以多组脑神经受损为首发症状的吉兰-巴雷综合征临床特点

Features of Guillain-Barré Syndrome with Complaint of Multiple Cranial Nerves Impairments:10 Cases Report

目的分析以多组脑神经受损为首发症状的吉兰-巴雷综合征(GBS)的临床特点。方法回顾分析10例以多组脑神经受损为首发症状的GBS患者的临床表现、电生理检测与CSF变化特点及治疗效果。结果本组病例均以脑神经Ⅶ、Ⅸ、Ⅹ受损为首发症状,男性患者为女性的4倍,发病年龄以18～55岁年龄段为主(8/10例),感染的前驱症状少见,膝腱及跟腱反射均减弱或消失,4例患者存在明显的脊神经根刺激症状。病程中有4例患者行呼吸机辅助呼吸。所有患者的脑脊液均在发病3周内出现蛋白-细胞分离现象,运动神经传导速度、F波异常率分别为81.25%及94.44%。与治疗前Hughes评分(4.00±0.82)相比,采用静脉注射免疫球蛋白(IVIG)治疗2周后Hughes评分为(2.25±0.96)(P=0.012),治疗后4周为(0.50±1.00)(P=0.000)。结论对于不明原因的多组脑神经受损,特别是累及双侧脑神经Ⅶ、Ⅸ、Ⅹ的患者,应考虑GBS的可能;早期脑脊液及电生理检查有助于明确诊断;IVIG可作为治疗以多组脑神经受损为首发症状的GBS的首选治疗方法。

Objective To investigate the clinical features of patients with multi-cranial nerves impairments as the onset of Gull lain Barr6 syndrome （GBS）. Methods 10 patients of GBS with complaint of multiple cranial neuropathy were analyzed retrospectively. Results The cranial nerves Ⅶ , Ⅸ and X were involved at the onset of GBS, tending to affect men rather than women （4：1）, aged of 18-55 years old （8/10）, and with less the antecedent of infection. The knee and ankle jerk reflexes were minimal or absent in all the patients, and the meningeal irritation signs were observed in 4 patients. Assisted ventilation was required in 4 patients during the course of their illness. The eerebrospinal fluid （CSF） characterized with increased protein concentration but a normal cell count in 2 patients in the first week, and all the patients in the following 3 weeks. The incidence of motor conduction velocity （MCV） and F waves abnormalities of electrophysiological evaluation were 81.25% and 94.44%% respectively. The Hughes scales were （4.00±0.82） before treatment, and were （2.25±0.96, P=0.012） and （0.50±1.00, P=0. 000） 14 d and 28 d after intrave nous immunoglobulin （IVIG）. Conclusion The probability of GBS should be considered in patients with multiple cranial neuropathy, especially the cranial nerves Ⅶ, Ⅸ and X impairments without precise causes. The early electrophysiological studies and CSF exam inations may be useful for diagnosis. IVIG can be preferred as an effective treatment.