Late-Onset Friedreich's Ataxia (LOFA) Mimicking Charcot-Marie-Tooth Disease Type 2: What Is Similar and What Is Different?

Cerebellum. 2017 Apr;16(2):599-601. doi: 10.1007/s12311-016-0822-9.

Abstract

Herein, we report a patient that presented with late-onset progressive steppage gait, neuropathy and pes cavus, suggesting Charcot-Marie-Tooth (CMT) disease. Subsequent genetic investigation confirmed Friedreich's ataxia (FRDA). We demonstrate that late-onset Friedreich's ataxia (LOFA) may be a CMT mimicker. This case reinforces that other genetic conditions may clinically resemble CMT. The clinical similarities between CMT and FRDA include a symmetrical neuropathy (axonal in FRDA), steppage gait, and eventually scoliosis. We suggest that late-onset forms of hereditary neuropathies should be carefully evaluated, since LOFA may be a CMT mimicker.

Keywords: Charcot–Marie–Tooth disease; Friedreich’s ataxia; Hereditary ataxias; hereditary neuropathies.

Publication types

  • Case Reports
  • Video-Audio Media

MeSH terms

  • Adult
  • Age of Onset
  • Charcot-Marie-Tooth Disease / diagnosis*
  • Diagnosis, Differential
  • Friedreich Ataxia / diagnosis*
  • Friedreich Ataxia / genetics
  • Friedreich Ataxia / pathology
  • Friedreich Ataxia / physiopathology
  • Humans
  • Male