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Related Experiment Video

Updated: Jan 17, 2026

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Bickerstaff Brainstem Encephalitis with a Decorticate-like Posture, Involuntary Movements, Impaired Visual Acuity,

Kei Ishizuchi1, Jun Sakai1, Ryotaro Okochi1

  • 1Department of Neurology, Keio University School of Medicine, Japan.

Internal Medicine (Tokyo, Japan)
|January 14, 2026
PubMed
Summary

Bickerstaff brainstem encephalitis (BBE) is an immune disorder typically diagnosed by specific symptoms. This case highlights rare BBE features, including coma, that can cause prolonged weakness, emphasizing the need for early diagnosis.

Keywords:
Bickerstaff brainstem encephalitisdecorticate postureimpaired visual acuityinvoluntary movements

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Area of Science:

  • Neurology
  • Immunology
  • Neuroimmunology

Background:

  • Bickerstaff brainstem encephalitis (BBE) is an acute immune-mediated neurological disorder.
  • It typically presents with consciousness disturbance, external ophthalmoplegia, and ataxia.
  • While usually diagnosed and treated effectively, atypical presentations can complicate diagnosis and prognosis.

Purpose of the Study:

  • To describe a case of BBE with uncharacteristic features.
  • To discuss the diagnostic challenges posed by atypical presentations.
  • To underscore the importance of early diagnosis despite rare manifestations.

Main Methods:

  • Case report of a Japanese patient with BBE.
  • Clinical observation of presenting symptoms, disease course, and recovery.
  • Review of diagnostic criteria and literature for BBE.

Main Results:

  • The patient exhibited atypical BBE features: decorticate posture, involuntary movements, impaired visual acuity, coma, and intubation.
  • The patient experienced prolonged weakness requiring orthosis-assisted ambulation for 10 months.
  • Despite atypical features, characteristic BBE signs aided in diagnosis.

Conclusions:

  • BBE can present with rare, uncharacteristic features that may obscure diagnosis.
  • Coma and intubation in BBE can be associated with prolonged weakness.
  • Early diagnosis of BBE remains crucial, even with atypical presentations, for timely management and improved outcomes.