Bickerstaff's brainstem encephalitis and Fisher syndrome form a continuous spectrum
Whether Bickerstaff's brainstem encephalitis (BBE) is a distinct disease or a subtype of Fisher syndrome (FS) is unclear as there have been no clinical studies with sufficiently large numbers of patients with FS or BBE. Our aim was to clarify the nosological relationship. Medical records of patients suffering acute ophthalmoplegia and ataxia within four weeks of onset were reviewed. BBE was the diagnosis for patients with impaired consciousness, FS for those with clear consciousness and areflexia. Clinical features, neuroimages, and laboratory findings were analyzed. Patients were grouped as having BBE (n = 53), FS (n = 466), or as unclassified (n = 62). The BBE and FS groups had similar features; positive serum anti-GQ1b IgG antibody (68 % versus 83 %), antecedent Campylobacter jejuni infection (23 % versus 21 %), CSF albuminocytological dissociation (46 % versus 76 %), brain MRI abnormality (11 % versus 2 %), and abnormal EEG findings (57 % versus 25 %). BBE (n = 4) and FS (n = 28) subgroups underwent detailed electrophysiological testing. Both groups frequently showed absent soleus H-reflexes, but normal sensory nerve conduction (75 % versus 74 %) and a 1-Hz power spectrum peak on postural body sway analysis (67 % versus 72 %). Common autoantibodies, antecedent infections, and MRI and neurophysiological results found in this large study offer conclusive evidence that Bickerstaff's brainstem encephalitis and Fisher syndrome form a continuous spectrum with variable CNS and PNS involvement.
Key wordsAnti-GQ1b antibody Bickerstaff's brainstem encephalitis Campylobacter jejuni Fisher syndrome Guillain-Barré syndrome
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- 4.Bickerstaff ER (1957) Brain-stem encephalitis: further observations on a grave syndrome with benign prognosis. Br Med J i:5032:1384–1387Google Scholar
- 5.Bickerstaff ER, Cloake PCP (1951) Mesencephalitis and rhombencephalitis. Br Med J ii:4723:77–81Google Scholar
- 12.Gilbert M, Karwaski MF, Bernatchez S, Young NM, Taboada E, Michniewicz J, Cunningham AM, Wakarchuk WW (2002) The genetic bases for the variation in the lipo-oligosaccharide of the mucosal pathogen, Campylobacter jejuni: biosynthesis of sialylated ganglioside mimics in the core oligosaccharide. J Biol Chem 277:327–337PubMedCrossRefGoogle Scholar
- 13.Hughes RAC, Raphaël JC, Swan AV, van Doorn PA (2006) Intravenous immunoglobulin for Guillain-Barré syndrome. Cochrane Database Syst Rev:CD002063Google Scholar
- 28.Overell J, Hsieh S, Odaka M, Yuki N, Willison H (2007) Treatment for Fisher syndrome, Bickerstaff 's brainstem encephalitis and related disorders. Cochrane Database Syst Rev CD004761Google Scholar
- 29.Raphaël JC, Chevret S, Hughes RAC, Annane D (2002) Plasma exchange for Guillain-Barré syndrome. Cochrane Database Syst Rev CD001798Google Scholar