CANINE CEROID-LIPOFUSCINOSES - A REVIEW AND CLASSIFICATION

The ceroid-lipofuscinoses are a group of inherited neurodegenerative diseases of humans and animals characterised by the accumulation of a fluorescent lipopigment in neurons and other cells within the body. Syndromes occurring in dogs are classified, on the basis of the age of onset and, to a lesser degree, the course of the disease, as prepubertal-protracted, early adult acute and adult onset. Clinical signs are generally those of progressive loss of vision, motor disturbances such as ataxia, tremors, seizures and proprioceptive deficits together with behavioural changes including loss of learned behaviour, fearfulness and aggression. The various syndromes can be expected to reflect different 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.