The electrophysiologic profile of Dejerine–Sottas disease (HMSN III)

Timothy J. Benstead, Nancy L. Kuntz, Robert G. Miller, Jasper R. Daube*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

35 Scopus citations

Abstract

Electrophysiologic studies in 11 patients with Dejerine‐Sottas disease (hereditary motor and sensory neuropathy type III, HMSN II) showed median and ulnar motor nerve conduction velocities less than 6 m/sec in all but 1 patient. Marked temporal dispersion without conduction block was present in all patients. Uniform slowing in adjacent motor nerves was consistent with other studies of inherited neuropathies, although marked temporal dispersion may make HMSN III more difficult to distinguish from acquired neuropathies than other hereditary conditions. The electrophysiologic features of HMSN III patients were significantly different from a series of patients with other hereditary neuropathies chosen because of very slow nerve conduction velocity.

Original languageEnglish (US)
Pages (from-to)586-592
Number of pages7
JournalMuscle & Nerve
Volume13
Issue number7
DOIs
StatePublished - Jul 1990

Keywords

  • Dejerine‐Sottas disease
  • conduction block
  • inherited neuropathy

ASJC Scopus subject areas

  • Physiology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Physiology (medical)

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