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

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.

Common complications of Friedreich’s Ataxia include:
- Aspiration pneumonia^[1]
- Hypertrophic cardiomyopathy^[2]
- Diabetic coma^[3]
- Embolic stroke^[4]
- Cerebral haemorrhage^[1]
- Trauma sequelae^[5]^[6]
- Renal failure^[4]
The presence of diabetes and dilated cardiomyopathy has a negative impact on survival of patients with Friedreich’s Ataxia.^[4]

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.

↑ ^1.0 ^1.1 Byard RW, Gilbert JD (October 2017). "Mechanisms of unexpected death and autopsy findings in Friedreich ataxia". Med Sci Law. 57 (4): 192–196. doi:10.1177/0025802417723809. PMID 28803513.
↑ Weidemann F, Störk S, Liu D, Hu K, Herrmann S, Ertl G, Niemann M (August 2013). "Cardiomyopathy of Friedreich ataxia". J. Neurochem. 126 Suppl 1: 88–93. doi:10.1111/jnc.12217. PMID 23859344.
↑ Körner A, Barta L (June 1983). "[Association of diabetes mellitus with Friedreich's ataxia]". Orv Hetil (in Hungarian). 124 (23): 1391–2. PMID 6224121.
↑ ^4.0 ^4.1 ^4.2 Marcus AJ, Safier LB, Ullman HL, Islam N, Broekman MJ, Falck JR, Fischer S, von Schacky C (1988). "Cell-cell interactions in the eicosanoid pathways". Prog. Clin. Biol. Res. 283: 559–67. PMID 3062632.
↑ FRIEDMAN JH, ARENGO A (January 1958). "Friedreich's ataxia ascribed to trauma; case report". Dis Nerv Syst. 19 (1): 35–6. PMID 13501109.
↑ Junck L, Gilman S, Gebarski SS, Koeppe RA, Kluin KJ, Markel DS (April 1994). "Structural and functional brain imaging in Friedreich's ataxia". Arch. Neurol. 51 (4): 349–55. PMID 8155012.