Electrophysiological comparison between males and females in HNPP

Neurol Sci. 2013 Aug;34(8):1429-32. doi: 10.1007/s10072-012-1258-8. Epub 2012 Dec 4.


Some evidences highlighted a higher clinical expression of hereditary neuropathy with liability to pressure palsy (HNPP) in males, and a higher load of traumatic nerve injuries due to different occupational activity has been invoked to explain this observation. It is unknown whether this increased clinical impairment corresponds to a greater electrophysiological involvement. Thus, we compared clinical and electrophysiological features between men and women in a large cohort of HNPP patients. Nerve palsies and electrophysiological abnormalities were more frequent in men, and electrophysiological findings which differentiated males from females did not show any age-related worsening. In conclusion, our findings showed a higher clinical and electrophysiological involvement in males which does not seem related to different cumulative nerve damage over time. We believe that the higher disease expression may increase the chance to detect the disease in males and, thereby, to underestimate the HNPP diagnosis in females.

Publication types

  • Comparative Study

MeSH terms

  • Adult
  • Arthrogryposis / diagnosis
  • Arthrogryposis / physiopathology*
  • Evoked Potentials, Motor*
  • Female
  • Hereditary Sensory and Motor Neuropathy / diagnosis
  • Hereditary Sensory and Motor Neuropathy / physiopathology*
  • Humans
  • Male
  • Neural Conduction*
  • Sex Factors

Supplementary concepts

  • Tomaculous neuropathy