Holmes cerebellar degeneration
Last reviewed 01/2018
Holmes cerebellar degeneration is an autosomal dominant condition in which there is a progressive degeneration of the cerebellar cortex with loss of Purkinje cells and a reactive gliosis.
Onset is usually in middle age. Symptoms begin in the lower limbs, the patient developing a marked ataxia. This spreads to the upper limbs and is accompanied by dysarthria.
Progression occurs over 12-15 years.