Pollanen M S, Bergeron C, Weyer L
Centre for Research in Neurodegenerative Diseases, University of Toronto, Ontario, Canada.
Acta Neuropathol. 1993;86(5):515-7. doi: 10.1007/BF00228588.
Dementia characterized by neuronal loss and status spongiosus (DNLS) is a non-Alzheimer degenerative process which is characterized by Pick-like lobar atrophy with neuronal depletion and gliosis of the cerebral cortex, corpus striatum, medial thalamus, and substantia nigra and the absence of neuronal inclusions. To further investigate the cause and pathogenesis of DNLS, we probed cerebral homogenates from three cases of DNLS for protease-resistant prion protein to determine if DNLS could be a variant of a human prion disease. Limited proteolysis of prion proteins and guanidine thiocyanate treatment of cortical homogenates was used to enrich potential abnormal prion protein immunoreactivity. Although protease-resistant prion protein was detected in a case of sporadic Creutzfeldt-Jakob disease no abnormal prion protein was found in the cases of DNLS. We conclude that DNLS is not a human prion disease and remains an important dementia of uncertain etiology.
以神经元丢失和海绵状状态为特征的痴呆症（DNLS）是一种非阿尔茨海默病性退行性病变，其特征为类似皮克病的脑叶萎缩，伴有大脑皮质、纹状体、丘脑内侧和黑质的神经元缺失及胶质细胞增生，且无神经元包涵体。为进一步探究DNLS的病因及发病机制，我们检测了3例DNLS患者的脑匀浆中抗蛋白酶朊蛋白，以确定DNLS是否可能是人类朊病毒病的一种变体。采用朊蛋白的有限蛋白酶解及用硫氰酸胍处理皮质匀浆来富集潜在的异常朊蛋白免疫反应性。尽管在1例散发性克雅氏病患者中检测到了抗蛋白酶朊蛋白，但在DNLS患者中未发现异常朊蛋白。我们得出结论，DNLS不是人类朊病毒病，仍然是一种病因不明的重要痴呆症。
