急性炎症性脱髓鞘性-多发性神经病ppt课件.ppt
《急性炎症性脱髓鞘性-多发性神经病ppt课件.ppt》由会员分享,可在线阅读,更多相关《急性炎症性脱髓鞘性-多发性神经病ppt课件.ppt(49页珍藏版)》请在咨信网上搜索。
1、急性炎症性脱髓鞘性多急性炎症性脱髓鞘性多发性神性神经病病Acute Inflammatory Demyelinating Polyneuropathy,AIDPAcute Inflammatory Demyelinating Polyneuropathy,AIDP浙江大学医学院附属第一医院浙江大学医学院附属第一医院 1.Introductionn nLandry Landry-Landrys paralysis 1859Landrys paralysis 1859n nLandryreportedLandryreportedan acute,ascendingan acute,ascendin
2、g,predominantlypredominantlymotorparalysiswithrespiratoryfailure,leadingtodeathmotorparalysiswithrespiratoryfailure,leadingtodeathn nGuillair-Barre 1916 2Guillair-Barre 1916 2例例 n nGuillain,Guillain,BarreBarre andand strohlstrohl (1916)(1916)reportedreported a a benignbenignpolyneuritispolyneuritis
3、withwith albuminocytologic albuminocytologic dissociation dissociation in in the the CSF CSF(raised(raised concentration concentration of of CSF CSF protein protein but but a a normal normal cell count)cell count)蛋白蛋白蛋白蛋白细细胞分离胞分离胞分离胞分离是本病的特征是本病的特征2.GuillainBarre LandryStrohl3.Introductionn nIn 1956,
4、C Miller Fisher described a triad of acute In 1956,C Miller Fisher described a triad of acute ophthalmoplegia,ataxia,and areflexia,now known as ophthalmoplegia,ataxia,and areflexia,now known as Fishers syndromeFishers syndromen nDuring the past 15 years,GBS has become clear that During the past 15 y
5、ears,GBS has become clear that this clinical picture,now called Guillain-Barr this clinical picture,now called Guillain-Barr syndrome,and have different pathological subtypes syndrome,and have different pathological subtypes 4.Epidemiologyn nWorldwide incidencen n0.6-4/100 000 per year throughout th
6、e world0.6-4/100 000 per year throughout the worldn nChina incidencen n0.66 per 100 000 for all ages0.66 per 100 000 for all agesn n可可可可发发生于任何年生于任何年生于任何年生于任何年龄龄,男女,男女,男女,男女发发病率相似,夏秋多病率相似,夏秋多病率相似,夏秋多病率相似,夏秋多见见5.6.临临床表床表现现:中国:中国n n儿童和青少年,夏初。n nEMG:轴索损害,AMAN。n nEMGEMG符合符合AMANAMAN的的为为6565,符合,符合AIDPAIDP的
7、的为为2424。n n66有CJ抗体,42有GM1抗体,其他神经节苷脂抗体为1726。与西方国家不同,GM1抗体与AMAN或AIDP无关。近来发现AMAN与GD1a抗体相关密切。7.临临床表床表现现:中国:中国n n病理:n nAMANAMAN:IgGIgG和和补补体在体在轴轴索周索周围围沉沉积积,巨噬,巨噬细细胞胞侵入侵入轴轴索周索周围间围间隙,隙,严严重者有重者有轴轴索索变变性。性。n nAIDPAIDP:IgGIgG和和补补体在髓鞘外沉体在髓鞘外沉积积,巨噬,巨噬细细胞也胞也在髓鞘外,在髓鞘外,“撕开撕开”髓鞘。髓鞘。n nAMSANAMSAN:感:感觉轴觉轴索比运索比运动轴动轴索索损损
8、害重。害重。n nEMG不能预测病理。8.Pathogenesis and Pathophysiologyn nThe cause of this syndrome is unknown,but it is generally viewed to be an autoimmune response to a bacterial or viral infection.n n病因尚未完全病因尚未完全病因尚未完全病因尚未完全阐阐明明明明9.Etiologyn nC CampylobacterampylobacterJ Jejuniejunin nEpstein-BarrVirusEpstein-Ba
9、rrVirus(EBV)(EBV)n nCytomegalovirusCytomegalovirus(CMV)(CMV)n nHIVHIVn nVaccinationsn n空空空空肠肠肠肠弯曲菌弯曲菌弯曲菌弯曲菌10.Pathogenesis and Pathophysiologyn nAn acute immune-mediated polyneuropathy,An acute immune-mediated polyneuropathy,component of pathogen was similar with myelin component of pathogen was sim
10、ilar with myelin sheath of peripheral nervesheath of peripheral nerven n与感染有关的自身免疫性疾病与感染有关的自身免疫性疾病与感染有关的自身免疫性疾病与感染有关的自身免疫性疾病,病原体某些成分与病原体某些成分与病原体某些成分与病原体某些成分与周周周周围围神神神神经经的髓鞘成分相似的髓鞘成分相似的髓鞘成分相似的髓鞘成分相似 11.Pathophysiologyn n主要病理特点主要病理特点主要病理特点主要病理特点(principal characteristic of (principal characteristic of
11、 pathology)pathology)n n节节段性脱髓鞘段性脱髓鞘段性脱髓鞘段性脱髓鞘(segmental demyelization)(segmental demyelization)n n小血管周小血管周小血管周小血管周围围炎性炎性炎性炎性细细胞浸胞浸胞浸胞浸润润12.13.14.15.Clinical manifestationsn n多数患者有前多数患者有前多数患者有前多数患者有前驱驱症状症状症状症状(起病前起病前起病前起病前1313周)周)周)周)n n呼吸道感染症状呼吸道感染症状呼吸道感染症状呼吸道感染症状n n喉痛、鼻塞、喉痛、鼻塞、喉痛、鼻塞、喉痛、鼻塞、发热发热n n消
12、化道症状消化道症状消化道症状消化道症状n n腹泻、呕吐腹泻、呕吐腹泻、呕吐腹泻、呕吐16.Clinical manifestationsn nProgressive ascending symmetrical weakness of the Progressive ascending symmetrical weakness of the limbslimbsn nInvolvement of proximal and distal musclesInvolvement of proximal and distal musclesn nNumbness and tingling in the
13、hands and feetNumbness and tingling in the hands and feetn nBack painBack pain17.Clinical manifestationsn nDepressed or absent reflexesDepressed or absent reflexesn nInvolvement of cranial nerves(facial nerves most Involvement of cranial nerves(facial nerves most commonly involved)commonly involved)
14、n nRespiratory failure(involved respiratory muscles)Respiratory failure(involved respiratory muscles)n nProgression to peak disability in 4 wkProgression to peak disability in 4 wkn nautonomic nerve symptom autonomic nerve symptom 18.Assessmentn nCerebrospinal fluidn nIncreased protein usually after
15、 7 to 10 days.Increased protein usually after 7 to 10 days.n nWhile some protein is normally present,an increased While some protein is normally present,an increased amount without an increase in the number of white amount without an increase in the number of white blood cells may indicate GBSblood
16、cells may indicate GBSn n蛋白蛋白细细胞分离胞分离19.Assessmentn nNerve conduction velocity testn n Nerve conduction studies are a dependable and Nerve conduction studies are a dependable and early diagnostic indicator of GBS.early diagnostic indicator of GBS.n nshows demyelization and damage to the nerve shows
17、demyelization and damage to the nerve sheathsheathn nF F反反应应、H H反射异常反射异常 PLPL延延长长,NCVNCV减慢减慢n n 传导传导阻滞阻滞现现象,伴或不伴有波幅降低象,伴或不伴有波幅降低20.Assessmentn n腓腓肠神神经活活检n n节节段性脱髓鞘段性脱髓鞘段性脱髓鞘段性脱髓鞘n n小血管周小血管周小血管周小血管周围围炎性炎性炎性炎性细细胞浸胞浸胞浸胞浸润润n nElectrocardiogram(EKG)n nMay show abnormalities in cardiac rhythmn n心律失常21.Sub
18、types of GBS n n经经典型典型典型典型 AIDPAIDPn nFisherFisher综综合症合症合症合症(Miller Fisher syndrome)(Miller Fisher syndrome):n n三三三三联联征征征征-“眼外肌麻痹眼外肌麻痹眼外肌麻痹眼外肌麻痹、共共共共济济失失失失调调、腱反射消失、腱反射消失、腱反射消失、腱反射消失”,还还有中枢神有中枢神有中枢神有中枢神经经系系系系统损统损害害害害 n nItwasthoughttobeavariantofGBSandcompriseItwasthoughttobeavariantofGBSandcomprisec
19、ompleteophthalmoplegiawithataxiaandareflexiacompleteophthalmoplegiawithataxiaandareflexian n脑脑神神神神经经型型型型22.Subtypes of GBS n n轴轴突型突型突型突型 n n纯纯运运运运动动型(型(型(型(AMANAMAN)n n运运运运动动 感感感感觉觉 型型型型 (AMSAN AMSAN)n n急性感急性感急性感急性感觉觉性多性多性多性多发发性神性神性神性神经经炎(炎(炎(炎(ASPASP)n n急性全自主神急性全自主神急性全自主神急性全自主神经经病(病(病(病(APNAPN)n n假
20、性肌假性肌假性肌假性肌营营养不良养不良养不良养不良n n复复复复发发型型型型23.Diagnosisn nRequired for diagnosisn nProgressive weakness of one or more limbProgressive weakness of one or more limbn nDistal areflexia with proximal areflexia or Distal areflexia with proximal areflexia or hyporeflexiahyporeflexia24.Diagnosisn nSupportive d
- 配套讲稿:
如PPT文件的首页显示word图标,表示该PPT已包含配套word讲稿。双击word图标可打开word文档。
- 特殊限制:
部分文档作品中含有的国旗、国徽等图片,仅作为作品整体效果示例展示,禁止商用。设计者仅对作品中独创性部分享有著作权。
- 关 键 词:
- 急性 炎症 脱髓鞘 多发性 神经病 ppt 课件
1、咨信平台为文档C2C交易模式,即用户上传的文档直接被用户下载,收益归上传人(含作者)所有;本站仅是提供信息存储空间和展示预览,仅对用户上传内容的表现方式做保护处理,对上载内容不做任何修改或编辑。所展示的作品文档包括内容和图片全部来源于网络用户和作者上传投稿,我们不确定上传用户享有完全著作权,根据《信息网络传播权保护条例》,如果侵犯了您的版权、权益或隐私,请联系我们,核实后会尽快下架及时删除,并可随时和客服了解处理情况,尊重保护知识产权我们共同努力。
2、文档的总页数、文档格式和文档大小以系统显示为准(内容中显示的页数不一定正确),网站客服只以系统显示的页数、文件格式、文档大小作为仲裁依据,平台无法对文档的真实性、完整性、权威性、准确性、专业性及其观点立场做任何保证或承诺,下载前须认真查看,确认无误后再购买,务必慎重购买;若有违法违纪将进行移交司法处理,若涉侵权平台将进行基本处罚并下架。
3、本站所有内容均由用户上传,付费前请自行鉴别,如您付费,意味着您已接受本站规则且自行承担风险,本站不进行额外附加服务,虚拟产品一经售出概不退款(未进行购买下载可退充值款),文档一经付费(服务费)、不意味着购买了该文档的版权,仅供个人/单位学习、研究之用,不得用于商业用途,未经授权,严禁复制、发行、汇编、翻译或者网络传播等,侵权必究。
4、如你看到网页展示的文档有www.zixin.com.cn水印,是因预览和防盗链等技术需要对页面进行转换压缩成图而已,我们并不对上传的文档进行任何编辑或修改,文档下载后都不会有水印标识(原文档上传前个别存留的除外),下载后原文更清晰;试题试卷类文档,如果标题没有明确说明有答案则都视为没有答案,请知晓;PPT和DOC文档可被视为“模板”,允许上传人保留章节、目录结构的情况下删减部份的内容;PDF文档不管是原文档转换或图片扫描而得,本站不作要求视为允许,下载前自行私信或留言给上传者【1587****927】。
5、本文档所展示的图片、画像、字体、音乐的版权可能需版权方额外授权,请谨慎使用;网站提供的党政主题相关内容(国旗、国徽、党徽--等)目的在于配合国家政策宣传,仅限个人学习分享使用,禁止用于任何广告和商用目的。
6、文档遇到问题,请及时私信或留言给本站上传会员【1587****927】,需本站解决可联系【 微信客服】、【 QQ客服】,若有其他问题请点击或扫码反馈【 服务填表】;文档侵犯商业秘密、侵犯著作权、侵犯人身权等,请点击“【 版权申诉】”(推荐),意见反馈和侵权处理邮箱:1219186828@qq.com;也可以拔打客服电话:4008-655-100;投诉/维权电话:4009-655-100。