Cerebellar cortical abiotrophy in Wiltshire sheep (abstract)

Authors: Johnson CB, Johnstone AC, Jolly RD
Publication: New Zealand Veterinary Journal, Volume 53, Issue 1, pp 93, Feb 2005
Publisher: Taylor and Francis

Animal type: Livestock, Production animal, Ruminant, Sheep
Subject Terms: Breed/breeding, Nervous system/neurology, Genetics, Inherited disease/conditions
Article class: Abstract
Abstract: Three 3-month-old Wiltshire lambs with slowly progressive cerebellar signs were investigated. Lambs were normal at birth but developed neurological signs of head ‘shimmer’ together with an up and down head nodding, hindlimb weakness and ataxia. When yarded or stressed, the hindlimbs would collapse and go under the body. The lambs would ‘bounce back’ again with the next stride. Histologically, primary lesions were confined to the cerebellum and were characterised by a variable loss of Purkinje cells and the presence of large hypertrophied dendrites of surviving Purkinje cells that 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. The cerebellar lesions were of a degenerative and reactive nature rather than hypoplastic. These and the history suggested a genetic cause with putative inheritance as an autosomal recessive trait. Accordingly, the disorder is described as a cerebellar abiotrophy.
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