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Rare disease
Case report
Case of Bickerstaff encephalitis with positive glutamic acid decarboxylase antibodies
  1. Hussnain Javaid1,
  2. Urwa Ejaz1 and
  3. Zbigniew Slowinski2
  1. 1General Medicine, Airedale General Hospital, Keighley, West Yorkshire, UK
  2. 2Neurology, Airedale General Hospital, Keighley, West Yorkshire, UK
  1. Correspondence to Dr Hussnain Javaid; hussnainamc{at}gmail.com

Abstract

Bickerstaff brainstem encephalitis (BBE) is a rare inflammatory demyelinating condition, which is similar to Miller-Fisher syndrome (MFS). Ophthalmoplegia and ataxia are common to these syndromes but unlike MFS, BBE is also characterised by central nervous system involvement, most commonly in the form of altered consciousness. BBE usually has a very good prognosis. We present a case of a young female with BBE. Unlike the majority of BBE patients, she (1) was negative for anti-GBQ1b antibodies but positive for glutamic acid decarboxylase (GAD) antibodies and borderline positive for voltage-gated calcium channel antibodies and (2) had a delayed recovery post treatment with intravenous immunoglobulins and plasma exchange. We contemplate a potential role for GAD antibodies as a marker for longer recovery time in patients with BBE.

  • brain stem / cerebellum
  • infection (neurology)
  • non-vascular

https://doi.org/10.1136/bcr-2020-234814

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    Footnotes

    • Contributors HJ: planned to write the case report; major role in acquisition and analysis of all the necessary investigations and information related to the patient; wrote the case report draft after thorough literature review; participated in the final approval of the version to be published. ZS: supervised and revised the case report; major role in acquisition and analysis of data; provided the final approval of the version to be published. UE: played a role in writing the case report draft and literature review. Participated in the final approval of the version to be published.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient consent for publication Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.