The hereditary cerebellar ataxias are a heterogeneous group of diseases affecting a number of breeds. The severity of clinical signs runs the gamut from fairly benign phenotype compatible with a good quality of life to severe signs necessitating euthanasia. While the prevalence of any single syndrome is low, the number of breeds affected means that the conditions overall are not uncommon. The advent of modern molecular genetics has led to the identification of the mutations responsible for some of these diseases. Thus recognition of the disease is important to appropriately advise breeders of the steps needed to decrease the incidence of the disease in the breed.This review will provide an overview of the development, anatomy and function of the cerebellum, review some of the syndromes which have been described, and discuss how the mutations which have been identified interfere with function.
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