Bovine ceroid-lipofuscinosis: pathology of blindness

Authors: Birtles MJ, Slack PM, Gibson AJ, Jolly RD, Healy PJ
Publication: New Zealand Veterinary Journal, Volume 40, Issue 3, pp 107-111, Sep 1992
Publisher: Taylor and Francis

Animal type: Cattle, Livestock, Production animal, Ruminant
Subject Terms: Eye/opthalmology, Genetics, Inherited disease/conditions, Fat/lipids, Disease/defect, Nervous system/neurology, Clinical examination, Pathology
Article class: Scientific Article

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Abstract: Five Devon cattle with suspected ceroid-lipofuscinosis and aged between 19 and 39 months of age were humanely slaughtered and subjected to post-mortem examination. There was severe atrophy of the cerebrum, particularly of the occipital cortex. Microscopy also showed severe atrophy of the retina with complete loss of photoreceptor cells, even in the youngest animal examined. Histopathologically the disease was characterised by accumulation of a fluorescent lipopigment in neurones, including those of the retina and a severe astrocytosis. The disease, which is characterised by the accumulation of subunit c of mitochondrial ATP synthase, is similar to that extensively described in South Hampshire sheep except that the retinal lesions were more severe. In contrast, tremors were not noted in the cattle. The clinical history and similarity to the disease in sheep and other species indicated inheritance was as an autosomal recessive trait.

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