An atypical Bickerstaff's brainstem encephalitis with involvement of spinal cord

Neuroradiol J. 2016 Oct;29(5):396-9. doi: 10.1177/1971400916665383. Epub 2016 Aug 18.

Abstract

Bickerstaff brainstem encephalitis (BBE) is a rare neurological disease that generally has a good prognosis. We describe an atypical case of a patient with severe BBE; the presentation was uncommon because of the lack of ophthalmoplegia and because of evidence of both peripheral neuropathy and brainstem encephalitis. The article reports clinical and biochemical evaluation and focuses on magnetic resonance imaging (MRI) findings in diagnosis and management of the patient. Notably, we found a previously unreported dramatic spinal cord involvement on MRI. We believe these findings could add to diagnostic tools, and that this case may represent a new variant of BBE with more aggressive behavior.

Keywords: Bickerstaff’s brainstem encephalitis; Guillain–Barré syndrome; MRI; Miller Fisher syndrome; anti-GQ1b antibody.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Antigens, CD1 / metabolism
  • Ataxia / diagnostic imaging
  • Ataxia / etiology*
  • Autoantibodies / metabolism
  • Brain Stem / diagnostic imaging
  • Brain Stem / pathology*
  • Encephalitis / complications*
  • Encephalitis / diagnostic imaging
  • Encephalitis / pathology*
  • Female
  • Gangliosides / immunology
  • Humans
  • Image Processing, Computer-Assisted
  • Magnetic Resonance Imaging

Substances

  • Antigens, CD1
  • Autoantibodies
  • Gangliosides
  • GQ1b ganglioside