Hereditary neuropathy with liability to pressure palsy in patients under 30 years old: Neurophysiological data and proposed electrodiagnostic criteria

Muscle Nerve. 2018 Feb;57(2):217-221. doi: 10.1002/mus.25666. Epub 2017 Jun 11.


Introduction: In young patients with mononeuropathy who lack family history and precipitating factors, hereditary neuropathy with liability to pressure palsy (HNPP) may be a possibility. Our objective is to propose neurophysiological criteria for HNPP in patients <30 years of age.

Methods: We conducted a national multicenter retrospective clinical and neurophysiological study in patients under 30 with genetically confirmed HNPP.

Results: All of the 51 patients included in the study had at least 1 demyelinating pattern in 2 asymptomatic nerves, and 3 abnormalities were found in almost 90%, including slowed motor nerve conduction velocity across the elbow in at least 1 ulnar nerve (97.5%), increased distal motor latency (DML) in at least 1 fibular nerve (95.8%), and increased DML in both median nerves (89%). Age influenced DML slightly only in the fibular nerve.

Discussion: Dissemination of nerve involvement in HNPP incites to perform a complete nerve conduction study. including bilateral ulnar, fibular, and median nerves. Muscle Nerve 57: 217-221, 2018.

Keywords: PMP22; criteria; hereditary neuropathy; hereditary neuropathy with liability to pressure palsy; neurophysiology; young people.

Publication types

  • Multicenter Study

MeSH terms

  • Adolescent
  • Adult
  • Age of Onset
  • Aging
  • Child
  • Demyelinating Diseases / complications
  • Demyelinating Diseases / pathology
  • Electrodiagnosis / standards*
  • Female
  • Hereditary Sensory and Motor Neuropathy / complications
  • Hereditary Sensory and Motor Neuropathy / diagnosis
  • Hereditary Sensory and Motor Neuropathy / physiopathology*
  • Humans
  • Male
  • Median Nerve / physiopathology
  • Motor Neurons
  • Neural Conduction
  • Paralysis
  • Peripheral Nerves / physiopathology*
  • Peroneal Nerve / physiopathology
  • Pressure
  • Retrospective Studies
  • Ulnar Nerve / physiopathology
  • Young Adult