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威尔特郡绵羊的小脑皮质营养障碍

Cerebellar cortical abiotrophy in Wiltshire sheep.

作者信息

Johnstone A C, Johnson C B, Malcolm K E, Jolly R D

机构信息

Institute of Veterinary, Animal and Biomedical Sciences, Massey University, Private Bag 11222, Palmerston North, New Zealand.

出版信息

N Z Vet J. 2005 Aug;53(4):242-5. doi: 10.1080/00480169.2005.36552.

DOI:10.1080/00480169.2005.36552
PMID:16044183
Abstract

AIM

To investigate the nature of a neurological disease in Wiltshire sheep.

METHODS

Three affected lambs were examined, humanely killed and necropsied. Selected neurological tissues were examined by light and electron microscopy.

RESULTS

Primary neurological lesions were confined to the cerebellum and were characterised by loss of Purkinje cells and the presence of large hypertrophied dendrites of surviving Purkinje cells. These contained stacks of smooth endoplasmic reticulum. There was hyperplasia and cell swelling of Bergmann glia. Mild Wallerian-type degeneration affected white matter in the cerebellum and spinal cord.

CONCLUSION

The cerebellar lesions were of a degenerative and reactive rather than hypoplastic nature. These, and the history, suggest a genetic cause with putative inheritance as an autosomal recessive trait. Accordingly, the disorder is described as a cerebellar abiotrophy.

摘要

目的

研究威尔特郡绵羊一种神经疾病的性质。

方法

对三只患病羔羊进行检查,实施安乐死后进行剖检。选取的神经组织通过光镜和电镜检查。

结果

原发性神经病变局限于小脑,其特征为浦肯野细胞缺失以及存活的浦肯野细胞出现大型肥大树突。这些树突含有堆叠的滑面内质网。伯格曼胶质细胞增生且细胞肿胀。轻度华勒氏型变性影响小脑和脊髓的白质。

结论

小脑病变具有退行性和反应性,而非发育不全的性质。这些病变以及病史提示其病因是遗传性的,推测为常染色体隐性遗传特征。因此,该病症被描述为小脑性共济失调。

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Cerebellar cortical abiotrophy in Wiltshire sheep.威尔特郡绵羊的小脑皮质营养障碍
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引用本文的文献

1
Cerebellar Abiotrophy Across Domestic Species.跨物种的小脑萎缩症。
Cerebellum. 2018 Jun;17(3):372-379. doi: 10.1007/s12311-017-0914-1.