Friedreich's ataxia epidemiology and demographics

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

Overview

The incidence of Friedreich’s ataxia is approximately 2-4 per 100,000 individuals worldwide. The prevalence of Friedreich’s ataxia is approximately 2-4 per 100,000 individuals worldwide. Friedreich’s Ataxia commonly affects individuals from early childhood through to early adulthood, starting with poor balance when walking, followed by slurred speech and upper-limb ataxia. Friedreich’s Ataxia is usually first diagnosed at age 10 to 15 years but onset of disease may be as early as age 2 years and as late as the 8th decade. The GAA triplet repeat expansion that causes Friedreich’s Ataxia usually affects only individuals of the European, North African, Middle Eastern, or Indian origin (Indo-European and Afro-Asiatic speakers). Sub-Saharan Africans, Amerindians, and individuals from China, Japan, and Southeast Asia are less likely to develop Friedreich’s Ataxia. Friedreich’s Ataxia affects men and women equally. Female are more commonly affected by clinical fractures than male.

Epidemiology and demographics

Incidence

  • The incidence of Friedreich’s ataxia is approximately 2-4 per 100,000 individuals worldwide.[1]

Prevalence

  • The prevalence of Friedreich’s ataxia is approximately 2-4 per 100,000 individuals worldwide.[1]

Age

  • Friedreich’s Ataxia commonly affects individuals from early childhood through to early adulthood, starting with poor balance when walking, followed by slurred speech and upper-limb ataxia.[1]
  • Friedreich’s Ataxia is usually first diagnosed at age 10 to 15 years but onset of disease may be as early as age 2 years and as late as the 8th decade.[1]

Race and Region

  • The GAA triplet repeat expansion that causes Friedreich’s Ataxia usually affects only individuals of the European, North African, Middle Eastern, or Indian origin (Indo-European and Afro-Asiatic speakers).[2]
  • Sub-Saharan Africans, Amerindians, and individuals from China, Japan, and Southeast Asia are less likely to develop Friedreich’s Ataxia.[3]

Gender

  • Friedreich’s Ataxia affects men and women equally.
  • Female are more commonly affected by clinical fractures than male.[1]

References

  1. 1.0 1.1 1.2 1.3 1.4 Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean L, Stephens K, Amemiya A, Bidichandani SI, Delatycki MB. PMID 20301458. Vancouver style error: initials (help); Missing or empty |title= (help)
  2. Grichois ML, Blanc J, Deckert V, Elghozi JL (June 1992). "Differential effects of enalapril and hydralazine on short-term variability of blood pressure and heart rate in rats". J. Cardiovasc. Pharmacol. 19 (6): 863–9. PMID 1376805.
  3. Escudero E, Moreyra A, Iveli C, Lardani H, Cingolani HE (April 1973). "[Myocardial contractility: an experimental analysis of various proposed indices]". Acta Physiol Lat Am (in Spanish; Castilian). 23 (4): 250–69. PMID 4768799.

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