Optic neuropathy and decorticate-like posture as presenting symptoms of Bickerstaff's brainstem encephalitis: A case report and literature review

Clin Neurol Neurosurg. 2018 Oct:173:159-162. doi: 10.1016/j.clineuro.2018.08.019. Epub 2018 Aug 16.

Abstract

A 72-year-old woman with a 10-day history of bilateral visual impairment after respiratory tract infection showed decorticate-like posture and progressive deterioration of consciousness leading to coma. Ophthalmoplegia was also noted and anti-GQ1b antibodies were positive, consistent with Bickerstaff's brainstem encephalitis. After intravenous immunoglobulin and steroid pulse therapy, her consciousness gradually improved. However, severe visual impairment at the level of hand motion was noticed, which gradually normalized after second steroid pulse therapy. Atypical findings including optic neuropathy and decorticate-like posture can be seen in patients with Bickerstaff's brainstem encephalitis, and early diagnosis is essential for adequate management.

Keywords: Bickerstaff’s brainstem encephalitis; Decorticate-like posture; Optic neuropathy; Spindle coma; Visual evoked potentials (VEP); anti-GQ1b antibody.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Autoantibodies
  • Coma / complications
  • Consciousness / physiology
  • Encephalitis / diagnosis
  • Encephalitis / surgery*
  • Gangliosides / immunology
  • Humans
  • Immunoglobulins, Intravenous
  • Optic Nerve Diseases / complications
  • Optic Nerve Diseases / surgery*
  • Optic Neuritis / complications
  • Optic Neuritis / surgery*
  • Posture / physiology*

Substances

  • Autoantibodies
  • Gangliosides
  • Immunoglobulins, Intravenous