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 θ 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.
Keywords
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Article info
Publication history
Accepted:
September 15,
2003
Received in revised form:
August 25,
2003
Received:
April 10,
2003
Identification
Copyright
© 2003 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
