Definition

Olivopontocerebellar atrophy (OPCA) is a group of disorders characterized by degeneration of three brain areas: the inferior olives, the pons, and the cerebellum. OPCA causes increasingly severe ataxia (loss of coordination) as well as other symptoms.

Description

Two distinct groups of diseases are called OPCA, leading to some confusion. Non-inherited OPCA, also called sporadic OPCA, is now considered a form of multiple system atrophy (MSA). Hereditary OPCA, also called inherited OPCA and familial OPCA, is caused by inheritance of a defective gene, which is recognized in some forms but not in others.

Demographics

Hereditary OPCA...