Immunosuppressive Treatment of Motor Neuron Syndromes. Attempts to Distinguish a Treatable Disorder

Arch Neurol. 1994 Feb;51(2):194-200. doi: 10.1001/archneur.1994.00540140104020.

Abstract

Objective: To determine if response to immunosuppressive treatment in motor neuron syndromes could be predicted on the basis of clinical features, anti-GM1 antibodies, or conduction block.

Design: Prospective, uncontrolled, treatment trial using prednisone for 4 months followed by intravenous cyclophosphamide (3 g/m2) continued orally for 6 months.

Setting: All patients were referred to university hospital medical centers.

Patients: Sixty-five patients with motor neuron syndromes were treated with prednisone; 11 patients had elevated GM1 antibody titers, and 11 patients had conduction block. Forty-five patients received cyclophosphamide, eight of whom had elevated GM1 antibodies and 10 had conduction block.

Results: One patient responded to prednisone, and five patients responded to cyclophosphamide treatment. Only patients with a lower motor neuron syndrome and conduction block improved with either treatment. Response to treatment did not correlate with GM1 antibodies.

Conclusions: GM1 antibodies did not serve as a marker for improvement in patients with motor neuron syndrome treated with immunosuppressive drugs. Patients with amyotrophic lateral sclerosis failed to improve irrespective of laboratory findings.

Publication types

  • Clinical Trial
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adult
  • Aged
  • Amyotrophic Lateral Sclerosis / drug therapy
  • Amyotrophic Lateral Sclerosis / immunology
  • Cyclophosphamide / therapeutic use*
  • Female
  • G(M1) Ganglioside / analysis
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Male
  • Middle Aged
  • Motor Neuron Disease / drug therapy*
  • Motor Neuron Disease / immunology
  • Motor Neuron Disease / physiopathology
  • Neural Conduction
  • Prednisone / therapeutic use*
  • Prospective Studies

Substances

  • Immunosuppressive Agents
  • G(M1) Ganglioside
  • Cyclophosphamide
  • Prednisone