河南省抗GQ1b抗体综合征患者临床特点分析

Clinical characteristics of patients with anti-GQ1b antibody syndrome in Henan Province

目的:分析河南省抗GQ1b抗体综合征患者的临床特点。方法:对815例临床怀疑周围神经病患者的血清进行抗神经节苷脂抗体检测,分析确诊为抗GQ1b抗体综合征的22例患者的临床特点。将22例患者按血清是否单一抗GQ1b抗体阳性分为2组,分析两组间临床表现不同点;将患者按应用一线免疫药物治疗(17例)和临床对症治疗(5例)分为2组,比较2组疗效的差异。结果:22例患者中男10例,女12例,有前驱感染病史14例,主要临床表现为四肢无力、腱反射减弱或消失、球麻痹;脑脊液蛋白-细胞分离9例。血清仅抗GQ1b抗体1项阳性者4例,均无意识障碍发生;多抗体阳性18例,其中伴有意识障碍发生的有5例。一线免疫治疗较临床对症治疗疗效更好(P=0. 018)。4例患者经一线免疫治疗8～12 d后血清抗GQ1b抗体转阴性。结论:抗GQ1b抗体综合征临床症状复杂,其中意识障碍的发生可能有多种抗体共同参与,根据临床特点、脑脊液检查和血清GQ1b抗体可明确诊断;给予人免疫球蛋白或激素治疗预后良好。

Aim: To analyze the clinical features of patients with anti-GQ1 b antibody syndrome in Henan Province.Methods: The anti-ganglioside antibody was detected in 815 patients with clinically suspected peripheral neuropathy,and the clinical features of 22 patients diagnosed as anti-GQ1 b antibody syndrome were analyzed. The 22 patients were allocated into two groups based on whether their serum was positive for the single anti-GQ1 b antibody,and the clinical manifestations were analyzed. The 22 patients were allocated into two groups according to the application of first-line immunotherapy( 17 cases) and clinical symptomatic treatment( 5 cases),and the non-parametric Kruskal-Wallis test was used to analyze the difference treatment effects. Results: Among the 22 patients,10 were male and 12 were female. There were 14 cases of pre-infection history. The main clinical manifestations were limb weakness,decreased or absent tendon reflex,and bulbar paralysis. Furthermore,cerebrospinal fluid protein-cell separation was observed in 9 cases. Only 4 patients were positive for single anti-GQ1 b antibody,all of whom had not conscious disorder. Additionally,18 patients were positive for multiple antibodies,of whom 5 showed disturbance of consciousness. First-line immunotherapy was more effective than clinical symptomatic treatment,and the difference was statistically significant( P = 0. 018). Four patients were negative for serum anti-GQ1 b antibody after 8 ~ 12 days of first-line immunotherapy. Conclusion: The clinical symptoms of anti-GQ1 b antibody syndrome are complex. Among them,disturbance of consciousness may be caused by multiple antibodies. The diagnosis can be confirmed by clinical features,cerebrospinal fluid examination,and serum GQ1 b antibody. The prognosis of patients with human immunoglobulin or hormone therapy is good.