ArticleNeurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology2026
Bickerstaff brainstem encephalitis: a 13-case series and literature review.
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Abstract
objectiveTo analyze the clinical characteristics, diagnostic features, treatment strategies, and prognosis of Bickerstaff brainstem encephalitis (BBE), providing insights into its diagnosis and management.
methodsA retrospective analysis was conducted on 13 BBE cases treated at a single center from January 2021 to December 2024. Clinical manifestations, diagnostic criteria, treatment regimens, and outcomes were reviewed and summarized of the 13 BBE cases. A comprehensive literature review was also conducted to fully understand the progress of the current study.
resultsAll patients presented with acute onset, with 6 (46%) having a history of preceding infections, predominantly upper respiratory tract infections (5,38%). Diplopia (5,38%) was the most common initial symptom, while ataxia (13,100%) and ophthalmoplegia (10,76%) were the core features. Anti-GQ1b antibodies were positive in 5 patients (38%), and MRI abnormalities in the brainstem were observed in 2 patients (15%). Combination therapy with intravenous immunoglobulin (IVIg) and steroids was administered to 8 patients (62%), while monotherapy was used in the remainder. At discharge, 1 patient (7%) achieved complete symptom resolution, 7 (54%) showed improvement, and 3 (23%) experienced symptom aggravation. No fatalities were reported, but residual limb weakness persisted in some cases.
conclusionBBE is clinically heterogeneous, often overlapping with GBS and MFS. Diagnosis relies on clinical findings, anti-GQ1b antibody testing, and neuroimaging. Immunotherapy, especially IVIg combined with steroids, is effective in most cases. However, rapid progression and limb weakness at onset may predict poor outcomes, highlighting the need for early intervention and further research to improve the diagnosis and treatment strategies.
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