Friedreich's ataxia natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] ; Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[[2]]

Overview

Natural History

• The symptoms of Friedreich’s Ataxia usually develop in the second decade of life but onset of disease may be as early as age 2 years and as late as the 8th decade, and start with progressive ataxiae.

Complications

• Common complications of Friedreich’s Ataxia include:
  • Aspiration pneumonia
  • Hypertrophic cardiomyopathy
  • Diabetic coma
  • Embolic stroke
  • Cerebral haemorrhage
  • Trauma sequelae
  • Renal failure
• The presence of diabetes and dilated cardiomyopathy has a negative impact on survival of patients with Friedreich’s Ataxia.

Prognosis

• The average age of death of patients with Friedreich’s Ataxia is at 37.5 years.
• Depending on the extent of the disease progression at the time of diagnosis, the prognosis may vary.
• The presence of hypertrophic cardiomyopathy is associated with a particularly poor prognosis among patients with Friedreich’s Ataxia.