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Is ‘Bickerstaff brainstem encephalitis’ really encephalitis?
  1. Satoshi Kuwabara,
  2. Sonoko Misawa,
  3. Masahiro Mori
  1. Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan
  1. Correspondence to Dr Satoshi Kuwabara, Department of Neurology, Graduate School of Medicine, Chiba University, 1-8-1 Inohana, Chuo-ku, Chiba 260-8670, Japan; kuwabara-s{at}

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Bickerstaff brainstem encephalitis (BBE) is characterised by acute ophthalmoplegia, ataxia, and some features suggesting CNS involvement such as consciousness disturbance. In 1951, Bickerstaff and Cloake described three patients in their original report entitled ‘Mesencephalitis and rhomboencephalitis’, and in 1957 Bickerstaff expanded his series to eight patients and proposed the condition ‘brainstem encephalitis’, subsequently termed ‘BBE’. Between the two publications, in 1956 Miller Fisher described three patients with “an unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia)”; this condition has been widely recognised as Fisher syndrome (FS). A detailed historical perspective of BBE and FS was presented in a review …

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