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Friedreich Ataxia (Friedreich's Ataxia) Summary

Description: An autosomal recessive disease, usually of childhood onset, characterized pathologically by degeneration of the spinocerebellar tracts, posterior columns, and to a lesser extent the corticospinal tracts. Clinical manifestations include GAIT ATAXIA, pes cavus, speech impairment, lateral curvature of spine, rhythmic head tremor, kyphoscoliosis, congestive heart failure (secondary to a cardiomyopathy), and lower extremity weakness. Most forms of this condition are associated with a mutation in a gene on chromosome 9, at band q13, which codes for the mitochondrial protein frataxin. (From Adams et al., Principles of Neurology, 6th ed, p1081; N Engl J Med 1996 Oct 17;335(16):1169-75) The severity of Friedreich ataxia associated with expansion of GAA repeats in the first intron of the frataxin gene correlates with the number of trinucleotide repeats. (From Durr et al, N Engl J Med 1996 Oct 17;335(16):1169-75)

Also Known As: Friedreich's Ataxia; Disease, Friedreich's; Spinocerebellar Ataxia, Friedreich; Hereditary Spinal Sclerosis; Friedreich Familial Ataxia Show All >>

Networked: 979 relevant articles (42 outcomes, 102 trials/studies) for this Disease

Key Drugs and Agents for Friedreich Ataxia

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. idebenone : 9 outcomes 19 studies in 50 results : IBA
  2. frataxin : 6 outcomes 10 studies in 435 results : IBA
  3. Iron : 4 outcomes 5 studies in 159 results : IBA
  4. Antioxidants : 4 outcomes 2 studies in 10 results : IBA
  5. Vitamin E : 3 outcomes 3 studies in 18 results : FDA 13 Generic
  6. deferiprone (CP20) : 3 outcomes 2 studies in 11 results : IBA
  7. Histone Deacetylase Inhibitors : 3 outcomes 1 study in 12 results : IBA
  8. coenzyme Q10 (CoQ10) : 3 outcomes 1 study in 9 results : IBA
  9. Ubiquinone : 3 outcomes 1 study in 5 results : IBA
  10. Chelating Agents : 2 outcomes 1 study in 8 results : IBA
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Diseases Related to Friedreich Ataxia

  1. Neurodegenerative Diseases (Neurodegenerative Disease)
  2. Cardiomyopathies (Cardiomyopathy)
  3. Huntington Disease (Huntington's Disease)
  4. Spasm (Spasms)
  5. Kidney Diseases (Kidney Disease)
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Key Therapies for Friedreich Ataxia

Efficacy Chart >>
  1. Therapeutics : 1 outcome 3 studies in 11 results
  2. Aftercare (After-Treatment) : 1 outcome in 5 results
  3. Complementary Therapies (Alternative Medicine) : 1 outcome in 1 result
  4. Activities of Daily Living (ADL) : 1 study in 5 results
  5. Drug Therapy (Chemotherapy) : 4 results
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