Bickerstaff's brainstem encephalitis associated with anti-GM1 and anti-GD1a antibodies.

Jonathan Cleaver, Richard James, Gurjit Chohan, Paul Lyons

BMJ case reports 2020 Sep 18

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Bickerstaff's brainstem encephalitis (BBE) is a Guillain-Barré syndrome (GBS) spectrum disorder associated with predominantly central nervous system predilection. Patients exhibit a variable constellation of depressed consciousness, bilateral external ophthalmoplegia, ataxia and long tract signs. Although the pathophysiology is not fully understood, it has been associated with anti-GQ1b antibodies in two-thirds of patients. We present a patient with clinical features consistent with BBE and positive anti-GM1 and anti-GD1a antibodies. A diagnostic approach to the acutely unwell patient with brainstem encephalitis is explored in this clinical context with a literature review of the aforementioned ganglioside antibody significance. Intravenous immunoglobulin therapy is highlighted in BBE using up-to-date evidence-based extrapolation from GBS. © BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.

Citation

Jonathan Cleaver, Richard James, Gurjit Chohan, Paul Lyons. Bickerstaff's brainstem encephalitis associated with anti-GM1 and anti-GD1a antibodies. BMJ case reports. 2020 Sep 18;13(9)