A rare form of hereditary ataxia, characterised by onset in early adult life of progressive, spinocerebellar and extrapyramidal disease with external ophthalmoplegia, rigidity dystonia symptoms, and, often, peripheral amyotrophy; found predominantly in people of Azorean ancestry; autosomal dominant inheritance.
Synonym: Azorean disease, Portuguese-Azorean disease.
Origin: Surnames of two families studied in major descriptions of the disease.
(05 Mar 2000)