Baringer J R, Prusiner S B
Ann Neurol. 1978 Sep;4(3):205-11. doi: 10.1002/ana.410040303.
Scrapie, kuru, and Creutzfeldt-Jakob disease are characterized by a similar spongiform pathology, prolonged incubation periods, and an agent with unique physical, chemical, and biological properties. Swiss mice were inoculated with the scrapie agent and sacrificed three to five months later for light and electron microscopy. At three months, small vacuoles were seen within the neuropil of the cerebral cortex and basal ganglia. By the fifth month these vacuoles had increased in number and size and were accompanied by moderate astrocytic proliferation. The brainstem, cerebellum, and spinal cord showed variable changes of much less intensity. Many dilated postsynaptic processes contained osmiophilic particles in random or crystalline arrays. The particles, measuring approximately 23 nm in diameter, appeared consistently in postsynaptic processes of brain from scrapie-infected mice, were lacking in controls, and were a size consistent with sedimentation and filtration data for the scrapie agent. Whether these particles represent the scrapie agent must await further studies.
羊瘙痒症、库鲁病和克雅氏病的特征是具有相似的海绵状病理、较长的潜伏期以及一种具有独特物理、化学和生物学特性的病原体。将羊瘙痒症病原体接种到瑞士小鼠体内,三到五个月后处死小鼠进行光镜和电镜检查。三个月时,在大脑皮层和基底神经节的神经毡内可见小空泡。到第五个月,这些空泡的数量和大小增加,并伴有中度星形细胞增生。脑干、小脑和脊髓显示出程度较轻的不同变化。许多扩张的突触后过程含有呈随机或晶体排列的嗜锇颗粒。这些颗粒直径约为23纳米,在感染羊瘙痒症的小鼠大脑的突触后过程中持续出现,在对照中则不存在,其大小与羊瘙痒症病原体的沉降和过滤数据一致。这些颗粒是否代表羊瘙痒症病原体还有待进一步研究。