Cerebellar ataxia and polyneuropathy in a patient with IgM M-protein specific to the Gal(beta 1-3)GalNAc epitope

J Neurol Sci. 1994 Nov;126(2):219-24. doi: 10.1016/0022-510x(94)90277-1.

Abstract

A 79-year-old man with sensory dominant polyneuropathy, cerebellar ataxia, and palatal myoclonus had serum IgM M-protein that specifically bound to GM1, GD1b, and asialo-GM1. IgM with the same specificity was detected in his cerebrospinal fluid. Results of immunohistochemical studies showed specific binding of this monoclonal IgM to the cerebellar granular layer, dentate nucleus, inferior olive, and gray matter of the cerebrum and spinal cord. Monoclonal antibody GGR12, monospecific to GD1b, had an immunostaining distribution similar to that of the patient's IgM M-protein. The binding of M-protein may be associated with the development of cerebellar ataxia and palatal myoclonus in this patient.

Publication types

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

MeSH terms

  • Aged
  • Antigens, Tumor-Associated, Carbohydrate / immunology*
  • Cerebellar Ataxia / blood
  • Cerebellar Ataxia / complications*
  • Chromatography, Thin Layer
  • Enzyme-Linked Immunosorbent Assay
  • Epitopes*
  • G(M1) Ganglioside / metabolism
  • Gangliosides / metabolism
  • Humans
  • Immunoglobulin M / metabolism
  • Immunohistochemistry
  • Male
  • Myelin Proteins / immunology*
  • Peripheral Nervous System Diseases / blood
  • Peripheral Nervous System Diseases / complications*

Substances

  • Antigens, Tumor-Associated, Carbohydrate
  • Epitopes
  • Gangliosides
  • Immunoglobulin M
  • Myelin Proteins
  • ganglioside, GD1b
  • Thomsen-Friedenreich antigen
  • G(M1) Ganglioside
  • asialo GM1 ganglioside