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[Fisher Syndrome and Bickerstaff Brainstem Encephalitis]
1Department of Neurology, Graduate School of Medicine, Chiba University.
Brain and Nerve = Shinkei Kenkyu No Shinpo
|November 13, 2015
Summary
Fisher syndrome and Bickerstaff brainstem encephalitis are inflammatory neurological disorders. Discovery of anti-GQ1b IgG antibodies suggests they are part of a single disease spectrum, with Bickerstaff brainstem encephalitis being a variant of Fisher syndrome.
Area of Science:
- Neurology
- Immunology
- Neuroimmunology
Context:
- Fisher syndrome (FS) and Bickerstaff brainstem encephalitis (BBE) are distinct neurological disorders.
- FS involves peripheral nerves (ophthalmoplegia, ataxia, areflexia).
- BBE affects the central nervous system (ophthalmoplegia, ataxia, consciousness disturbance).
Purpose:
- To explore the relationship between Fisher syndrome and Bickerstaff brainstem encephalitis.
- To investigate the role of anti-GQ1b IgG antibodies in both conditions.
- To elucidate the underlying pathophysiology and disease spectrum.
Summary:
- Both FS and BBE share features like preceding infections, albumin-cytological dissociation, and links to Guillain-Barré syndrome.
- The presence of anti-GQ1b IgG antibodies is a key finding supporting a unified disease spectrum.
- Lesions are linked to GQ1b ganglioside expression in peripheral and central nervous systems.
- BBE is proposed as a variant of FS with CNS involvement.
Impact:
- Reclassifies BBE as a CNS variant of FS, refining diagnostic criteria.
- Highlights the diagnostic significance of anti-GQ1b IgG antibodies in neuroinflammatory disorders.
- Advances understanding of GQ1b-related autoimmune neuropathies and encephalitides.
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