Treatable gait disorder and polyneuropathy associated with high titer serum IgM binding to antigens that copurify with myelin-associated glycoprotein

Muscle Nerve. 1994 Nov;17(11):1293-300. doi: 10.1002/mus.880171108.

Abstract

We studied clinical and electrodiagnostic features of 9 patients with very high titers (> 1:10,000) of serum IgM binding to a CNS myelin antigen (CMA) preparation that copurified with myelin-associated glycoprotein (MAG). We found that 8 of the 9 patients had a combined syndrome of gait ataxia and polyneuropathy (GAPN) with late-age onset (mean = 70 years of age). In the 8 GAPN patients progressive difficulty with ambulation led to significant functional disability and frequent falling. Examination showed a wide-based unsteady gait, especially when standing still or turning. There was mild-to-moderate distal sensory loss with involvement of joint position sense only in the toes. Motor changes, when present, were mild and mainly involved distal leg musculature. Treatment of 5 GAPN patients resulted in clear improvement of 2 after intravenous human immunoglobulin and of 3 others after other immunodulating agents. Immune-mediated GAPN syndromes with high titers of serum IgM binding to CMA appear to be treatable causes of gait disorders in older patients.

Publication types

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

MeSH terms

  • Aged
  • Antibodies / blood*
  • Antigens / analysis
  • Ataxia / complications
  • Ataxia / immunology
  • Female
  • Gait*
  • Humans
  • Immunoglobulin M / analysis*
  • Immunoglobulins, Intravenous / therapeutic use
  • Middle Aged
  • Movement Disorders / complications*
  • Movement Disorders / immunology*
  • Movement Disorders / therapy
  • Myelin Proteins / immunology*
  • Myelin-Associated Glycoprotein
  • Nerve Tissue Proteins / analysis
  • Peripheral Nervous System Diseases / complications*
  • Peripheral Nervous System Diseases / immunology*
  • Peripheral Nervous System Diseases / therapy

Substances

  • Antibodies
  • Antigens
  • Immunoglobulin M
  • Immunoglobulins, Intravenous
  • Myelin Proteins
  • Myelin-Associated Glycoprotein
  • Nerve Tissue Proteins