A STUDY OF CEREBELLAR AND CEREBRAL CORTICAL DEGENERATION IN MINIATURE POODLE PUPS WITH EMPHASIS ON THE ULTRASTRUCTURE OF PURKINJE-CELL CHANGES
A STUDY OF CEREBELLAR AND CEREBRAL CORTICAL DEGENERATION IN MINIATURE POODLE PUPS WITH EMPHASIS ON THE ULTRASTRUCTURE OF PURKINJE-CELL CHANGES
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DOI：
10.1007/bf00690534
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发表时间：
1988-01-01
影响因子：
12.7
通讯作者：
DELAHUNTA, A
中科院分区：
文献类型：
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作者：
CUMMINGS, JF;DELAHUNTA, A
在一窝三只迷你贵宾犬中，一只正常的雌性小狗与两只出生后不久就表现出严重平衡和姿势障碍的雄性小狗形成对比。在雄性幼崽中，弥漫性大脑皮质变性伴小脑皮质变性，并伴有浦肯野神经元的广泛丢失和变性。在一些先前的犬小脑萎缩的组织学描述中，退化的浦肯野细胞表现为苍白、肿胀和空泡状或萎缩和深染。肿胀的空泡状细胞体和浓缩的坏死神经元分散在大脑皮层。超微结构研究显示浦肯野神经元和脑神经细胞的空泡变性与内质网的明显扩张和核糖体的丢失有关。在萎缩的浦肯野神经元中，细胞质嗜酸性粒细胞增多与尼氏小体的减少和线粒体（尤其是板层小体）的积累有关。后者是内质网的堆积衍生物，在萎缩的脑核周围未见。片状体在退化的浦肯野神经元的树突茎中达到了巨大的比例。然而，在浦肯野轴突中，轴质小管的聚集往往掩盖了这些小体。这些贵宾犬幼崽的细胞学变化与早期犬遗传性小脑变性的超微结构研究报告明显不同。
In a litter of three Miniature Poodles, a normal female pup contrasted with two males which displayed severe equilibrium and postural disturbances soon after birth. In the male pups diffuse cerebral cortical degeneration accompanied cerebellar cortical degeneration accompanied by extensive loss and degeneration of Purkinje neurons. As in some prior histological descriptions of canine cerebellar atrophies, degenerating Purkinje cells apppeared either pale, swollen and vacuolated or shrunken and hyperchromatic. Swollen vacuolated cell bodies and condensed necrotic neurons were scattered through the cerebral cortex. Ultrastructural studies revealed that the vacuolar degeneration in Purkinje and cerebral neurons was referable to marked dilation of endoplasmic reticulum and loss of ribsomes. In shruken Purkinje neurons, increased cytoplasmic eosinophilia was associated with a decline in Nissl bodies and accumulations of mitochondria and especially lamellar bodies. The latter, stacked derivatives of endoplasmic reticulum, were not encountered in shrunken cerebral perikarya. Lamellar bodies reached giant proportions in the dendritic stems of degenerating Purkinje neurons. In Purkinje axons, however, these bodies often were overshadowed by aggregated of axoplasmic tubules. The cytological changes in these Poodle pups were notably different from those reported in earlier ultrastructural studies of canine inherited cerebellar degenerations.