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 the onset of disease may be as early as age 2 years and as late as the 8th decade, and start with progressive ataxiae.[1]
Complications
- Common complications of Friedreich’s Ataxia include:
- The presence of diabetes and dilated cardiomyopathy has a negative impact on survival of patients with Friedreich’s Ataxia.[5]
Prognosis
- The average age of death of patients with Friedreich’s Ataxia is at 37.5 years.[8]
- 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.[9]
References
- ↑ Pandolfo M (March 2009). "Friedreich ataxia: the clinical picture". J. Neurol. 256 Suppl 1: 3–8. doi:10.1007/s00415-009-1002-3. PMID 19283344.
- ↑ 2.0 2.1 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.
- ↑ 5.0 5.1 5.2 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.
- ↑ Feldman JP, Douvier S, Smail M, Michiels Y, Jahier J, Degrolard M (1990). "[Mother-to-fetus contamination in lower genital tract infection caused by mycoplasma and papillomaviruses]". J Gynecol Obstet Biol Reprod (Paris) (in French). 19 (5): 544–6. PMID 2170494.
- ↑ Auboiron S, Bauchart D, David L (June 1991). "Separation and determination of polyether carboxylic antibiotics from Streptomyces hygroscopicus NRRL B 1865 by thin-layer chromatography with flame ionization detection". J. Chromatogr. 547 (1–2): 411–8. PMID 1894724.