Cerebellar cortical abiotrophy in Wiltshire sheep |
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| Authors: | AC Johnstone CB Johnson KE Malcolm RD Jolly |
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| Affiliation: | 1. Institute of Veterinary, Animal and Biomedical Sciences , Massey University , Private Bag 11222, Palmerston North, New Zealand E-mail: A.C.Johnstone@massey.ac.nz;2. John Hill Road, RD3, Papakura, New Zealand |
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| 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. |
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| Keywords: | Sheep cerebellum Purkinje cells abiotrophy smooth endoplasmic reticulum |
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