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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Univ Lille, Hop R Salengro, Dept Neurol, Lille, FranceUniv Lille, Hop R Salengro, Dept Neurol, Lille, France
Simon, Olivier
Lacour, Arnaud
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Univ Lille, Hop R Salengro, Dept Neurol, Lille, France
Ctr Rare Neuromuscular Dis, Lille, FranceUniv Lille, Hop R Salengro, Dept Neurol, Lille, France
Lacour, Arnaud
Delval, Arnaud
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Univ Lille, Hop R Salengro, Dept Neurol, Lille, FranceUniv Lille, Hop R Salengro, Dept Neurol, Lille, France
Delval, Arnaud
Beaume, Anne
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Univ Poitiers, Hosp La Miletrie, Dept Immunol Inflammat, Poitiers, FranceUniv Lille, Hop R Salengro, Dept Neurol, Lille, France
Beaume, Anne
Vermersch, Patrick
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Univ Lille, Hop R Salengro, Dept Neurol, Lille, France
Ctr Rare Neuromuscular Dis, Lille, FranceUniv Lille, Hop R Salengro, Dept Neurol, Lille, France
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Univ Roma Tor Vergata, Dept Syst Med, Inst Neurol, Unit Dysimmune Neuropathies, Rome, ItalyUniv Roma Tor Vergata, Dept Syst Med, Inst Neurol, Unit Dysimmune Neuropathies, Rome, Italy
Studer, V
Mataluni, G.
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Univ Roma Tor Vergata, Dept Syst Med, Inst Neurol, Unit Dysimmune Neuropathies, Rome, ItalyUniv Roma Tor Vergata, Dept Syst Med, Inst Neurol, Unit Dysimmune Neuropathies, Rome, Italy
Mataluni, G.
Rocchi, C.
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Univ Roma Tor Vergata, Dept Syst Med, Inst Neurol, Unit Dysimmune Neuropathies, Rome, ItalyUniv Roma Tor Vergata, Dept Syst Med, Inst Neurol, Unit Dysimmune Neuropathies, Rome, Italy
Rocchi, C.
Marfia, G. A.
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Univ Roma Tor Vergata, Dept Syst Med, Inst Neurol, Unit Dysimmune Neuropathies, Rome, ItalyUniv Roma Tor Vergata, Dept Syst Med, Inst Neurol, Unit Dysimmune Neuropathies, Rome, Italy
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Peoples Hosp Suzhou New Dist, Dept Neurol, Suzhou, Jiangsu, Peoples R ChinaPeoples Hosp Suzhou New Dist, Dept Neurol, Suzhou, Jiangsu, Peoples R China
Zhang, Limei
Ma, Linqing
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Peoples Hosp Suzhou New Dist, Dept Neurol, Suzhou, Jiangsu, Peoples R China
Peoples Hosp Suzhou New Dist, Dept Neurol, 95 Huashan Rd, Suzhou 215129, Jiangsu, Peoples R ChinaPeoples Hosp Suzhou New Dist, Dept Neurol, Suzhou, Jiangsu, Peoples R China
Ma, Linqing
Zhou, Lihua
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Peoples Hosp Suzhou New Dist, Dept Neurol, Suzhou, Jiangsu, Peoples R ChinaPeoples Hosp Suzhou New Dist, Dept Neurol, Suzhou, Jiangsu, Peoples R China
Zhou, Lihua
Sun, Lu
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Peoples Hosp Suzhou New Dist, Dept Neurol, Suzhou, Jiangsu, Peoples R ChinaPeoples Hosp Suzhou New Dist, Dept Neurol, Suzhou, Jiangsu, Peoples R China
Sun, Lu
Han, Chunru
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Peoples Hosp Suzhou New Dist, Dept Neurol, Suzhou, Jiangsu, Peoples R ChinaPeoples Hosp Suzhou New Dist, Dept Neurol, Suzhou, Jiangsu, Peoples R China
Han, Chunru
Fang, Qi
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Soochow Univ, Affiliated Hosp 1, Dept Neurol, Suzhou, Jiangsu, Peoples R ChinaPeoples Hosp Suzhou New Dist, Dept Neurol, Suzhou, Jiangsu, Peoples R China