本文選題：吉蘭-巴雷綜合征　切入點：臨床特征　出處：《第四軍醫(yī)大學》2015年碩士論文　論文類型：學位論文

【摘要】：研究背景:GBS(Guillain-Barrésyndrome,GBS)是一種自身免疫介導的周圍神經(jīng)疾病,以周圍神經(jīng)及神經(jīng)根脫髓鞘改變和小血管炎性細胞浸潤為主要特點,甚至可有軸索變性。急性炎性脫髓鞘性多發(fā)性神經(jīng)病(Acute inflammatory demyelinating polyneuropathy,AIDP)為GBS中最經(jīng)典的一型,近年來國內(nèi)外學者根據(jù)GBS患者的電生理表現(xiàn)、臨床表現(xiàn)及神經(jīng)病理改變等特征將該疾病分為AIDP、急性運動軸索型神經(jīng)病(Acute motor axonal neuropathy,AMAN)、急性運動感覺軸索性神經(jīng)病(Acute motor-axonal neuropathy,AMSAN)等不同亞型。但并不是所有患者的電生理亞型都一成不變,部分患者的電生理表現(xiàn)隨病程進展發(fā)生著改變。在GBS患者中有一部分病例伴隨傳導阻滯(conduction block,CB),傳統(tǒng)觀念認為脫髓鞘是導致CB發(fā)生的主要原因,但近些年有研究表明CB更可能是脫髓鞘、軸索膜的損傷及鈉離子通道失活等多方面原因綜合作用的結果[1]。近年來有臨床及電生理分型變化及CB與預后轉(zhuǎn)歸方面的個案報道,提示早期電生理改變出現(xiàn)傳導阻滯可能與GBS電生理分型變化和病情轉(zhuǎn)歸有關[2]。研究目的:通過回顧性研究分析GBS患者的一般資料、臨床特征、實驗室檢查結果、電生理特點及治療效果的差別;通過GBS患者的電生理隨診觀察病程發(fā)展中電生理分型變化、早期電生理特征及其與病情嚴重程度和轉(zhuǎn)歸的相關性,早期識別預后不良gbs患者早期電生理的特點,為臨床診治提供參考和依據(jù)。研究方法:實驗一:回顧性研究2008年至2013年初西京醫(yī)院神經(jīng)內(nèi)科收治符合中華醫(yī)學會吉蘭-巴雷綜合征診療指南中診斷標準的gbs患者臨床及神經(jīng)電生理資料共338例,其中20例未查神經(jīng)電生理,最后納入318例進行分析,其中24例診斷為gbs變異型miller-fisher綜合征(miller-fishersyndrome,mfs)。對所收集病例資料進行歸納整理,建立gbs患者臨床及電生理資料數(shù)據(jù)庫,觀察病歷數(shù)據(jù)一般資料及臨床特征;收集所有研究對象的電生理結果并進行分型,觀察電生理一般特征及分型特點。實驗二:回顧性研究132例符合指南診斷標準并符合aidp、aman或同時有脫髓鞘和軸索損害,排除其它變異型及未有可辨認波形,且進行過電生理檢查和復查的病例,對其臨床及神經(jīng)電生理檢測結果進行分析,觀察臨床特征,并進行臨床及電生理分型和再分型,研究電生理分型變化情況和特點,觀察早期電生理表現(xiàn)與病程進展中分型變化的相關性及其與病情嚴重程度的相關性。研究結果:gbs患者發(fā)病男性多于女性,青中年發(fā)病多于兒童和老年人,夏秋季節(jié)發(fā)病多于春冬季,發(fā)病前驅(qū)事件以前驅(qū)感染多見;患者最常見的首發(fā)癥狀為肢體無力,臨床癥狀以肢體無力最多見,其次為感覺障礙和顱神經(jīng)癥狀,多數(shù)患者有腱反射減低或消失,mfs患者以顱神經(jīng)癥狀為主要特點;gbs臨床分型以aidp最為多見,aman型次之,amsan型和不能分型的患者例數(shù)相當,mfs和asn少見;gbs患者的感覺傳導檢測存在感覺分離現(xiàn)象;患者的電生理分型變化在病程發(fā)展過程中會發(fā)生改變;患者在病程早期出現(xiàn)神經(jīng)傳導阻滯(cb)與從其它亞型轉(zhuǎn)變?yōu)閍man間存在相關性;早期出現(xiàn)cb與病情嚴重程度有相關性。結論:gbs患者在整個病情進展過程中其電生理分型并不是一成不變的,有一部分患者的電生理表現(xiàn)會隨病情進展發(fā)生變化,其電生理分型也隨之發(fā)生改變,并且這種變化與病情嚴重程度有一定相關性;早期電生理檢測發(fā)生神經(jīng)傳導阻滯現(xiàn)象提示隨后病情進展中電生理分型由AIDP轉(zhuǎn)變?yōu)锳MAN的可能;GBS患者早期發(fā)生神經(jīng)傳導阻滯現(xiàn)象可在一定程度上提示病情進展加重;GBS患者電生理結果存在感覺分離現(xiàn)象。在GBS患者診治過程中必要定期進行神經(jīng)電生理復查,并應重視不同神經(jīng)感覺傳導檢測的差異與傳導阻滯的檢出,以期及時了解患者的診斷、電生理分型及變化,及早發(fā)現(xiàn)有提示病情可能加重的指標,及時調(diào)整診治措施,最大程度的減輕患者臨床癥狀,改善患者預后,減少疾病后遺癥。
[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.

【學位授予單位】：第四軍醫(yī)大學
【學位級別】：碩士
【學位授予年份】：2015
【分類號】：R745.43

【參考文獻】

相關期刊論文 前1條

1 朱真真;張玉生;郭黎;;梅毒相關性吉蘭—巴雷綜合征1例報告[J];中國神經(jīng)精神疾病雜志;2013年11期

，

本文編號：1598546