Continuous spectrum of pharyngeal-cervical-brachial variant of Guillain-Barre syndrome
Continuous spectrum of pharyngeal-cervical-brachial variant of Guillain-Barre syndrome
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DOI：
10.1001/archneur.64.10.1519
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发表时间：
2007-10-01
影响因子：
--
通讯作者：
Yuki, Nobuhiro
中科院分区：
文献类型：
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作者：
Nagashima, Takahide;Koga, Michiaki;Yuki, Nobuhiro
背景：咽部-颈部-肱肌无力（PCB）被认为是格林-巴利综合征（GBS）的一种变体。由于其罕见性，没有对多氯联苯患者的大量研究。目的：明确多氯联苯的分类学。设计：回顾性研究。设置：学术研究。患者：回顾了首次发病4周内表现为咽部、颈部和上肢进行性无力的患者的医疗记录。主要观察指标：分析临床特征，调查既往感染和抗神经节苷脂抗体。结果：100例患者诊断为“纯PCB”（13例）、PCB伴肌肉拉伸反射保留（8例）、GBS重叠（48例）、Fisher综合征重叠（26例）、Bickerstaff脑干脑炎重叠（5例）。血清学检测结果显示，31.0%的PCB感染是由空肠弯曲杆菌引起的。抗神经节苷脂抗体检测中，51.0%的患者抗gt1a IgG抗体阳性。抗gq1b IgG抗体（Fisher综合征和比克斯塔夫脑干脑炎的血清学标志物）阳性占39.0%。GM1、GM1b、GD1a或GalNAc-GD1a（轴突GBS亚型血清学标志物）IgG抗体阳性的比例为27.0%。结论：这项大型研究确定了PCB的临床特征。PCB、GBS、Fisher综合征和Bickerstaff脑干脑炎患者的临床重叠、频繁的C型空肠感染和常见的抗神经节苷脂抗体提供了确凿的证据，证明PCB与这些疾病形成了一个连续的谱。
Background: Pharyngeal-cervical-brachial weakness (PCB) is considered a variant of Guillain-Barre syndrome (GBS). Because of its rarity, there have been no studies of large numbers of patients with PCB.Objective: To clarify the nosological classification of PCB.Design: Retrospective study.Setting: Academic research.Patients: Medical records were reviewed of patients who manifested progressive weakness of the pharynx, neck, and upper limbs within 4 weeks of initial onset.Main Outcome Measures: Clinical features were analyzed, and antecedent infections and antiganglioside antibodies were investigated.Results: Diagnoses for 100 patients were "pure PCB" (n=13), PCB with preserved muscle stretch reflexes (n=8), GBS overlap (n=48), Fisher syndrome overlap (n=26), and Bickerstaff brainstem encephalitis overlap (n=5). Serological test results showed that 31.0% of antecedent infections in PCB were caused by Campylobacter jejuni. Of the antiganglioside antibodies tested, anti-GT1a IgG antibodies were positive in 51.0% of the patients. Anti-GQ1b IgG antibodies (a serological marker of Fisher syndrome and Bickerstaff brainstem encephalitis) were positive in 39.0%. The IgG antibodies to GM1, GM1b, GD1a, or GalNAc-GD1a ( serological markers of an axonal GBS subtype) were positive in 27.0%.Conclusion: This large study identified the clinical profiles of PCB. Clinical overlapping, frequent C jejuni infection, and common antiganglioside antibodies present in PCB, GBS, Fisher syndrome, and Bickerstaff brainstem encephalitis provide conclusive evidence that PCB and these conditions form a continuous spectrum.