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Bickerstaff brainstem encephalitis: clinical, neurophysiological, laboratory and postmortem findings of a case presenting as encephalomyelitis
  1. Ibrahim Imam1,
  2. Ptolemaios G Sarrigiannis2 and
  3. Aditya G Shivane3
  1. 1Neurology, Royal Devon and Exeter NHS Foundation Trust, Exeter, Devon, UK
  2. 2Neurophysiology, Royal Devon and Exeter NHS Foundation Trust, Exeter, Devon, UK
  3. 3Department of Cellular and Anatomical Pathology, University Hospitals Plymouth NHS Trust, Plymouth, UK
  1. Correspondence to Dr Ibrahim Imam; ibrahimimam2000{at}yahoo.com

Abstract

Our patient was admitted to hospital with a 1-week history of an upper respiratory tract infection and a rapidly progressive encephalopathy dominated by brainstem features and widespread areflexia. Her antiganglioside antibodies and electroencephalography were consistent with Bickerstaff brainstem encephalitis (BBE), and her postmortem examination revealed a predominantly florid brainstem encephalitis and myelitis. Her sputum and throat swabs isolated Haemophilus influenzae and Fusobacterium, respectively, the former being the most probable trigger of BBE. Our patient’s death, despite the otherwise good prognosis of the disorder, may reflect the severity of the pathological changes at postmortem or the association of comorbid disorders such as sepsis-associated encephalopathy. Her poor outcome may also be an indication to treat rapidly progressive cases of BBE with more than one immune modulating drug.

  • brain stem/cerebellum
  • clinical neurophysiology

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Footnotes

  • Contributors II managed the patient, wrote the draft and approved the final version. PGS provided the neurophysiological report, contributed to the case report and approved the final version. AS provided the neuropathological report, contributed to the case report and approved the final version.

  • 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.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

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