45例抗GQ1b抗体阳性患者临床疾病谱

Clinical spectrum of 45 patients with positive anti-GQ1b antibodies

目的:分析具有不同临床特征的抗GQ1b抗体阳性患者的临床疾病谱。方法:回顾性分析45例抗GQ1b抗体阳性患者的临床症状、体征、辅助检查、治疗及预后等临床资料。结果:45例(男24,女21)抗GQ1b抗体阳性患者中,抗GQ1b抗体综合征32例,抗GQ1b抗体阳性吉兰巴雷综合征(GBS)10例,抗GQ1b抗体阳性慢性炎症性脱髓鞘性多发性神经病(CIDP)3例。平均年龄(49.8±11.5)岁。血清抗GQ1b-IgG阳性40例,抗GQ1b-IgM阳性5例,神经节苷脂抗体重叠阳性19例。抗GQ1b抗体综合征分为:经典型Miller-Fisher综合征(MFS)12例(43.8%),Bickerstaff脑干脑炎(BBE)3例(9.4%),伴有眼肌麻痹的GBS 5例(15.6%),不完全形式的MFS 12例[(43.8%,包括无共济失调的急性眼轻瘫(AO)8例,急性瞳孔扩大2例,急性球麻痹1例,急性前庭综合征1例)]。10例抗GQ1b抗体阳性GBS与抗体阴性的GBS在临床经过及症状体征方面比较,差异无统计学意义(P均>0.05),3例抗GQ1b抗体阳性CIDP中,抗GQ1b-IgM阳性2例,临床表现为经典CIDP。结论:抗GQ1b抗体综合征是一组血清抗GQ1b抗体阳性、临床表现多样的自身免疫性疾病连续谱。

Objective:To investigate the clinical spectrum of 45 patients with positive serum anti-GQ1b antibodies.Methods:The clinical data including the clinical symptoms,physical examination signs,adjuvant examinations,treatment,and prognosis of 45 patients with positive anti-GQ1b antibodies were retrospectively analyzed.Results:Of the 45 patients with positive serum anti-GQ1b antibodies,there were 32 cases of anti-GQ1b antibodies syndrome,10 cases of Guillain-Barre syndrome(GBS)and 3 cases of chronic inflammatory demyelinating polyradiculoneuropathy(CIDP).A total of 24 cases were male and 21 cases were female.The average age was(49.78±11.49)years old.The IgG-anti-GQ1b antibody in serum was positive in 40 patients,while the IgM-anti-GQ1b antibody was positive in 5 patients.A total of 19 of 45 patients were positive for other anti-ganglioside antibodies at the same time.The anti-GQ1b antibodies syndrome was comprised of 12(43.8%)of classical Miller Fisher syndrome(MFS),3(9.4%)of Bickerstaff brainstem encephalitis(BBE),5(15.6%)of GBS with ophthalmoplegia,and 12(43.8%)of incomplete forms of MFS which including 8 cases of acute ophthalmoparesis(AO),2 cases of acute mydriasis,1 case of acute oropharyngeal palsy,1 case of acute vestibular syndrome.There was no statistically significant difference between the 10 cases of positive anti-GQ1b antibody GBS and negative-antibody GBS in clinical course and characteristics.A total of 3 cases of positive anti-GQ1b antibody CIDP clinically presented as classical CIDP.Conclusions:The anti-GQ1b antibodies syndrome is a serial of clinical disease spectrum,characterizing as positive serum anti-GQ1b antibodies and diverse clinical symptom involving with peripheral and central nerve systems respectively or simultaneously.