Fulminant case of hereditary neuropathy with liability to pressure palsy

Muscle Nerve. 2000 Jun;23(6):979-83. doi: 10.1002/(sici)1097-4598(200006)23:6<979::aid-mus23>3.0.co;2-0.


Hereditary neuropathy with liability to pressure palsy (HNPP) is typified as isolated nerve palsies caused by trivial compression or trauma. It rarely presents in two extremities and even more infrequently affects all four limbs simultaneously. We present a patient who concurrently experienced right shoulder, left hand, and bilateral foot weakness mimicking several multifocal conditions. Electromyography suggested HNPP and subsequent nerve biopsy and genetic testing were confirmatory. The case demonstrates that HNPP can present in a fulminant manner and should be included in the differential diagnosis of acute multiple mononeuropathies. The possible causes for such a rapid clinical course in our patient are discussed.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Biopsy
  • Charcot-Marie-Tooth Disease / genetics*
  • Charcot-Marie-Tooth Disease / pathology*
  • Diagnosis, Differential
  • Female
  • Hereditary Sensory and Motor Neuropathy / genetics*
  • Hereditary Sensory and Motor Neuropathy / pathology*
  • Humans
  • Paralysis / genetics
  • Paralysis / pathology
  • Pressure
  • Sural Nerve / pathology