吉兰—巴雷综合征临床特征及电生理分型演变的研究

发布时间：2018-03-11 14:21

本文选题：吉兰-巴雷综合征　切入点：临床特征　出处：《第四军医大学》2015年硕士论文　论文类型：学位论文

【摘要】：研究背景:GBS(Guillain-Barrésyndrome,GBS)是一种自身免疫介导的周围神经疾病,以周围神经及神经根脱髓鞘改变和小血管炎性细胞浸润为主要特点,甚至可有轴索变性。急性炎性脱髓鞘性多发性神经病(Acute inflammatory demyelinating polyneuropathy,AIDP)为GBS中最经典的一型,近年来国内外学者根据GBS患者的电生理表现、临床表现及神经病理改变等特征将该疾病分为AIDP、急性运动轴索型神经病(Acute motor axonal neuropathy,AMAN)、急性运动感觉轴索性神经病(Acute motor-axonal neuropathy,AMSAN)等不同亚型。但并不是所有患者的电生理亚型都一成不变,部分患者的电生理表现随病程进展发生着改变。在GBS患者中有一部分病例伴随传导阻滞(conduction block,CB),传统观念认为脱髓鞘是导致CB发生的主要原因,但近些年有研究表明CB更可能是脱髓鞘、轴索膜的损伤及钠离子通道失活等多方面原因综合作用的结果[1]。近年来有临床及电生理分型变化及CB与预后转归方面的个案报道,提示早期电生理改变出现传导阻滞可能与GBS电生理分型变化和病情转归有关[2]。研究目的:通过回顾性研究分析GBS患者的一般资料、临床特征、实验室检查结果、电生理特点及治疗效果的差别;通过GBS患者的电生理随诊观察病程发展中电生理分型变化、早期电生理特征及其与病情严重程度和转归的相关性,早期识别预后不良gbs患者早期电生理的特点,为临床诊治提供参考和依据。研究方法:实验一:回顾性研究2008年至2013年初西京医院神经内科收治符合中华医学会吉兰-巴雷综合征诊疗指南中诊断标准的gbs患者临床及神经电生理资料共338例,其中20例未查神经电生理,最后纳入318例进行分析,其中24例诊断为gbs变异型miller-fisher综合征(miller-fishersyndrome,mfs)。对所收集病例资料进行归纳整理,建立gbs患者临床及电生理资料数据库,观察病历数据一般资料及临床特征;收集所有研究对象的电生理结果并进行分型,观察电生理一般特征及分型特点。实验二:回顾性研究132例符合指南诊断标准并符合aidp、aman或同时有脱髓鞘和轴索损害,排除其它变异型及未有可辨认波形,且进行过电生理检查和复查的病例,对其临床及神经电生理检测结果进行分析,观察临床特征,并进行临床及电生理分型和再分型,研究电生理分型变化情况和特点,观察早期电生理表现与病程进展中分型变化的相关性及其与病情严重程度的相关性。研究结果:gbs患者发病男性多于女性,青中年发病多于儿童和老年人,夏秋季节发病多于春冬季,发病前驱事件以前驱感染多见;患者最常见的首发症状为肢体无力,临床症状以肢体无力最多见,其次为感觉障碍和颅神经症状,多数患者有腱反射减低或消失,mfs患者以颅神经症状为主要特点;gbs临床分型以aidp最为多见,aman型次之,amsan型和不能分型的患者例数相当,mfs和asn少见;gbs患者的感觉传导检测存在感觉分离现象;患者的电生理分型变化在病程发展过程中会发生改变;患者在病程早期出现神经传导阻滞(cb)与从其它亚型转变为aman间存在相关性;早期出现cb与病情严重程度有相关性。结论:gbs患者在整个病情进展过程中其电生理分型并不是一成不变的,有一部分患者的电生理表现会随病情进展发生变化,其电生理分型也随之发生改变,并且这种变化与病情严重程度有一定相关性;早期电生理检测发生神经传导阻滞现象提示随后病情进展中电生理分型由AIDP转变为AMAN的可能;GBS患者早期发生神经传导阻滞现象可在一定程度上提示病情进展加重;GBS患者电生理结果存在感觉分离现象。在GBS患者诊治过程中必要定期进行神经电生理复查,并应重视不同神经感觉传导检测的差异与传导阻滞的检出,以期及时了解患者的诊断、电生理分型及变化,及早发现有提示病情可能加重的指标,及时调整诊治措施,最大程度的减轻患者临床症状,改善患者预后,减少疾病后遗症。
[Abstract]:Background: GBS (Guillain-Barr syndrome GBS) is an autoimmune disease mediated by peripheral nerve, peripheral nerve and nerve root demyelination and inflammatory cell infiltration vasculitis as the main character, even with axonal degeneration. Acute inflammatory demyelinating polyneuropathy (Acute inflammatory demyelinating polyneuropathy AIDP, GBS) is the most classic type of domestic and foreign scholars in recent years, according to the electrophysiological features of GBS patients, the clinical manifestations and pathological changes characteristic of the disease will be divided into AIDP, acute motor axonal neuropathy (Acute motor axonal neuropathy, AMAN), acute motor sensory axonal neuropathy (Acute motor-axonal neuropathy, AMSAN) of different subtypes. But not all patients with electrophysiological subtype are immutable and frozen electrophysiological part of the patients, with the progression of change in patients with GBS. Some cases with conduction block (conduction block, CB), the traditional idea that demyelination is the main cause of the occurrence of CB, but in recent years, studies have shown that CB is more likely to demyelination, axonal membrane damage and sodium channel inactivation reasons such as the comprehensive effect of [1]. in recent years, the clinical and electrophysiological points the change of CB and the prognosis and outcome of the case report, suggesting that early electrophysiological changes appear to block GBS and electrophysiological changes and prognosis of type [2]. Objective: through retrospective analysis of general information, GBS patients with clinical features, laboratory findings, electrophysiological characteristics and the effect of treatment by difference; electrophysiological follow-up observation of patients with GBS disease progression in type of electrophysiological changes, early electrophysiological characteristics and correlation with the severity of the disease and prognosis, early identification of poor prognosis of G Electrophysiological characteristics of early BS patients, and provide reference for the clinical diagnosis and treatment. Methods: experiment one: a retrospective study from 2008 to early 2013 Xijing Hospital neurology, Chinese medical association with Guillain Barre syndrome diagnosis and treatment guidelines in the diagnostic criteria of GBS patients with clinical and electrophysiological data of 338 cases, including 20 cases without check the electrophysiology, finally into the analysis of 318 cases, 24 cases were diagnosed as GBS variant of Miller-Fisher syndrome (miller-fishersyndrome, MFS). The collected data were summed up, the establishment of GBS database of patients with clinical and electrophysiological data, observe the clinical features of medical data and information; electrophysiological results were collected in all subjects the classification, to observe the electrophysiological characteristics and typing characteristics. Experiment two: a retrospective study of 132 patients with diagnostic criteria and guidelines in accordance with AIDP, aman or both Have demyelination and axonal damage, the exclusion of other variants and no identifiable waveform, and conducted electrophysiological examination and review of the case, the clinical and electrophysiological examination results were analyzed to observe the clinical features, and clinical and electrophysiological types and sub type, research type of electrophysiological changes the correlation and characteristics of the observed correlation between type changes in physiological performance and the early course of power and with the severity of the disease. Results: the patients with GBS more men than women, middle-aged patients were more than children and the elderly, in summer and autumn than in spring and winter festival incidence, onset of prodromal events before the flooding occurred; the first symptom for patients with common clinical symptoms and limb weakness, limb weakness is most common, followed by sensory disorder and cranial nerve symptoms, most patients with tendon reflexes to reduce or disappear, MFS patients with cranial nerve symptoms. To GBS in clinical features; AIDP is the most common, aman type, AMSAN type and the number of patients not classified, MFS and Asn are rare; sensory conduction detection of GBS in patients with existing feeling separation phenomenon; electrophysiological type of the patients with change will change in the course of the development process; nerve conduction block appeared early in the course of the disease (CB) patients and from other subtypes into correlation between aman; early CB correlated with the severity of the disease. Conclusion: Patients with GBS in the whole process of the progression of electrophysiological typing is not immutable and frozen, a part of patients with electrophysiological findings with disease progress of change, the electrophysiological type also changed, and this change and the severity of the disease have a certain correlation; early electrophysiological detection nerve block suggests the subsequent progression in electrophysiological type changed from AIDP For AMAN; GBS patients with early occurrence of nerve conduction block phenomenon to a certain extent, suggesting that the disease progression in GBS patients; electrophysiological results are feeling separation phenomenon. In the process of GBS in the diagnosis and treatment of patients with the necessary regular electrophysiological examination, and should pay attention to different detection by God and differences in sensory conduction block detection, in order to understand the patient's diagnosis, typing and electrophysiological changes, suggesting that the disease may increase the detection indicators, timely adjustment of treatment measures, to the maximum extent reduce the clinical symptoms of patients, improve the prognosis of patients, reduce disease sequelae.

【学位授予单位】：第四军医大学
【学位级别】：硕士
【学位授予年份】：2015
【分类号】：R745.43

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