重症肌无力合并吉兰-巴雷综合征临床特点分析研究

目的:探讨重症肌无力(MG)合并吉兰巴雷综合征(GBS)疾病的发生机制、特征性临床表现、诊断策略、治疗及预后。方法:报道我院2例确诊为MG合并GBS临床病例,并结合文献对国内外共21例MG合并GBS病例资料进行系统性回顾分析。结果:共得到国内外MG合并GBS患者21例,男16例,女5例,年龄17~84岁,平均(44.67±17.11)岁。多数患者起病前有呼吸道或消化道感染诱因;2种疾病起病时间最长间隔可达23年。大部分患者具有典型眼外肌无力及迟缓性四肢瘫痪表现,此外一些共同临床特征还包括吞咽困难、构音障碍、呼吸困难、面肌瘫痪等。实验室检查提示,所有行肌电图检查患者神经传导及低频重复电刺激实验结果均异常,19例患者脑脊液提示蛋白细胞分离,15例患者测定乙酰胆碱抗体阳性,未测定乙酰胆碱抗体的5例患者新斯的明试验均为阳性。经临床治疗后,21例患者中(19例记录)14例患者预后较好(Hughes≤2)。结论:MG合并GBS多见于青年男性,起病前有感染等诱因,临床诊断主要依据典型MG及GBS临床表现、乙酰胆碱抗体测定、脑脊液分析结果及神经电生理异常,治疗上应采用吡斯的明、类固醇及丙种球蛋白等联合用药,早期识别及正确诊断对预后至关重要。

Clinical Characteristics of Myasthenia Gravis Patients with Guillain-Barre'Syndrome

Objective:To investigate the possible mechanisms,clinical characteristics,diagnosis,treatment and prognosis of patients with myasthenia gravis (MG) and Guillain-Barre'syndrome (GBS) for an insight into this rare clinical entity.Methods:We reported 2 cases at our hospital who were diagnosed to have both MG and GBS and then collected the data of other 19 cases from literature.All the clinical data of all the 21 cases were analyzed.Results:Of all the cases,5 were female and 16 were male,aged from 17 to 84 years old,mean (44.67± 17.11) years.Majority of the patients had predisposing factors such as upper respiratory infection or acute gastroenteritis before the onset.The longest interval of the onset of the two diseases was 23 years.Most of the patients showed symptoms of classic extraocular muscle weakness and areflexic flaccid tetraplegia,and other common presentations were dysphagia,dysarthria,dyspnea and facial palsy.NCV and RNS were detected in all the tested cases.Data of CSF examination showed increased spinal fluid protein without pleocytosis in 19 patients.AChR antibody was positive in all tested patients and the neostigmine test was positive in 5 patients without detection of AChR antibody.Functional outcome,ranked according to the adopted scale by Hughes,was mentioned in 19 patients and seven had good outcomes (Hughes scale ＜ 2) after treatment.Conclusion:Our results suggest that the MG associated with GBS is adult male predominance.The diagnosis of this syndrome is mainly based on classical clinical manifestations of MG and GBS,positive AChR antibody,CSF examination and neuroelectrophysiologic abnormalities.The valid treatment is a combination of the drugs including pyridostigmine,corticosteroids,IVIG,plasmaphoresis,and/or azathioprinum.With early and correct diagnosis,and timely application of immunotherapy,favorable outcomes could be achieved.