome are classified as transmissible cerebral amyloidoses, in contrast to the non-transmissible amyloidoses of Alzheimer's disease type. While the aetiologies of Creutzfeldt-Jakob disease and Alzheimer's disease and the molecular composition of their amyloids are different, similar basic pathogenetic mechanisms operate in both diseases through synthesis and processing of amyloid precursor proteins, to produce an accumulation of amyloid deposits. We report here a case of Creutzfeldt-Jakob disease exhibiting numerous diffuse A beta immunoreactive plaques, thus presenting features of both Creutzfeldt-Jakob disease and Alzheimer's disease. The existence of such cases underlines the existence of a 'grey' area between the two types of amyloidoses.
克雅氏病和格斯特曼-施特劳斯勒-谢林克综合征被归类为可传播性脑淀粉样变性,与阿尔茨海默病类型的非传播性淀粉样变性形成对比。虽然克雅氏病和阿尔茨海默病的病因及其淀粉样蛋白的分子组成不同,但两种疾病通过淀粉样前体蛋白的合成和加工存在相似的基本致病机制,以产生淀粉样沉积物的积累。我们在此报告一例克雅氏病病例,该病例表现出大量弥漫性β-淀粉样蛋白免疫反应性斑块,因此呈现出克雅氏病和阿尔茨海默病的特征。此类病例的存在突显了两种类型淀粉样变性之间“灰色地带”的存在。