Saul Robert F
Department of Neurology, University of Mississippi Medical Center, 2500 North State Street, Jackson, MS 39216, USA.
Curr Neurol Neurosci Rep. 2009 Sep;9(5):379-83. doi: 10.1007/s11910-009-0055-0.
The discovery of the association of the anti-GQ1b IgG antibody with the postinfectious clinical syndromes of ophthalmoplegia, ataxia, and areflexia helped house the phenotypes of the Miller Fisher syndrome (MFS), atypical MFS, Guillain-Barré syndrome with ophthalmoplegia, and Bickerstaff's brainstem encephalitis under one roof. The neuro-ophthalmologic signs classically predominate and may vary from case to case, but they maintain clinically recognizable patterns that assist with the diagnosis. The identification of a common lipopolysaccharide on the plasma membrane in human cranial and peripheral nerves at the GQ1b epitope and on infectious particles of bacteria and viruses (ie, Campylobacter jejuni) demonstrates molecular mimicry. The high frequency of oculomotor dysfunction is partially explained by the tissue ganglioside concentration and distribution and the attraction of antibody-stimulating complement activation. Current experimental treatment targets antibody removal and neutralization and prevents membrane attack complex formation through deactivation of complement. This article aims to bring together the historically disparate opinions on the origins of these syndromes as either a purely peripheral nervous system or central nervous system dysfunction, highlight the clinical neuro-ophthalmologic signs, discuss some of the biology of the anti-GQ1b antibody, and review imaging abnormalities and treatment of this fascinating disorder.
抗GQ1b IgG抗体与感染后眼肌麻痹、共济失调和无反射临床综合征之间关联的发现，有助于将米勒费雪综合征（MFS）、非典型MFS、伴有眼肌麻痹的吉兰-巴雷综合征以及比克斯特法夫脑干脑炎的表型归为一类。经典的神经眼科体征占主导地位，且因病例而异，但它们保持着临床上可识别的模式，有助于诊断。在人颅神经和周围神经的质膜上，在GQ1b表位以及细菌和病毒（如空肠弯曲菌）的感染颗粒上鉴定出一种共同的脂多糖，这表明存在分子模拟现象。动眼功能障碍的高频率部分可通过组织神经节苷脂的浓度和分布以及抗体刺激补体激活的吸引力来解释。目前的实验性治疗靶点是去除和中和抗体，并通过使补体失活来防止膜攻击复合物的形成。本文旨在汇集关于这些综合征起源的历来不同观点，这些观点认为其要么是纯粹的外周神经系统功能障碍，要么是中枢神经系统功能障碍，突出临床神经眼科体征，讨论抗GQ1b抗体的一些生物学特性，并回顾这种迷人疾病的影像学异常和治疗方法。
