Acute bulbar palsy as a variant of Guillain-Barre syndrome
Acute bulbar palsy as a variant of Guillain-Barre syndrome
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DOI：
10.1212/wnl.0000000000002256
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发表时间：
2016-02-23
期刊：
影响因子：
9.9
通讯作者：
Bae, Jong Seok
中科院分区：
文献类型：
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作者：
Kim, Jong Kuk;Kim, Byung-Jo;Bae, Jong Seok
目的：将一种表现为突出的急性延髓麻痹（ABP）而无肢体运动无力的综合征归类为格林-巴利综合征（GBS）的变异型，并与米勒费舍尔综合征（MFS）和咽-颈-肱（PCB）变异型相鉴别。方法：我们分析了11例ABP而无肢体运动无力的病例，这些病例基于包含临床信息的数据集和急性免疫介导的神经病的抗神经节苷脂抗体测定结果。所有入选病例均以ABP为主要症状，无肢体无力。以下特征伴随ABP，按频率降序排列：眼肌麻痹（n = 9，82%）、共济失调（n = 9，82%）和面神经麻痹（n = 6，55%）。酶联免疫吸附试验的研究发现，免疫球蛋白G（IgG）抗GT 1a抗体是最常见的（n = 11），其次是IgG抗GQ 1b抗体（n = 6）。结论：我们建议，ABP-plus综合征没有颈部或四肢无力是GBS的变异，是不同于MFS和PCB的变异。IgG抗GT 1a抗体的存在可以解释不同的临床特征和潜在的病理机制之间的关系。
Objective:To categorize a syndrome manifesting as prominent acute bulbar palsy (ABP) without limb motor weakness as a variant form of Guillain-Barre syndrome (GBS) and differentiate it from Miller Fisher syndrome (MFS) and pharyngeal-cervical-brachial (PCB) variants.Methods:We analyzed cases of ABP without limb motor weakness based on a dataset containing clinical information and the results of antiganglioside antibodies assays for acute immune-mediated neuropathies.Results:Eleven cases with an age at onset ranging from 18 to 65 years (mean 33.8 years) were identified as ABP-plus syndrome. All of the enrolled cases manifested with ABP as the predominant symptom, and with no limb weakness. The following features accompanied ABP in order of decreasing frequency: ophthalmoplegia (n = 9, 82%), ataxia (n = 9, 82%), and facial palsy (n = 6, 55%). An enzyme-linked immunosorbent assay study disclosed that immunoglobulin G (IgG) anti-GT1a antibodies were the most frequent (n = 11), followed by IgG anti-GQ1b antibodies (n = 6).Conclusions:We propose that ABP-plus syndrome without neck or limb weakness is a variant of GBS that is distinct from the MFS and PCB variants. The presence of IgG anti-GT1a antibodies can explain the relationships between the distinct clinical characteristics and the underlying pathomechanisms.