An inflammatory factor in Charcot-Marie-Tooth disease

Two French publications suggest that an inflammatory mechanism may be involved in certain forms of Charcot-Marie-Tooth disease (CMT), which are generally considered to be exclusively genetically determined.

  • Four patients with CMT 1A followed up at two reference centres developed Guillain-BarrĂ© syndrome, which progressed favourably overall. The high frequency of this association (4 cases in 5 years in two French regions, compared with 1 case expected every 137 years if the two diseases were independent) suggests that CMT1A could be a risk factor for Guillain-BarrĂ© syndrome.
  • Two patients with CMT 4A from islands in the Indian Ocean showed signs of inflammation, suggesting Guillain-BarrĂ© syndrome in one of them.

These observations reinforce the hypothesis that an inflammatory process contributes to nerve damage in certain forms of CMT.

 

Guillain-Barré syndrome in patients with Charcot-Marie-Tooth type 1A disease, probably a non-random association. Davion JB., Devaux J., Tard C. et al Neurophysiol Clin. 2025 Mar

 

New double homozygous GDAP1 variations associated with polyneuropathy and prominent inflammatory features in unrelated individuals from Indian Ocean islands. Faye PA., Magdelaine C., Espil C. et al. Clin Neurophysiol. 2025 Mar