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Bickerstaff's brainstem encephalitis associated with anti-GM1 and anti-GD1a antibodies
被引:1
|作者:
Cleaver, Jonathan
[1
]
James, Richard
[2
]
Chohan, Gurjit
[1
]
Lyons, Paul
[1
]
机构:
[1] Royal United Hosp Bath NHS Fdn Trust, Dept Neurol, Bath, Avon, England
[2] Royal United Hosp Bath NHS Fdn Trust, Dept Neuroradiol, Bath, Avon, England
关键词:
neurology;
brain stem;
cerebellum;
cranial nerves;
neuro ITU;
CLINICAL-FEATURES;
FISHER SYNDROME;
D O I:
10.1136/bcr-2020-236545
中图分类号:
R5 [内科学];
学科分类号:
1002 ;
100201 ;
摘要:
Bickerstaff's brainstem encephalitis (BBE) is a Guillain-Barre 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.
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