Olivopontine cerebellar degeneration may occur sporadically or as an autosomal dominant trait. It is characterised by a general atrophy of the cerebellum spreading in time to involve the pons, medullary olives and other brain stem structures. It can occur at any age but onset in middle life is most common.

Presentation is initially with ataxia, dysarthria, and tremor. Parkinsonian features may develop, accompanied by mild dementia, ophthalmoplegia, pyramidal tract signs and autonomic disturbance.

Survival ranges from 23 - 30 years from onset.