Axonal multifocal motor neuropathy without conduction block or other features of demyelination

Neurology. 2002 Feb 26;58(4):615-20. doi: 10.1212/wnl.58.4.615.

Abstract

Background: Conduction block is considered an essential finding for the distinction between motor neuropathies and lower motor neuron disorders. Only a small number of reports describe patients with multifocal motor neuropathies who lack overt conduction block, although in these cases other features of demyelination still suggest the presence of a demyelinating disorder. In contrast, a purely axonal multifocal motor neuropathy has not been described.

Methods: This report describes nine patients with slowly or nonprogressive multifocal motor neuropathies who had purely axonal electrodiagnostic features.

Results: GM1 antibodies titers were normal in all nine cases. Six patients were treated with either prednisone or IV immunoglobulin and three showed convincing improvement.

Conclusions: These findings suggest an immune-mediated motor neuropathy with axonal electrophysiologic features that appears to be distinct from both multifocal motor neuropathy and established motor neuron disorders.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Anti-Inflammatory Agents / therapeutic use
  • Axons / pathology*
  • Demyelinating Diseases / diagnosis*
  • Electromyography
  • Female
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Male
  • Middle Aged
  • Neural Conduction* / physiology
  • Polyneuropathies / diagnosis*
  • Polyneuropathies / drug therapy
  • Polyneuropathies / physiopathology
  • Prednisone / therapeutic use

Substances

  • Anti-Inflammatory Agents
  • Immunoglobulins, Intravenous
  • Prednisone