Bickerstaff's brainstem encephalitis associated with anti-GM1 and anti-GD1a antibodies. Issue 9 (18th September 2020)

• Record Type: Journal Article
• Authors: Cleaver, Jonathan; James, Richard; Chohan, Gurjit; Lyons, Paul
• Is Part Of: BMJ case reports. Volume 13:Issue 9(2020)
• Publication Date: 2020-09-18
• Subjects: neurology -- brain stem / cerebellum -- cranial nerves -- neuro ITU; Medicine -- Case studies -- Periodicals; 610.5
• DOI: 10.1136/bcr-2020-236545
• Languages: English
• ISSNs: 1757-790X

Abstract

Abstract : 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.