CANINE CEROID-LIPOFUSCINOSES - A REVIEW AND CLASSIFICATION

The ceroid-lipofuscinoses are a group of inherited neurodegenerative d iseases of humans and animals characterised by the accumulation of a f luorescent lipopigment in neurons and other cells within the body. Syn dromes occurring in dogs are classified, on the basis of the age of on set and, to a lesser degree, the course of the disease, as prepubertal -protracted, early adult acute and adult onset. Clinical signs are gen erally those of progressive loss of vision, motor disturbances such as ataxia, tremors, seizures and proprioceptive deficits together with b ehavioural changes including loss of learned behaviour, fearfulness an d aggression. The various syndromes can be expected to reflect differe nt mutations, at least some of which affect the catabolism of subunit c of mitochondrial adenosine triphosphate (ATP) synthase. Confirmation of diagnosis depends on the demonstration of lipopigment in brain or skin biopsies by histochemistry, fluorescence or electron microscopy.