Late onset Friedreich’s ataxia (LOFA)

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Late onset Friedreich’s ataxia (LOFA). Classical onset age: the onset age is <20 years. Fully referenced neurology checklist.

FA onset age definitions

  • Classical onset age: the onset age is <20 years
  • Very young onset age: this is associated with p.C282Y heterozygosity
  • Late-onset Friedreich’s ataxia (LOFA): the onset age is > 25 years
  • Very late onset Friedreich’s ataxia (VLOFA): the onset age is >40 years

Features more frequent in LOFA

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Features less frequent in LOFA: neurological

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Features less frequent in LOFA: systemic

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References

  1. Lecocq C, Charles P, Azulay JP, et al. Delayed-onset Friedreich's ataxia revisited. Mov Disord 2016; 31:62-69.
  2. Koppel AH. Friedreich's ataxia: pathology, pathogenesis, and molecular genetics. J Neurol Sci 2011; 303:1-12. 
  3. Martinez AR, Moro A, Abrahao A, et al. Nonneurological involvement in late-onset Friedreich ataxia (LOFA): exploring the phenotypes. Cerebellum 2017; 16:253-256. 
  4. Fearon C, Lonergan R, Ferguson D, Byrne S, Bradley D, Langan Y, Redmond J. Very-late-onset Friedreich's ataxia: diagnosis in a kindred with late-onset cerebellar ataxia. Pract Neurol 2020; 20:55-58. 
  5. Vidhale TA, Gupta HR, Pj R, Gandhi C. Very late-onset Friedreich's ataxia with rapid course mimicking as possible multiple system atrophy cerebellar type. BMJ Case Rep 2021; 14:e242073.
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