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Review
Bickerstaff brainstem encephalitis and Fisher syndrome: anti-GQ1b antibody syndrome
  1. Nortina Shahrizaila1,
  2. Nobuhiro Yuki2
  1. 1Division of Neurology, Department of Medicine, Faculty of Medicine, University of Malaya, Malaysia
  2. 2Department of Medicine, Yong Loo Lin School of Medicine, National University of Singapore, Singapore
  1. Correspondence to Professor Nobuhiro Yuki, Department of Medicine, Unit 09-01, National University of Singapore, Centre for Translational Medicine, 14 Medical Drive, Singapore 117599, Singapore; yuki.research{at}gmail.com

Abstract

In the 1950s, Bickerstaff and Fisher independently described cases with a unique presentation of ophthalmoplegia and ataxia. The neurological features were typically preceded by an antecedent infection and the majority of patients made a spontaneous recovery. In the cases with Bickerstaff brainstem encephalitis, there was associated altered consciousness and in some, hyperreflexia, in support of a central pathology whereas in Fisher syndrome, patients were areflexic in keeping with a peripheral aetiology. However, both authors recognised certain similarities to Guillain–Barré syndrome such as the presence of peripheral neuropathy and cerebrospinal fluid albuminocytological dissociation. The discovery of immunoglobulin G anti-GQ1b antibodies in patients with Fisher syndrome and later in Bickerstaff brainstem encephalitis was crucial in providing the necessary evidence to conclude that both conditions were in fact part of the same spectrum of disease by virtue of their common clinical and immunological profiles. Following this, other neurological presentations that share anti-GQ1b antibodies emerged in the literature. These include acute ophthalmoparesis and acute ataxic neuropathy, which represent the less extensive spectrum of the disease whereas pharyngeal-cervical-brachial weakness and Fisher syndrome overlap with Guillain–Barré syndrome represent the more extensive end of the spectrum. The conditions can be referred to as the ‘anti-GQ1b antibody syndrome’. In this review, we look back at the historical descriptions and describe how our understanding of Fisher syndrome and Bickerstaff brainstem encephalitis has evolved from their initial descriptions more than half a century ago.

  • Bickerstaff brainstem encephalitis
  • Fisher syndrome
  • anti-GQ1b antibody
  • Guillain–Barré syndrome
  • neuromuscular
  • neuropathy
  • ganglioside
  • CIDP
  • Miller Fisher syndrome

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Footnotes

  • Funding Dr Shahrizaila receives grant support from University of Malaya (RG351/11HTM) and Dr Yuki receives support from the National Medical Research Council, Singapore (IRG 10nov086).

  • Competing interests None.

  • Provenance and peer review Commissioned; externally peer reviewed.

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