Generalised ceroid-lipofuscinosis and brown bowel syndrome in Cocker spaniel dogs

Authors: Palmer AC, Johnstone AC, Jones BR, Blakemore WF, Jolly RD, Hartley WJ
Publication: New Zealand Veterinary Journal, Volume 42, Issue 6, pp 236-239, Dec 1994
Publisher: Taylor and Francis

Animal type: Companion animal, Dog
Subject Terms: Genetics, Inherited disease/conditions, Locomotor, Nervous system/neurology, Muscle/myology, Disease/defect
Article class: Short Communication

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Abstract: The ceroid-lipofuscinoses comprise a group of inherited storage diseases of human beings and animals that are characterised by progressive neurodegenerative disease and the accumulation within cells of a fluorescent lipopigment. A distinct syndrome occurs in Cocker spaniel dogs in which there is a generalised accumulation of a lipofuscin-like pigment, with such a heavy accumulation in smooth muscle that the intestine and other organs have a brown discolouration. Such discolouration is not observed in other forms of ceroid-lipofuscinosis. Dogs are clinically affected in adulthood and show progressive hind limb paresis, inco-ordination, and deficient postural reactions and proprioception. Spinal reflexes may be exaggerated. Behavioural or temperament changes, seizures or blindness may occur.

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