Neuraxonal dystrophy in Coopworth sheep (abstract)

Authors: Johnstone AC
Publication: New Zealand Veterinary Journal, Volume 40, Issue 1, pp 35-36, Mar 1992
Publisher: Taylor and Francis

Animal type: Livestock, Production animal, Ruminant, Sheep
Subject Terms: Genetics, Inherited disease/conditions, Nervous system/neurology, Pathology
Article class: Abstract

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Abstract: Coopworth sheep have been identified in the Taranaki region with a multisystems abiotrophy of proximal axonal processes which is most likely inherited as a recessive condition. Lambs are affected from birth with a progressive syndrome in which cerebellar and proprioceptive signs dominate. Few survive without human assistance beyond 6 weeks of age
The brain and spinal cord of eight affected lambs were examined.
The outstanding histological feature was the wide distribution of large proximal axonal spheroids (spinal ganglia, spinal grey matter, vesicular and red nuclei in all lambs). In all lambs the nuclei of the cerebellar roof were depleted of neurones and had numerous chromatolytic cell bodies. This change was accompanied by mild diffuse gliosis. Cerebellar grey matter had widespread mild to moderate Purkinje cell loss and scattered swollen axons. In all lambs there was moderate to severe depletion of Purkinje cells of the nodules and flocculus. Empty axis cylinders and myelin “bubbles” were regular features in spinal nerve rootlets and cerebellar white matter but were infrequently present outside these areas.
Two lambs had widespread neurogenic atrophy of muscles.

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