MFN2-related Charcot-Marie-Tooth Disease with Atypical Ocular Manifestations

Intern Med. 2021 Dec 15;60(24):3969-3974. doi: 10.2169/internalmedicine.7463-21. Epub 2021 Jun 12.

Abstract

We herein describe a Charcot-Marie-Tooth disease (CMT) family with a MFN2 mutation with atypical ocular manifestations. The proband, his mother, his third daughter, and his deceased maternal grandfather all had symptoms of CMT and a visual impairment (either cataracts or severe astigmatism). On whole-exome sequencing for the proband having CMT and congenital cataracts, we identified a c.314C>T (p.Thr105Met) mutation in MFN2, but no mutation in the causative genes associated with cataracts. This missense mutation in MFN2 co-segregated with CMT and the atypical ocular manifestations in this family. The findings of this study might help to expand the clinical phenotype of heterogeneous MFN2-related CMT.

Keywords: Charcot-Marie-Tooth disease; MFN2; astigmatism; cataracts; visual impairment.

Publication types

  • Case Reports

MeSH terms

  • Charcot-Marie-Tooth Disease* / diagnosis
  • Charcot-Marie-Tooth Disease* / genetics
  • Female
  • GTP Phosphohydrolases / genetics
  • Humans
  • Mitochondrial Proteins / genetics
  • Mothers
  • Phenotype

Substances

  • Mitochondrial Proteins
  • GTP Phosphohydrolases
  • MFN2 protein, human