title: Distinctive patterns of sonographic nerve enlargement in Charcot-Marie-Tooth type 1A and hereditary neuropathy with pressure palsies.
abstract: OBJECTIVE
  The extent of sonomorphologic differences of peripheral nerves between CMT and HNPP is unknown.
  
  
  METHODS
  We recruited 9 patients with CMT-1A and 9 with HNPP. Patients underwent a standardized sonographic protocol, which evaluated nerve size and vascularization. We quantitatively assessed fascicle size and echogenicity.
  
  
  RESULTS
  All 18 patients demonstrated nerve enlargement, but no increased vascularization. HNPP demonstrated larger nerves at sites of entrapment (median nerve at the carpal tunnel p=0.049, ulnar nerve at the sulcus p<0.001), greater swelling ratios of median (p<0.001), ulnar (p=0.017) and fibular nerve (p=0.005) than CMT-1A. CMT-1A revealed larger nerves proximal to sites of entrapment (median and fibular nerve, brachial plexus p<0.001). Nerve fascicles where larger (p<0.001) and more hypo-echogenic in CMT-1A. Nerve, fascicle size nor echogenicity correlated with age, gender or MRC sum-score.
  
  
  CONCLUSIONS
  Ultrasonography of nerves reveals specific phenotypes differentiating CMT-1A from HNPP. In CMT-1A enlargement of nerves and fascicles is multifocal among multiple nerves, whereas in HNPP nerve enlargement is restricted to sites of entrapment.
  
  
  CONCLUSIONS
  Our findings of specific sonomorphological phenotypes, differentiating CMT-1A from HNPP, may help to improve our pathophysiological insights in CMT and HNPP.
authors.affiliation: Department of Neurology, UMC Utrecht, Utrecht, The Netherlands; Brain Center Rudolf Magnus, Department of Neuroscience, UMC Utrecht, Utrecht, The Netherlands. Electronic address: h.s.goedee-2@umcutrecht.nl.
authors.forename: Stephan H
authors.initials: SH
authors.lastname: Goedee
authors.affiliation: Department of Neurology, St. Elisabeth Hospital, Tilburg, The Netherlands; Department of Clinical Neurophysiology, St. Elisabeth Hospital, Tilburg, The Netherlands.
authors.forename: Geert J F
authors.initials: GJ
authors.lastname: Brekelmans
authors.affiliation: Department of Neurology, UMC Utrecht, Utrecht, The Netherlands; Brain Center Rudolf Magnus, Department of Neuroscience, UMC Utrecht, Utrecht, The Netherlands.
authors.forename: Leonard H
authors.initials: LH
authors.lastname: van den Berg
authors.affiliation: Department of Neurology, St. Elisabeth Hospital, Tilburg, The Netherlands; Department of Clinical Neurophysiology, St. Elisabeth Hospital, Tilburg, The Netherlands.
authors.forename: Leo H
authors.initials: LH
authors.lastname: Visser
mesh_terms: D000328:Adult; D000368:Aged; D001176:Arthrogryposis / Q000175:diagnosis* / Q000000981:diagnostic imaging*; D001917:Brachial Plexus / Q000000981:diagnostic imaging; D002607:Charcot-Marie-Tooth Disease / Q000175:diagnosis* / Q000000981:diagnostic imaging*; D003937:Diagnosis, Differential; D005260:Female; D015417:Hereditary Sensory and Motor Neuropathy / Q000175:diagnosis* / Q000000981:diagnostic imaging*; D006801:Humans; D006984:Hypertrophy; D008297:Male; D008475:Median Nerve / Q000503:physiopathology; D008875:Middle Aged; D010525:Peripheral Nerves / Q000000981:diagnostic imaging*; D010543:Peroneal Nerve / Q000000981:diagnostic imaging; D010641:Phenotype; D014459:Ulnar Nerve / Q000000981:diagnostic imaging; D014463:Ultrasonography / Q000379:methods
keywords: Charcot–Marie–Tooth (CMT); Hereditary neuropathy with pressure palsies (HNPP); Nerve hypertrophy; Polyneuropathy; Sonography