Bickerstaff's brainstem encephalitis associated with anti-GM1 and anti-GD1a antibodies.
brain stem / cerebellum
cranial nerves
neuro ITU
neurology
Journal
BMJ case reports
ISSN: 1757-790X
Titre abrégé: BMJ Case Rep
Pays: England
ID NLM: 101526291
Informations de publication
Date de publication:
18 Sep 2020
18 Sep 2020
Historique:
entrez:
19
9
2020
pubmed:
20
9
2020
medline:
20
2
2021
Statut:
epublish
Résumé
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.
Identifiants
pubmed: 32948528
pii: 13/9/e236545
doi: 10.1136/bcr-2020-236545
pmc: PMC7511636
pii:
doi:
Substances chimiques
Autoantibodies
0
Gangliosides
0
ganglioside, GD1a
12707-58-3
G(M1) Ganglioside
37758-47-7
Types de publication
Journal Article
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Informations de copyright
© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.
Déclaration de conflit d'intérêts
Competing interests: None declared.
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