Ultrasound of inherited vs. acquired demyelinating polyneuropathies

J Neurol. 2013 Dec;260(12):3115-21. doi: 10.1007/s00415-013-7123-8. Epub 2013 Oct 8.

Abstract

We compared features of nerve enlargement in inherited and acquired demyelinating neuropathies using ultrasound. We measured median and ulnar nerve cross-sectional areas in proximal and distal regions in 128 children and adults with inherited [Charcot-Marie-Tooth-1 (CMT-1) (n = 35)] and acquired [chronic inflammatory demyelinating polyneuropathy (CIDP) (n = 55), Guillaine-Barre syndrome (GBS) (n = 21) and multifocal motor neuropathy (MMN) (n = 17)] demyelinating neuropathies. We classified nerve enlargement by degree and number of regions affected. We defined patterns of nerve enlargement as: none, no enlargement; mild, nerves enlarged but never more than twice normal; regional, nerves normal in at least one region and enlarged more than twice normal in at least one region; diffuse, nerves enlarged at all four regions with at least one region more than twice normal size. Nerve enlargement was commonly diffuse (89 %) and generally more than twice normal size in CMT-1, but not (p < 0.001) in acquired disorders which mostly had either no, mild or regional nerve enlargement [CIDP (64 %), GBS (95 %), and MMN (100 %)]. In CIDP, subjects treated within 3 months of disease onset had less nerve enlargement than those treated later. Ultrasound identified patterns of diffuse nerve enlargement can be used to screen patients suspected of having CMT-1. Normal, mildly, or regionally enlarged nerves in demyelinating polyneuropathy suggests an acquired etiology. Early treatment in CIDP may impede nerve enlargement.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Charcot-Marie-Tooth Disease / diagnostic imaging*
  • Child
  • Child, Preschool
  • Female
  • Guillain-Barre Syndrome / diagnostic imaging*
  • Humans
  • Male
  • Middle Aged
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / diagnostic imaging*
  • Ultrasonography
  • Young Adult