Montgomery D L, Storts R W
J Neuropathol Exp Neurol. 1984 May;43(3):263-75. doi: 10.1097/00005072-198405000-00005.
The character and progression of ultrastructural lesions in the caudate nucleus and cerebellar cortex were studied in four Kerry Blue Terriers afflicted with a hereditary neurodegenerative disease. In the caudate nucleus, the initial lesion was mitochondrial hypertrophy in dendrites of intrinsic neurons. Degeneration of these neurons became widespread while axons of passage and terminal boutons were spared. During the final stages, there was severe disruption of the neuropil with loss of both neurons and glia. A narrow zone bordering the lateral ventricles, however, remained unaffected. In the cerebellar cortex, the lesions involved principally Purkinje cells and progressed through a pattern of degeneration comparable to that involving intrinsic neurons of the caudate nucleus. In the later stages, there was astroglial scarring of the molecular layer. In contrast to the caudate nucleus, there was no disruption of the neuropil with loss of structure in the cerebellum. The fact that progression of lesions during the early stages of the disease in both the caudate nucleus and cerebellar cortex was similar suggested a common mechanism for the neurodegeneration .
对四只患有遗传性神经退行性疾病的凯利蓝梗犬的尾状核和小脑皮质超微结构损伤的特征及进展进行了研究。在尾状核中，最初的损伤是固有神经元树突中的线粒体肥大。这些神经元的变性变得广泛，而传导轴突和终末小体则未受影响。在疾病的最后阶段，神经毡严重破坏，神经元和神经胶质均丧失。然而，靠近侧脑室的一个狭窄区域未受影响。在小脑皮质中，损伤主要累及浦肯野细胞，其进展模式与尾状核固有神经元的变性模式相似。在后期，分子层出现星形胶质细胞瘢痕形成。与尾状核不同，小脑中神经毡没有结构破坏。尾状核和小脑皮质在疾病早期损伤进展相似这一事实表明神经退行性变存在共同机制。
