Holmes cerebellar degeneration
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.
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