GENERALIZED CEROID-LIPOFUSCINOSIS AND BROWN BOWEL SYNDROME IN COCKER-SPANIEL DOGS

The ceroid-lipofuscinoses comprise a group of inherited storage diseas es of human beings and animals that are characterised by progressive n eurodegenerative disease and the accumulation within cells of a fluore scent lipopigment. A distinct syndrome occurs in Cocker spaniel dogs i n which there is a generalised accumulation of a lipofuscin-like pigme nt, 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 clinica lly affected in adulthood and show progressive hind limb paresis, inco -ordination, and deficient postural reactions and proprioception. Spin al reflexes may be exaggerated. Behavioural or temperament changes, se izures or blindness may occur.