34例变异型吉兰-巴雷综合征的临床特点分析

A Study on the Clinical Features of 34 Cases of Variant Guillain-Barre Syndrome

目的分析变异型吉兰-巴雷综合征(GBS)的临床特点。方法回顾性分析2012年10月-2019年3月变异型GBS患者的临床表现、电生理及脑脊液特点。结果共34例患者,包括Miller-Fisher综合征(MFS)及MFS变异亚型共12例,脑神经变异型(CNV)12例,急性感觉神经病(ASN)1例,急性泛自主神经病(APN)1例,咽-颈-臂(PCB)2例以及不能明确分型6例。34例变异型GBS患者中男20例、女14例(P=0.392);发病年龄17~80岁,平均年龄(53.38±14.99)岁,中年(41~65岁)组所占比例最多(P=0.000);20例有前驱事件,上呼吸道感染占65%;首发症状以肢体麻木(38.2%)、吞咽困难(29.4%)、吐词不清(23.5%)多见;97.1%的患者发病4周内达高峰;需机械通气者5.9%;疾病开始恢复的中位时间13 d,住院中位时长13.5 d;64.7%的患者腱反射减弱或消失;在完成腰椎穿刺检查的患者中脑脊液蛋白-细胞分离者58.6%;发病到完善神经电生理检查的平均时间(10.70±7.32)d,85.2%神经电生理检查表现异常;50%患者给予静脉注射免疫球蛋白(IVIg)治疗,14.7%患者给予激素治疗,8.8%患者给予免疫球蛋白联合激素治疗,除外1例患者主动要求出院,其余治疗均有效。结论变异型GBS临床表现多样,常无典型急性四肢对称性迟缓性麻痹等症状,临床诊断需要综合判断,出现一些少见的临床表现并不能除外GBS的诊断,脑脊液和神经电生理检查可以帮助提高诊断,详尽的病史及神经系统查体尤为重要,免疫球蛋白和激素治疗有效。

Objective To analyze clinical features of variant Guillain-Barré syndrome(GBS).Methods The medical records of patients with variant GBS from October 2012 to March 2019 were retrospective analyzed, and the clinical manifestations, electrophysiology and cerebrospinal fluid characteristics were analyzed.Results A total of 34 patients, including 12 cases with Miller-Fisher Syndrome(MFS) and MFS variant subtypes, 12 cases with cranial nerve variants(CNV),1 case with acute sensory neuropathy(ASN), 1 case with acute panautonomic neuropathy(APN), 2 cases with pharyngeal-cervicai-brachia(PCB), and 6 cases with unclear classification. Among 34 patients with variant GBS, there were 20 males and 14 females(P=0.392), the age of onset was 17~80 years old, with an average age of(53.38±14.99) years, in which the number of the middle-aged group(41~65 years old) had the largest proportion(P=0.000). There were antecedent events in 20 cases, and upper respiratory tract infections accounted for 65%, first symptoms were more common with limb numbness(38.2%), difficulty swallowing(29.4%), and unclear words(23.5%). 97.1% of patients had peaks within 4 weeks of onset. Patients requiring mechanical ventilation accounted for 5.9%. The median time to start recovery of the disease was 13 days, and the median duration of hospitalization was 13.5 days, 64.7% of patients had reduced or disappeared tendon reflexes. Among the patients who completed the lumbar puncture examination, 58.6% of them showed cerebrospinal fluid protein-cell separation. The average time from onset to finish neurophysiological examination was(10.70±7.32) days, 85.2% of the results were abnormal, 50% of patients were treated with intravenous immunoglobulin(IVIg), 14.7% were given hormone therapy, 8.8% were treated with immunoglobulin and hormone, except for one who was asked to discharge the patient, and the remaining patients were treated effectively.Conclusion The clinical manifestations of variant GBS were diverse, and there were often no typical symptoms such as sympathetic slowness and weakness of the limbs. The clinical diagnosis required comprehensive judgment and the emergence of some rare clinical manifestations could not exclude the diagnosis of GBS, cerebrospinal fluid and neurophysiological examination could help improve the diagnosis, and the detailed history and nervous system physical examination were particularly important, the therapy of immunoglobulin and hormone was effective.