Guiroy D C, Williams E S, Yanagihara R, Gajdusek D C
Laboratory of Central Nervous System Studies, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892.
Acta Neuropathol. 1991;81(5):475-8. doi: 10.1007/BF00310125.
Chronic wasting disease (CWD), a progressive neurological disorder of captive mule deer, black-tailed deer, hybrids of mule deer and white-tailed deer and Rocky Mountain elk, is characterized neuropathologically by widespread spongiform change of the neuropil, intracytoplasmic vacuolation in neuronal perikarya and astrocytic hypertrophy and hyperplasia. We report the topographic distribution of amyloid plaques reactive to antibodies prepared against scrapie amyloid in CWD-affected captive mule deer (Odocoileus hemionus hemionus). Scrapie amyloid-immunoreactive plaques were found in the cerebral gray and white matter, in deep subcortical nuclei, in isolation or in clusters in areas of vacuolation, and perivascularly, in subpial and subependymal regions. In the cerebellum, immunoreactive amyloid plaques were observed in the molecular, pyramidal and granular layers. Scrapie amyloid-immunoreactive deposits were also seen in neuronal perikarya. Furthermore, amyloid plaques in CWD-affected captive mule deer were alcianophilic at 0.3 M magnesium chloride indicating the presence of weakly to moderately sulfated glycosaminoglycans. Our data corroborate that CWD in captive mule deer belongs to the subacute virus spongiform encephalopathies.
慢性消耗病(CWD)是一种影响圈养骡鹿、黑尾鹿、骡鹿与白尾鹿杂交种以及落基山麋鹿的进行性神经疾病,其神经病理学特征为神经纤维网广泛的海绵状变化、神经元胞体的胞浆内空泡化以及星形胶质细胞肥大和增生。我们报告了在受CWD影响的圈养骡鹿(Odocoileus hemionus hemionus)中,对针对羊瘙痒病淀粉样蛋白制备的抗体有反应的淀粉样斑块的地形分布。在脑灰质和白质、深部皮质下核团、空泡化区域的孤立或成簇部位以及软膜下和室管膜下区域的血管周围发现了羊瘙痒病淀粉样蛋白免疫反应性斑块。在小脑中,在分子层、锥体层和颗粒层观察到免疫反应性淀粉样斑块。在神经元胞体中也可见到羊瘙痒病淀粉样蛋白免疫反应性沉积物。此外,受CWD影响的圈养骡鹿中的淀粉样斑块在0.3 M氯化镁中呈阿利新蓝阳性,表明存在弱至中度硫酸化的糖胺聚糖。我们的数据证实,圈养骡鹿中的CWD属于亚急性病毒海绵状脑病。