Normal Early Investigations Do Not Exclude Bickerstaff Brainstem Encephalitis: Highlighting Diagnostic and Treatment Pitfalls
- 1Faculty of Medicine Arab American University (AAUP) Jenin State of Palestine aauj.edu
- 2Department of Internal Medicine Ibn Sina Hospital Jenin State of Palestine ibnsinatrust.com
Abstract
Bickerstaff brainstem encephalitis (BBE) is a rare immune‐mediated disorder characterized by ophthalmoplegia, cerebellar ataxia, and impaired consciousness. It is now recognized as a variant of Guillain–Barré syndrome (GBS), sharing common pathogenic mechanisms with Miller Fisher syndrome. However, early diagnosis remains challenging, particularly when initial investigations are normal. We report a 20‐year‐old previously healthy woman presenting with rapidly progressive ophthalmoplegia, bulbar dysfunction, flaccid quadriparesis, areflexia, and impaired consciousness following a febrile upper respiratory illness. Early cerebrospinal fluid analysis and brain MRI were normal. Anti‐GQ1b antibodies were positive, and nerve conduction studies showed isolated proximal conduction abnormalities with absent F‐waves. The patient developed respiratory failure requiring mechanical ventilation and was treated with an extended course of plasma exchange, resulting in near‐complete recovery. This case highlights a critical diagnostic pitfall: BBE, a variant of GBS, can present with a life‐threatening brainstem syndrome despite entirely normal early investigations. We emphasize the value of recognizing the paradoxical combination of areflexia and extensor plantar responses and the use of F‐wave studies as an early electrophysiological foothold when standard studies are unrevealing. Furthermore, it demonstrates that even in catastrophic, ventilator‐dependent presentations, an extended course of plasma exchange can lead to a favorable outcome, suggesting a potential therapeutic strategy for severe presentations of BBE.
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BibTeX
@article{kmail2026normal,
title = {{Normal Early Investigations Do Not Exclude Bickerstaff Brainstem Encephalitis: Highlighting Diagnostic and Treatment Pitfalls}},
author = {Hidaya Kmail and Mohammad Jaradat and Ahmad Abbas and Omar Abu Zaineh and Alaa Kharouf and Qasem N. AlShaer},
journal = {Case Reports in Neurological Medicine},
year = {2026},
month = oct,
doi = {10.1155/crnm/8222960},
url = {https://doi.org/10.1155/crnm/8222960},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Normal Early Investigations Do Not Exclude Bickerstaff Brainstem Encephalitis: Highlighting Diagnostic and Treatment Pitfalls
AU - Hidaya Kmail
AU - Mohammad Jaradat
AU - Ahmad Abbas
AU - Omar Abu Zaineh
AU - Alaa Kharouf
AU - Qasem N. AlShaer
PY - 2026
DA - 2026/10/26
JO - Case Reports in Neurological Medicine
DO - 10.1155/crnm/8222960
UR - https://doi.org/10.1155/crnm/8222960
AB - Bickerstaff brainstem encephalitis (BBE) is a rare immune‐mediated disorder characterized by ophthalmoplegia, cerebellar ataxia, and impaired consciousness. It is now recognized as a variant of Guillain–Barré syndrome (GBS), sharing common pathogenic mechanisms with Miller Fisher syndrome. However, early diagnosis remains challenging, particularly when initial investigations are normal. We report a 20‐year‐old previously healthy woman presenting with rapidly progressive ophthalmoplegia, bulbar dysfunction, flaccid quadriparesis, areflexia, and impaired consciousness following a febrile upper respiratory illness. Early cerebrospinal fluid analysis and brain MRI were normal. Anti‐GQ1b antibodies were positive, and nerve conduction studies showed isolated proximal conduction abnormalities with absent F‐waves. The patient developed respiratory failure requiring mechanical ventilation and was treated with an extended course of plasma exchange, resulting in near‐complete recovery. This case highlights a critical diagnostic pitfall: BBE, a variant of GBS, can present with a life‐threatening brainstem syndrome despite entirely normal early investigations. We emphasize the value of recognizing the paradoxical combination of areflexia and extensor plantar responses and the use of F‐wave studies as an early electrophysiological foothold when standard studies are unrevealing. Furthermore, it demonstrates that even in catastrophic, ventilator‐dependent presentations, an extended course of plasma exchange can lead to a favorable outcome, suggesting a potential therapeutic strategy for severe presentations of BBE.
SN - 2090-6668
SN - 2090-6676
ER -