Bickerstaff brainstem encephalitis and Fisher syndrome: anti-GQ1b antibody syndrome

Shahrizaila, Nortina; Yuki, Nobuhiro

doi:10.1136/jnnp-2012-302824

Cited by 233 publications

(220 citation statements)

References 72 publications

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“…The association between specific anti-ganglioside antibodies and specific GBS subtypes became accepted, but the proportion of patients who were seropositive for these antibodies was generally relatively low and of limited diagnostic use. By contrast, in 1992 Chiba and colleagues detected anti-GQ1b IgG antibodies in six consecutive patients with MFS, and consequently proposed that it could be a useful diagnostic marker 58 .This report opened the door to a plethora of studies that confirmed that the presence of anti-GQ1b IgG antibodies in 80-95% of patients with MFS and in many patients with MFS-GBS overlap conditions or Bickerstaff encephalitis 17 . Anti-GQ1b IgG antibodies have, therefore, served as a useful clinical diagnostic marker of MFS.…”

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confidence: 56%

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Guillain–Barré syndrome: a century of progress

2016

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In 1916, Guillain, Barré and Strohl reported on two cases of acute flaccid paralysis with high cerebrospinal fluid protein levels and normal cell counts -novel findings that identified the disease we now know as Guillain-Barré syndrome (GBS).100 years on, we have made great progress with the clinical and pathological characterization of GBS. Early clinicopathological and animal studies indicated that GBS was an immune-mediated demyelinating disorder with secondary axonal injury if severe; the current treatments of plasma exchange and intravenous immunoglobulin, which were developed in the 1980s, are based on this premise. Subsequent work has, however, shown that the underlying disease can be an axonal injury. The association of Campylobacter jejuni strains has led to confirmation that anti-ganglioside antibodies are pathogenic and that axonal GBS involves an antibody and complement-mediated disruption of nodes of Ranvier, neuromuscular junctions and other neuronal and glial membranes. Now, ongoing clinical trials of the complement inhibitor eculizumab are the first of targeted immunotherapy in GBS.Guillain-Barré syndrome (GBS) is the commonest cause of acute flaccid paralysis and manifests as rapidly evolving weakness and sensory disturbance in arms, legs and in some patients, facial, bulbar and respiratory muscles. Many patients will make a good recovery over many months but in severe cases patients can require months of intensive care support and be left with permanent severe weakness, sensory disturbance and pain. Furthermore, around 5% die from complications including respiratory failure, pneumonia and arrhythmias, making it a medical emergency with a high morbidity and significant mortality.Descriptions of clinical cases that closely resemble the condition we now know as GBS were made at least as early as 1859, when Jean Baptiste Octave Landry reported on "acute ascending paralysis". His report led to use of the term "Landry's ascending paralysis" to describe subacute ascending peripheral sensory and motor dysfunction. He observed that:

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confidence: 56%

“…He identified signs that the CNS could be involved, which ultimately led to the realization that Miller Fisher syndrome (MFS), Bickerstaff brainstem encephalitis and GBS represent different points on the same immunopathological spectrum 17 .…”

Section: [H1] Early Pathological and Animal Studiesmentioning

confidence: 99%

Guillain–Barré syndrome: a century of progress

2016

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“…Given the clinicopathological continuum of GBS, MFS and its variants, including BBE,24 mild CNS involvement in the aforementioned patients can not be fully excluded, although, there was no supportive clinical, electrophysiologic, or radiographic evidence for brainstem involvement. The clinical variations of distinct GBS and MFS subtypes might be related to the locoregional distribution of different ganglioside species 22, 31 in glial and neuronal membranes of PNS and CNS accessible to circulating anti‐ganglioside antibodies that provoke a complex inflammatory response 32. Although topospecific mapping of brain gangliosides is challenging, as demonstrated in animal models, a spatial distribution could be detected demonstrating a predominant expression of certain ganglioside species in the brainstem and periaqueductal grey area (GD1, GT1, GT3, GQ1) 33.…”

Section: Discussionmentioning

confidence: 99%

Area postrema syndrome as frequent feature of Bickerstaff brainstem encephalitis

Zeiner

Brandhofe

Müller–Eschner

et al. 2018

Ann Clin Transl Neurol

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ObjectiveArea postrema (AP) syndrome (defined as: nausea and/or emesis and/or singultus at onset of brainstem dysfunction) comprises complex pathophysiologic mechanisms triggered by different entities. The first objective was to assess the frequency of AP syndrome as a clinical feature in brainstem encephalitis (BE). Finding an especially high prevalence of AP syndrome in Bickerstaff brainstem encephalitis (BBE), we also analyzed the frequency of AP syndrome in other autoimmune diseases with anti‐ganglioside antibodies (Guillain–Barré syndrome (GBS) and its variants).MethodsWe systematically evaluated the prevalence of AP syndrome in BE in all patients treated at our university hospital during a 15‐year period. In a second step, BBE patients were compared to GBS and Miller Fisher syndrome (MFS) patients as clinical subtypes of a disease continuum without brainstem dysfunction.ResultsWe found AP syndrome in 8 of 21 BE patients, including 3 of 7 BBE and in 4 of 112 GBS/MFS patients. AP syndrome was as a frequent but under‐recognized feature of BE with a significant impact on patients’ well being.InterpretationManifestation of AP syndrome in BBE but also in GBS and its subtypes point toward a role of autoimmune antibodies that should be investigated in future studies. Considerable misdiagnosis or nonrecognition complicates diagnostic and therapeutic management. Therefore, AP syndrome should be considered in any episode of otherwise unexplained nausea, emesis, or singultus.

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“…De igual manera, estas moléculas comparten una gran similitud con los gangliósidos presentes en los axones tanto a nivel periférico como en el tallo cerebral, y que servirán de blanco para los anticuerpos de tipo IgG presentes hasta en 66 % de los pacientes (2,7,10,11).…”

Section: Fisiopatologíaunclassified

“…Otros hallazgos clínicos descritos fueron debilidad facial, blefaroptosis, nistagmo, parálisis bulbar y signo de Babinski (2,3,8). De acuerdo con la instauración de la sintomatología, es importante descartar otras entidades que pueden comportarse de manera similar.…”

Section: Manifestaciones Clínicasunclassified