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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. [ABSTRACT FROM AUTHOR]

Ataxic Guillain-Barré syndrome (GBS) associated with anti-GQ1b IgG antibody has been reported. We, however, have had a patient with ataxic GBS who had IgG antibodies to the minor gangliosides GM1b and GalNAc-GD1a, and we therefore retrospectively investigated the clinical features of patients who had antibodies to GM1b or GalNAc-GD1a, but not to GQ1b. Information on patients’ antecedent illnesses, initial symptoms, neurological signs, and CSF findings was reviewed in those with ataxic GBS or Fisher syndrome (FS) with anti-GM1b or anti-GalNAc-GD1a IgG antibodies. We tested whether the anti-GM1b and anti-GalNAc-GD1a antibodies are cross-reactive and constructed three-dimensional structural models of GM1b and GalNAc-GD1a. Ataxic GBS was diagnosed in 1 of 65 patients who had both anti-GM1b and anti-GalNAc-GD1a antibodies and in 3 of 159 patients who had anti-GM1b antibody without anti-GalNAc-GD1a antibody: FS was diagnosed in 1 of the 159 patients and in 1 of 35 who had anti-GalNAc-GD1a antibody without anti-GM1b antibody. All the patients’ antibodies to GM1b or GalNAc-GD1a were associated with the IgG isotype. The clinical features of patients with ataxic GBS associated with anti-GM1b or anti-GalNAc-GD1a IgG antibodies did not differ from those of patients who had anti-GQ1b IgG antibody. Absorption study findings for serum from the patient who had both anti-GM1b and anti-GalNAc-GD1a IgG antibodies showed significant absorbance of anti-GM1b IgG antibody by GalNAc-GD1a and of anti-GalNAc-GD1a IgG antibody by GM1b, indicating that these antibodies are cross-reactive. This is the first report of ataxic GBS or FS associated with anti-GM1b or anti-GalNAc-GD1a IgG antibodies. These autoantibodies, as well as anti-GQ1b IgG antibody, may function in the development of some patients with ataxic GBS and FS. [ABSTRACT FROM AUTHOR]