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比克法斯特脑干脑炎和费舍尔综合征:抗 GQ1b 抗体综合征。

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

机构信息

Division of Neurology, Department of Medicine, Faculty of Medicine, University of Malaya, Malaysia.

出版信息

J Neurol Neurosurg Psychiatry. 2013 May;84(5):576-83. doi: 10.1136/jnnp-2012-302824. Epub 2012 Sep 15.

DOI:10.1136/jnnp-2012-302824
PMID:22984203
Abstract

In the 1950s, Bickerstaff and Fisher independently described cases with a unique presentation of ophthalmoplegia and ataxia. The neurological features were typically preceded by an antecedent infection and the majority of patients made a spontaneous recovery. In the cases with Bickerstaff brainstem encephalitis, there was associated altered consciousness and in some, hyperreflexia, in support of a central pathology whereas in Fisher syndrome, patients were areflexic in keeping with a peripheral aetiology. However, both authors recognised certain similarities to Guillain-Barré syndrome such as the presence of peripheral neuropathy and cerebrospinal fluid albuminocytological dissociation. The discovery of immunoglobulin G anti-GQ1b antibodies in patients with Fisher syndrome and later in Bickerstaff brainstem encephalitis was crucial in providing the necessary evidence to conclude that both conditions were in fact part of the same spectrum of disease by virtue of their common clinical and immunological profiles. Following this, other neurological presentations that share anti-GQ1b antibodies emerged in the literature. These include acute ophthalmoparesis and acute ataxic neuropathy, which represent the less extensive spectrum of the disease whereas pharyngeal-cervical-brachial weakness and Fisher syndrome overlap with Guillain-Barré syndrome represent the more extensive end of the spectrum. The conditions can be referred to as the 'anti-GQ1b antibody syndrome'. In this review, we look back at the historical descriptions and describe how our understanding of Fisher syndrome and Bickerstaff brainstem encephalitis has evolved from their initial descriptions more than half a century ago.

摘要

在 20 世纪 50 年代,Bickerstaff 和 Fisher 分别独立描述了一组具有独特眼肌瘫痪和共济失调表现的病例。这些神经学特征通常先于前驱感染,大多数患者可自发恢复。在 Bickerstaff 脑干脑炎病例中,存在意识改变,部分患者存在反射亢进,支持中枢性病变;而在 Fisher 综合征中,患者反射消失,符合周围性病因。然而,两位作者都认识到某些与吉兰-巴雷综合征相似的特征,如周围神经病和脑脊液白蛋白细胞分离。在 Fisher 综合征和后来的 Bickerstaff 脑干脑炎患者中发现免疫球蛋白 G 抗-GQ1b 抗体,为这两种情况实际上属于同一疾病谱提供了必要的证据,这是基于它们共同的临床和免疫学特征。此后,文献中出现了其他具有抗-GQ1b 抗体的神经表现。这些包括急性眼肌瘫痪和急性共济失调性神经病,代表了疾病的较轻范围,而咽颈臂无力和 Fisher 综合征与吉兰-巴雷综合征重叠,代表了疾病谱的更广泛范围。这些情况可以被称为“抗-GQ1b 抗体综合征”。在这篇综述中,我们回顾了历史描述,并描述了我们对半世纪多前对 Fisher 综合征和 Bickerstaff 脑干脑炎的最初描述的理解是如何发展的。

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