Bickerstaff brainstem encephalitis (BBE) is a rare inflammatory demyelinating condition, which is similar to Miller-Fisher syndrome (MFS). Ophthalmoplegia and ataxia are common to these syndromes but unlike MFS, BBE is also characterised by central nervous system involvement, most commonly in the form of altered consciousness. BBE usually has a very good prognosis. We present a case of a young female with BBE. Unlike the majority of BBE patients, she (1) was negative for anti-GBQ1b antibodies but positive for glutamic acid decarboxylase (GAD) antibodies and borderline positive for voltage-gated calcium channel antibodies and (2) had a delayed recovery post treatment with intravenous immunoglobulins and plasma exchange. We contemplate a potential role for GAD antibodies as a marker for longer recovery time in patients with BBE.
- brain stem / cerebellum
- infection (neurology)
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Contributors HJ: planned to write the case report; major role in acquisition and analysis of all the necessary investigations and information related to the patient; wrote the case report draft after thorough literature review; participated in the final approval of the version to be published. ZS: supervised and revised the case report; major role in acquisition and analysis of data; provided the final approval of the version to be published. UE: played a role in writing the case report draft and literature review. Participated in the final approval of the version to be published.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.