Guillain-Barré syndrome, Fisher syndrome and Bickerstaff brainstem encephalitis: Understanding the pathogenesis
- Author:
Nortina Shahrizaila
;
Nobuhiro Yuki
- Publication Type:Review
- From:Neurology Asia
2010;15(3):203-209
- CountryMalaysia
- Language:English
-
Abstract:
Guillain-Barré syndrome (GBS), Fisher syndrome (FS) and Bickerstaff brainstem encephalitis represent
a spectrum of acute post-infectious immune-mediated diseases. GBS can present as acute infl ammatory
demyelinating neuropathy or acute motor axonal neuropathy (AMAN). The epidemiological association
of Campylobacter jejuni infection and antiganglioside antibodies with AMAN and FS is well established.
Gangliosides GM1 and GD1a, target molecules in AMAN, are identical to the terminal carbohydrate
residues of C jejuni lipo-oligosaccharides. AMAN can be reproduced in rabbits sensitized with the
gangliosides and lipo-oligosaccharides, thus verifying GBS as the fi rst example of molecular mimicry
in autoimmune diseases. Immunohistochemical studies on AMAN rabbit models demonstrated
autoantibody binding at the nodes of Ranvier, triggering complement activation followed by formation
of membrane attack complexes. This leads to the disappearance of sodium channel clusters, causing
muscle weakness and axonal degeneration. Like AMAN, FS also displays molecular mimicry but
between GQ1b and C jejuni lipo-oligosaccharides. The development of either AMAN or FS following
C jejuni infection depends on which ganglioside-like lipo-oligosaccharides are expressed by C jejuni
strains as a result of the bacterial genetic polymorphism. Bickerstaff brainstem encephalitis share
common fi ndings of anti-GQ1b antibodies with FS making the two disorders related, thus extending
the spectrum of the GBS phenotype.
- Full text:P020150902414198177361.pdf
