Genetic and Rare Diseases Information Center (GARD)


Other names people use for this condition
  • SCA3
  • Machado Joseph disease
  • MJD
  • Nigrospinodentatal degeneration
  • Azorean neurologic disease
  • Spinocerebellar atrophy type 3
  • Spinopontine atrophy

Spinocerebellar ataxia 3
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Spinocerebellar ataxia 3 is a rare movement disorder that is characterized by ataxia, clumsiness and weakness in the arms and legs, spasticity, a staggering lurching walk easily mistaken for drunkenness, difficulty with speech and swallowing, involuntary eye movements, double vision, and frequent urination. Some patients have dystonia or symptoms similar to those of Parkinson's disease. Others have twitching of the face or tongue, or bulging eyes. Symptoms can begin any time between early adolescence and about 70 years of age. Spinocerebellar ataxia 3 is a progressive disease, meaning that symptoms get worse with time. Life expectancy ranges from the mid-thirties for those with severe forms of the disorder to a normal life expectancy for those with mild forms. Spinocerebellar ataxia is inherited in an autosomal dominant pattern and is caused by a trinucleotide repeat expansion in the ataxin-3 gene (ATXN3).[1]

References
  1. Machado-Joseph Disease Fact Sheet. National Institute of Neurological Disorders and Stroke Website. April 24, 2009 Available at: http://www.ninds.nih.gov/disorders/machado_joseph/detail_machado_joseph.htm. Accessed May 4, 2009.

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