Important

It is possible that the main title of the report Machado-Joseph Disease is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

• Autosomal Dominant Spinocerebellar Degeneration
• Azorean Neurologic Disease
• Machado Disease
• Joseph Disease
• MJD
• Nigrospinodentatal Degeneration
• Striatonigral Degeneration, Autosomal Dominant Type
• Spinocerebellar Ataxia Type III (SCA 3)

Disorder Subdivisions

• Machado-Joseph Disease Type I (MJD-I)
• Machado-Joseph Disease Type II (MJD-II)
• Machado-Joseph Disease Type III (MJD-III)

General Discussion

Machado-Joseph Disease (MJD-III), also called spinocerebellar ataxia type III, is a rare, inherited, ataxia (lack of muscular control) affecting the central nervous system and characterized by the slow degeneration of particular areas of the brain called the hindbrain. Patients with MJD may eventually become crippled and/or paralyzed but their intellect remains intact. The onset of symptoms of MJD varies from early teens to late adulthood.

Three forms of Machado-Joseph Disease are recognized: Types MJD-I, MJD-II, and MJD-III. The differences in the types of MJD relate to the age of onset and severity. Earlier onset usually produces more severe symptoms.