Bickerstaff's brainstem encephalitis associated with IgM antibodies to GM1b and GalNAc-GD1a

J Neurol Sci. 2004 Feb 15;217(2):225-8. doi: 10.1016/j.jns.2003.09.008.

Abstract

This is the first report of a case of Bickerstaff's brainstem encephalitis (BBE) associated with IgM antibodies to GM1b and GalNAc-GD1a. Subsequent to Campylobacter jejuni enteritis, the patient rapidly developed consciousness disturbance and hyperreflexia in addition to external ophthalmoplegia and cerebellar-like ataxia. EEG showed transient 7 Hz monorhythmic theta activities, predominantly in the front-central area. He received high doses of immunoglobulin intravenously and had completely recovered 3 months later. High anti-GM1b and anti-GalNAc-GD1a IgM antibody titers present during the acute phase decreased with his clinical improvement. An absorption study showed the anti-GM1b and anti-GalNAc-GD1a IgM antibodies to be cross-reactive. Anti-GM1b and anti-GalNAc-GD1a antibodies have been detected in some patients who developed Guillain-Barré syndrome after C. jejuni enteritis, whereas the anti-GQ1b IgG antibody is associated with BBE. Infection by C. jejuni bearing a GM1b-like or GalNAc-GD1a-like lipooligosaccharide may trigger the production of anti-GalNAc-GD1a and anti-GM1b IgM antibodies. It is not clear why our patient developed BBE rather than Guillain-Barré syndrome. These antibodies may, however, prove useful serological markers for identifying BBE patients who do not have the anti-GQ1b IgG antibody.

Publication types

  • Case Reports

MeSH terms

  • Ataxia / immunology
  • Ataxia / physiopathology
  • Autoantibodies / blood
  • Autoantibodies / immunology*
  • Brain Stem / immunology*
  • Brain Stem / pathology
  • Brain Stem / physiopathology
  • Campylobacter Infections / complications
  • Campylobacter Infections / immunology
  • Campylobacter jejuni / immunology
  • Child
  • Consciousness Disorders / immunology
  • Consciousness Disorders / physiopathology
  • Electroencephalography
  • Encephalitis / blood
  • Encephalitis / immunology*
  • Encephalitis / physiopathology
  • G(M1) Ganglioside / analogs & derivatives*
  • G(M1) Ganglioside / immunology*
  • Gangliosides / immunology*
  • Humans
  • Immunoglobulin M / blood
  • Immunoglobulin M / immunology*
  • Male
  • Recovery of Function
  • Reflex, Abnormal / immunology

Substances

  • Autoantibodies
  • Gangliosides
  • Immunoglobulin M
  • G(M1) Ganglioside
  • ganglioside M1b
  • IV(4)-galactosyl-N-acetylganglioside GD1a