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Bickerstaff's brainstem encephalitis: can it recur?
1Division of Neurology, National University Hospital, 5 Lower Kent Ridge Road, Singapore 119074, Singapore.
Abstract:
Bickerstaff's brain-stem encephalitis is usually a monophasic post-viral inflammatory illness characterized by progressive ophthalmoplegia, ataxia and disturbance of consciousness (or hyper-reflexia). Since the clinical spectrum of Bickerstaff encephalitis may overlap with the Miller-Fisher and Guillain-Barré syndromes, the presence of anti- GQ1b antibodies and abnormal brain MRI can help to support its diagnosis. However, absence of anti-GQ1b antibodies and normal MRI do not exclude the diagnosis, which remains based on clinical criteria and exclusion of other etiologies. We report a case of recurrent Bickerstaff's brainstem encephalitis with no identifiable antecedent illness, and overlapping features of Miller Fisher and Guillain-Barré syndromes, in the presence of negative anti-GQ1b antibodies and repeatedly normal MRI of the brain.
Insights
Bickerstaff's brainstem encephalitis can recur and present atypically. This case highlights recurrent Bickerstaff encephalitis with overlapping syndromes, normal MRI, and negative anti-GQ1b antibodies, challenging typical diagnostic criteria.
Area of Science:
- Neurology
- Neuroimmunology
- Inflammatory Brainstem Encephalitis
Background:
- Bickerstaff's brainstem encephalitis (BBE) is typically a monophasic post-viral inflammatory condition.
- BBE often presents with ophthalmoplegia, ataxia, and consciousness disturbances.
- Diagnostic support includes anti-GQ1b antibodies and brain MRI, though their absence doesn't exclude BBE.
Observation:
- This report details a rare case of recurrent Bickerstaff's brainstem encephalitis.
- The patient exhibited overlapping clinical features of Miller Fisher Syndrome and Guillain-Barré Syndrome.
- Crucially, the patient had no identifiable antecedent illness, negative anti-GQ1b antibodies, and normal brain MRIs.
Findings:
- Recurrent BBE can occur without a clear preceding illness.
- Clinical presentation can mimic or overlap with other autoimmune neuropathies.
- Diagnostic certainty for BBE may rely on clinical criteria and exclusion, even with atypical serological and imaging findings.
Implications:
- This case expands the known clinical spectrum of Bickerstaff's brainstem encephalitis.
- It underscores the importance of considering BBE in recurrent neurological presentations, even with negative biomarkers.
- Highlights the need for careful clinical evaluation and differential diagnosis in complex autoimmune neurological disorders.
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