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  • Atypical Bickerstaff brainstem encephalitis: ataxic hypersomnolence without ophthalmoplegia.

Atypical Bickerstaff brainstem encephalitis: ataxic hypersomnolence without ophthalmoplegia.

Journal of neurology, neurosurgery, and psychiatry (2013-04-09)
Benjamin R Wakerley, Derek Soon, Yee-Cheun Chan, Nobuhiro Yuki
ABSTRACT

Clinical and immunological evaluation of 'incomplete' Bickerstaff brainstem encephalitis (BBE). We studied two patients with postinfectious brainstem syndromes who presented at National University Hospital Singapore. Laboratory work-up included measurement of antiganglioside antibodies. Both patients displayed hypersomnolence and cerebellar-like ataxia in the absence of external ophthalmoplegia and carried high serum titres of IgG anti-GQ1b antibodies, strongly indicative of BBE. Ophthalmoplegia can be absent or incomplete in BBE, and the absence of this clinical feature should not exclude BBE from the clinicians' differential. Such cases of incomplete BBE could be defined as 'ataxic hypersomnolence without ophthalmoplegia'.

MATERIALS
Product Number
Brand
Product Description

Sigma-Aldrich
Disialoganglioside GD1a from bovine brain, ≥95% (TLC), lyophilized powder
Sigma-Aldrich
Monosialoganglioside GM1 from bovine brain, ≥95%, lyophilized powder
Sigma-Aldrich
Monosialoganglioside GM1 from bovine brain, lyophilized powder, BioXtra, γ-irradiated, ≥95% (TLC)