神经节苷脂相关吉兰巴雷综合征病例的回顾性分析

Ganglioside-associated Guillain-Barre Syndrome: Reports of 5 Adverse Reactions and Literature Review

目的:了解单唾液酸四己糖神经节苷脂(GM1)相关吉兰巴雷综合征的发生与特点,为临床安全用药提供参考。方法:收集某院静注GM1注射液致吉兰巴雷综合征病例,同时检索中文期刊数据库中GM1相关吉兰巴雷综合征的个案报道,合并文献病例与院内病例,回顾性统计分析患者性别、年龄、原患疾病、基础疾病、前驱感染史、用药剂量及疗程、合并用药、不良反应起始症状、主要表现、双侧腱反射减弱或消失情况、发生时间、疾病平均达峰时间、实验室检查、严重程度、治疗和转归等项目。结果:收集院内病例5例,文献检索获得病例17例,合计入选病例22例,其中男21例,女1例,50~60岁患者占50.00%。GM1相关吉兰巴雷综合征多发生于脑出血、脑梗死及脑外伤患者;31.82%的患者有自身免疫相关性疾病。GM1的剂量、疗程与不良反应严重程度无明确相关性。GM1相关吉兰巴雷综合征多以肌力及肌张力下降起病,所有病例均以四肢无力为主要表现,且大多合并颅神经受累、意识障碍、自主神经功能障碍、感觉及呼吸功能异常。不良反应多在8~14 d出现,1~3 d达峰;所致肢体瘫痪程度较重,临床结局较差。结论:GM1可能导致吉兰巴雷综合征,临床应用时应严格把握用药指征,用药时加强用药监护,确诊吉兰巴雷综合征后应及时停药并应用静注人免疫球蛋白,避免不良反应的进一步加重。

Objective: To understand the characteristies of monosialotetrahexosyl ganglioside( GM1 )-related Guillain- Barre syndrome ( GBS),and provide reference for clinical safe drug usage. Methods : Five cases of GBS caused by intravenous injection of CM1 were reported in our hospital. The case reports of CBS related to ganglioside in domestie academic journals from January 2002 to December 2018 were retrieved. The literature cases were screened and merged with inhospital cases. Cender, age, original disease, underlying disease, history of precursor infection, dosage and course of treatment, combined use of drugs, initial symptoms of adverse reactions,main manifestations , weakening or disappearance of bilateral tendon relex, vecurrenee time of adverse reactions, average peak time of disease ,laboratory exarmination, severity, etc. Data of treatment and outcome were collected and analyzed. Results:A total of 22 cases were enurolled, 21 males and I females, 50. 00% of them were 50-60 years old. GM l-as8ociated GBS occured mostly in patients with cerebral hermorrhage, cerebral infarction und traumnatie brain injury. 31. 82% of the paticnts had auloimmunc- related discascs. There was no clear correlation between the dosage, course of treatment and the severity of adverse reactions of gangliosides. GM 1-associated GBS mostly starls with decreased muscle strength and muscle tone. All cases were characterized by limb weakness, and most of them were accompanied by cranial nerve involvement, conscious disturbance, autonomic nervous dysfunction, sensory and respiratory dysfunction. Adverse reactions mostly occurred in 8-14 days and peaked in 1-3 days. The degree of limb paralysis was serious and the clinical outcome was poor. Conclusion :CM1 may lead to CBS. In clinical application, we should strictly grasp the indications of medication, strengthen medication monitoring. stop medieation in time und use intravenous human imnnunoglobulin uller diugnosis of CBS, so us lo avoid the occurrence o[ adverse reactions and further aggravation.