Familial cerebellar ataxia with hydrocephalus in bull mastiffs

A family of bull mastiff pups in which 16 animals developed certain neurological signs is described. It is probable that the disease is inherited in an autosomal recessive manner. Clinical and pathological examination of six animals revealed ataxia, behavioural abnormalities and a visual defect associated with symmetrical lesions in the cerebellar nuclei, lateral vestibular nucleus and inferior colliculus together with a communicating hydrocephalus. The cerebellar lesions consisted of vacuolation, gliosis and axonal degeneration. Evidence for both axonal and oligodendroglial abnormalities was present and these probably result from a genetically determined metabolic disturbance in the affected neurones.