Corticosteroid-responsive dominantly inherited neuropathy in childhood

Neurology. 1991 Mar;41(3):437-9. doi: 10.1212/wnl.41.3.437.


We describe three children with corticosteroid-responsive inflammatory demyelinating polyneuropathy from families with dominantly inherited neuropathy. There were atypical clinical, electrophysiologic, and pathologic characteristics that suggested a coexistent inflammatory demyelinating neuropathy and that should alert the clinician to the possibility of an associated acquired, potentially treatable disorder.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Child
  • Child, Preschool
  • Demyelinating Diseases / diagnosis
  • Demyelinating Diseases / drug therapy
  • Demyelinating Diseases / genetics*
  • Electrodiagnosis
  • Female
  • Genes, Dominant*
  • Humans
  • Male
  • Peripheral Nervous System Diseases / diagnosis
  • Peripheral Nervous System Diseases / drug therapy
  • Peripheral Nervous System Diseases / genetics*
  • Prednisone / therapeutic use*


  • Prednisone