Machado-Joseph disease /mächä′dō jō′səf/ [Machado and Joseph, afflicted families] , a progressive degenerative disease of the central nervous system occurring in families of Portuguese-Azorean descent, having a variety of forms and inherited as an autosomal-dominant trait. There are four major types: Type I, with pyramidal and extrapyramidal deficits; Type II, with cerebellar, pyramidal, and extrapyramidal deficits; Type III, with cerebellar deficits and distal sensorimotor neuropathy; and Type IV, with parkinsonism and distal sensory neuropathy. Also called Azorean disease, Joseph disease, Portuguese-Azorean disease.
