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Published on: March 26, 2019
Bickerstaff's brainstem encephalitis presenting to the ICU
Gary Hunter1, G Bryan Young, Lee Cyn Ang
1Division of Neurology, Department of Medicine, University of Saskatchewan, 112 3502 Taylor St. E., Saskatoon, SK, S7V 1J1, Canada. grwhunter@gmail.com
Background:
Bickerstaff's brainstem encephalitis continues to pose a diagnostic and treatment challenge since the original descriptions by Bickerstaff and Miller-Fisher. The clinical syndrome overlaps with AIDP and MFS, but is accompanied by decreased level of consciousness not attributable to other causes, and the variable presence of long-tract signs.
Methods:
The methods in this study include the case presentation with autopsy findings of an elderly male presented with progressive weakness and impairment of consciousness and the literature review.
Results:
Examination revealed areflexia and loss of most brainstem reflexes. Some improvement occurred after several weeks in the ICU, prior to death from pulmonary embolism. Pathologic specimens were similar to others in the literature, with inflammatory changes in nerve roots and brainstem.
Conclusions:
The above findings led us to conclude that Bickerstaff's brainstem encephalitis remains a clinical diagnosis despite advances in electrophysiologic testing and neuroimaging. BBE likely represents part of a spectrum, overlapping with AIDP and MFS. Immunomodulation may be helpful in shortening the clinical course.
Insights
Bickerstaff's brainstem encephalitis (BBE) is challenging to diagnose and treat, overlapping with other conditions. Early immunomodulation may improve outcomes for this rare neurological disorder.
Area of Science:
- Neurology
- Neuroimmunology
Background:
- Bickerstaff's brainstem encephalitis (BBE) presents diagnostic and therapeutic challenges.
- Clinical overlap exists with Guillain-Barré syndrome (GBS) and Miller Fisher syndrome (MFS).
- Key features include impaired consciousness and long-tract signs.
Observation:
- A case study of an elderly male with progressive weakness and altered consciousness.
- Autopsy revealed inflammatory changes in nerve roots and brainstem.
- Clinical course showed areflexia and loss of brainstem reflexes.
Findings:
- BBE diagnosis remains primarily clinical, despite advanced testing.
- Pathological findings consistent with literature on BBE.
- The condition may represent a spectrum with GBS and MFS.
Implications:
- BBE diagnosis requires careful clinical evaluation.
- Early immunomodulatory therapy may shorten the disease course.
- Further research into BBE's spectrum and treatment is warranted.
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