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Spinocerebellar ataxia type 3

What is Spinocerebellar ataxia type 3?

Spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease, is the most common subtype of type 1 autosomal dominant cerebellar ataxia (ADCA type 1; see this term), a neurodegenerative disorder, and is characterized by ataxia, external progressive ophthalmoplegia, and other neurological manifestations.

Other condition names

  • Azorean disease of the nervous system
  • MJD
  • Machado disease
  • Machado-Joseph disease
  • Nigro-spino-dentatal degeneration with nuclear ophthalmoplegia
  • SCA3

Inheritance type

Autosomal dominant

Prevalence

  • Worldwide: 1-9 in 100,000

Age of Onset

  • Adolescent
  • Adult
  • Childhood
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on "https://www.orphadata.com". Data version 1.3.16 / 4.1.7.