title: Electrodiagnostic Characterization of Hereditary Neuropathy With Liability to Pressure Palsies.
abstract: OBJECTIVE
  The study objective was electrodiagnostic characterization of a large cohort of patients with genetically confirmed hereditary neuropathy with liability to pressure palsies (HNPP).
  
  
  METHODS
  A retrospective review was conducted on all patients with HNPP seen at the neuromuscular clinic (London, Canada) from 1977 to 2015. Clinical data obtained included patient characteristics, examination findings, and nerve conduction study results.
  
  
  RESULTS
  A total of 46 patients were analyzed. The mean age and median disease duration were 42.6 and 5.0 years, respectively. Most patients had abnormalities on sural nerve conduction studies. The most common focal neuropathies at compressive sites were ulnar nerve at the elbow (85.7%), distal median nerve at the wrist (84.4%), and fibular nerve at the fibular head (36.7%). Distal median neuropathy was associated with a mean terminal latency of 6.64 milliseconds.
  
  
  CONCLUSIONS
  The presence of polyneuropathy, median terminal motor latency prolongation, and multiple compressive neuropathies are the most common findings associated with HNPP.
authors.affiliation: *Department of Clinical Neurological Sciences, London Health Sciences Centre, University Hospital, London, ON, Canada; and †Schulich School of Medicine and Dentistry, Western University, London, ON, Canada.
authors.forename: Sachiko
authors.initials: S
authors.lastname: Takahashi
authors.forename: Marvin
authors.initials: M
authors.lastname: Chum
authors.forename: Kurt
authors.initials: K
authors.lastname: Kimpinski
mesh_terms: D000200:Action Potentials / Q000502:physiology*; D000328:Adult; D001176:Arthrogryposis / Q000175:diagnosis* / Q000503:physiopathology; D004568:Electrodiagnosis; D005260:Female; D015417:Hereditary Sensory and Motor Neuropathy / Q000175:diagnosis* / Q000503:physiopathology; D006801:Humans; D008297:Male; D008475:Median Nerve / Q000503:physiopathology; D008875:Middle Aged; D009431:Neural Conduction / Q000502:physiology*; D010641:Phenotype; D012189:Retrospective Studies; D013497:Sural Nerve / Q000503:physiopathology; D063189:Symptom Assessment