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Machado-Joseph Disease (Spinocerebellar Ataxia Type 3) Summary

163 relevant articles (3 outcomes, 15 trials/studies) found for this Disease

Description: A dominantly-inherited ATAXIA first described in people of Azorean and Portuguese descent, and subsequently identified in Brazil, Japan, China, and Australia. This disorder is classified as one of the SPINOCEREBELLAR ATAXIAS (Type 3) and has been associated with a mutation of the MJD1 gene on chromosome 14. Clinical features include progressive ataxia, DYSARTHRIA, postural instability, nystagmus, eyelid retraction, and facial FASCICULATIONS. DYSTONIA is prominent in younger patients (referred to as Type I Machado-Joseph Disease). Type II features ataxia and ocular signs; Type III features MUSCULAR ATROPHY and a sensorimotor neuropathy; and Type IV features extrapyramidal signs combined with a sensorimotor neuropathy. (From Clin Neurosci 1995;3(1):17-22; Ann Neurol 1998 Mar;43(3):288-96)

Also Known As: Spinocerebellar Ataxia Type 3; Joseph Disease; Disease, Joseph; Autosomal Dominant Striatonigral Degeneration; Azorean Disease (Machado-Joseph) Show All >>

Key Drugs and Agents for Machado-Joseph Disease

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. Trimethoprim (Proloprim) : 2 outcomes 2 studies in 6 results : FDA 73 Generic
  2. Sulfamethoxazole : 2 outcomes 2 studies in 5 results : FDA 74 Generic
  3. tandospirone : 1 outcome in 4 results : IBA
  4. Citric Acid (Citrate) : 1 outcome in 2 results : FDA 12 Generic
  5. polyglutamine : 2 studies in 74 results : IBA
  6. DNA (Deoxyribonucleic Acid) : 2 studies in 8 results : IBA
  7. Genetic Markers (Genetic Marker) : 2 studies in 3 results : IBA
  8. Proteins (Proteins, Gene) : 1 study in 11 results : IBA
  9. Levodopa (L Dopa) : 1 study in 6 results : FDA 24 Generic
  10. Dopamine Plasma Membrane Transport Proteins (Dopamine Transporter) : 1 study in 2 results : IBA
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Diseases Related to Machado-Joseph Disease

  1. Ataxia (Dyssynergia)
  2. Spinocerebellar Ataxias (Spinocerebellar Ataxia)
  3. Cerebellar Ataxia (Dysmetria)
  4. Machado-Joseph Disease (Spinocerebellar Ataxia Type 3)
  5. Huntington Disease (Huntington's Disease)
Show All >>

Key Therapies for Machado-Joseph Disease

Efficacy Chart >>
  1. Vaginal Hysterectomy : 1 result

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