Abstract
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 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 and proprioceptive signs dominate. Few survive without human assistance beyond 6 weeks of age.
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