作者：张梦歌,蒋玙姝,马伟锋,张涛,贾亚珍,周珂珂,贾琳琳,秦灵芝,李玮

单位：

1.450003 河南省郑州市，郑州大学人民医院 河南省人民医院神经内科 2.453000 河南省新乡市，新乡医学院 通信作者：李玮，E-mail：liwei71@126.com

Units：

1.Department of Neurology, Zhengzhou University People's Hospital/Henan Provincial People's Hospital, Zhengzhou 450003,China 2.Xinxiang Medical University, Xinxiang 453000, China Corresponding author: LI Wei, E-mail: liwei71@126.com

关键词：

吉兰-巴雷综合征; 截瘫型; 临床特征;

Keywords：

Guillain-Barré syndrome; Paraparetic; Clinical characteristics

CLC：

DOI：

10.12114/j.issn.1008-5971.2021.00.238

Funds：

河南省医学科技攻关计划项目（SBGJ2018077）

摘要：

背景吉兰-巴雷综合征(GBS)是急性神经肌肉麻痹的常见病因,该疾病谱系包括经典型GBS和变异型GBS。截瘫型GBS属于变异型GBS,其在临床上罕见报道。目的 分析10例截瘫型GBS的临床特征,以期提高临床医生对截瘫型GBS的认识。方法 选取2018年10月至2021年3月河南省人民医院收治的240例GBS患者,其中符合截瘫型GBS诊断的患者10例,占4.2%。回顾性分析10例截瘫型GBS患者的临床资料,包括人口学特征、临床症状、影像学检查结果、实验室检查指标、神经电生理检查结果、治疗及预后情况。结果 10例截瘫型GBS患者中男4例,女6例;年龄21～83岁,平均(46.1±7.5)岁;5例患者无前驱症状,3例患者前驱症状为上呼吸道感染,2例患者前驱症状为腹泻;10例患者首发症状均为双下肢无力/麻木;6例患者伴有感觉异常,主要为双下肢麻木和/或疼痛;仅2例患者出现颅神经或自主神经受损表现;9例患者存在腱反射减弱或消失。10例截瘫型GBS患者中6例患者脑脊液蛋白-细胞分离阳性;仅2例患者血清抗神经节苷脂抗体阳性;10例患者均伴有不同程度双下肢周围神经传导异常,但双上肢神经传导均正常;Hadden电生理分型:急性运动轴索型神经病(AMAN)3例、急性炎性脱髓鞘型多发性神经病(AIDP)3例、急性运动感觉轴索型神经病(AMSAN)型1例、无法分型3例。10例截瘫型GBS患者均接受静脉注射人免疫球蛋白(IVIG)及B族维生素治疗,治疗6个月后门诊或电话随访,所有患者恢复日常生活能力,随访期间疾病未进展、无复发。结论 本组GBS患者中截瘫型GBS占4.2%。截瘫型GBS多见于女性,多无前驱症状,主要首发表现为无上肢受累的双下肢无力,常伴有感觉异常及腱反射减弱或消失,患者首选IVIG治疗,且预后较好。

Abstract：

【Abstract】　Background Guillain-Barré syndrome (GBS) is a common cause of acute neuromuscular paralysis, andits disease spectrum includes classical GBS and variant GBS. Paraparetic GBS was a variant GBS, there are few reports about itin the clinic. Objective To analyze the clinical characteristics of 10 cases of paraparetic GBS, in order to improve clinicians'understanding of paraplegic GBS. Methods A total of 240 patients with GBS treated in Henan Provincial People's Hospital fromOctober 2018 to March 2021 were selected, including 10 patients (4.2%) who met the diagnosis of paraplegic GBS. The clinicaldata of 10 patients with paraplegic GBS were analyzed retrospectively, including demographic characteristics, clinical symptoms,imaging examination results, laboratory examination indexes, neuroelectrophysiological examination results, treatment andprognosis. Results There were 4 males and 6 females in 10 patients with paraplegic GBS; the age ranged from 21 to 83 years old,with an average of (46.1±7.5) years old; 5 patients had no prodromal symptoms, the prodromal symptom of 3 patients was upperrespiratory tract infection and the prodromal symptom of 2 patients was diarrhea; the first symptoms of 10 patients with paraplegicGBS were weakness/numbness of both lower limbs; 6 patients were accompanied by paresthesia, mainly numbness and/or painof both lower limbs; only 2 patients had cranial nerve or autonomic nerve damage; and 9 patients had weakened or disappearedtendon reflex. Six of 10 patients with paraplegic GBS were positive for protein cell separation in cerebrospinal fluid; serum antiganglioside antibody was positive in only 2 patients; all 10 patients were accompanied by abnormal peripheral nerve conduction inboth lower limbs, but the nerve conduction in both upper limbs was normal; Hardden electrophysiological classification: 3 casesof acute motor axonal neuropathy (AMAN) , 3 cases of acute inflammatory demyelinating polyneuropathy (AIDP) , 1 case of acutemotor sensory axonal neuropathy (AMSAN) and 3 cases without classification. Ten patients with paraplegic GBS were treatedwith intravenous immunoglobulin (IVIG) and B vitamins. After 6 months of treatment, all patients recovered their ability of dailyliving. During the follow-up period, the disease did not progress or relapse. Conclusion Paraplegic GBS accounted for 4.2% ofGBS patients in this group. Paraplegic GBS is more common in women, most patients have no prodromal symptoms, its main firstmanifestation is weakness of both lower limbs without upper limb involvement, often accompanied by paresthesia and weakeningor disappearance of tendon reflex. IVIG is the first choice for patients with paraplegic GBS, and the prognosis is good.

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