免疫介导的周围神经病.ppt

神内二病例讨论,ImmunemediatedneuropathyCIDPMGUS,病例特点,老年男性，慢性病程，反复缓解复发；长期慢性腹泻病史；自1998年2月开始双上肢麻木无力，1999年出现四肢麻木活动无力，症状反复迁延复发，无明确完全缓解期，使用激素和环磷酰胺等有效；查体可见双上肢伸肌肌力-级，双下肢肌力级。四肢肌张力和腱反射低下，双手掌指关节以下、双踝关节以下针刺觉、音叉震动觉减退,肌电图：右胫前肌、右拇短外展肌可见巨大电位，运动和感觉神经传导速度减慢诱发电位：VEP左侧各波潜伏期延长，BAEP左耳波未引出，SEP双侧P15至N20潜伏期延长，左侧C7、Erbs点及右侧Erbs点波形未引出。双下肢SEP掴窝未引出波形。,病例特点,脑脊液检查：细胞数2600/mm3,WBC2/mm3，生化：Pro76mg/dl,C-Glu3.1mmol/L,C-Cl124mmol/L；GM1-IgM(+),GM1-IgG(-)。血GM1-IgM1：200（参考值800），GM1-IgG1：50（参考值200）。尿本周蛋白阴性,病例特点,血免疫全项均未见异常；颈MRI：C56间盘后突，后缘骨刺压迫脊髓，髓内可见长T2信号,病例特点,定位诊断,周围神经：四肢麻木，针刺觉减退，提示感觉神经小纤维受累；四肢远端音叉觉减退，提示感觉神经大纤维受累；电生理检查出现感觉神经传导速度慢、传导阻滞。四肢以远端为重的运动功能下降，腱反射低下，无病理征，肌电图运动神经传导速度慢，提示运动神经纤维受累。,运动神经元或神经根：电生理检查右胫前肌可见巨大电位，多相电位增多，右拇短外展肌可见巨大电位。,定位诊断,定性诊断,免疫介导的周围神经病IgMmonoclonalgammopathyofundeterminedsignificanceChronicinflammatorydemyelinatingpolyneuropathyParaneoplasticneuropathies,未明意义的单克隆丙球蛋白病（IgMmonoclonalgammopathyofundeterminedsignificance,发病时间大于2年，慢性迁延病程；远端对称性感觉运动神经病；肌电图脱髓鞘改变与运动神经元受累表现；脑脊液GM1-IgM(+),GM1-IgG(-)激素和环磷酰胺治疗有效。腓肠神经活检：脱髓鞘改变，部分轴索变性，血管周围少量淋巴细胞,AcausalrelationbetweendemyelinatingpolyneuropathyandMGUSshouldbeconsideredinapatientwith:(1)DemyelinatingpolyneuropathyaccordingtotheelectrodiagnosticANNcriteriaforidiopathicCIDP(2)PresenceofanMprotein(IgM,IgG,orIgA),withoutevidenceofmalignantplasmacelldyscrasiaslikemultiplemyeloma,lymphoma,Waldenstromsmacroglobulinemia,oramyloidosis.(3)Familyhistorynegativeforneuropathy.(4)Age30years,ProposalforcriteriafordemyelinatingpolyneuropathyassociatedwithMGUS,Therelationisdefinitewhenthefollowingispresent:(1)IgMMproteinwithanti-MAGantibodiesTherelationisprobablewhenatleastthreeofthefollowingarepresentinapatientwithoutanti-MAGantibodies:(1)Timetopeakoftheneuropathy2years(2)Chronicslowlyprogressivecoursewithoutrelapsingorremittingperiods(3)Symmetricaldistalpolyneuropathy(4)Sensorysymptomsandsignspredominateovermotorfeatures,ProposalforcriteriafordemyelinatingpolyneuropathyassociatedwithMGUS,ProposalforcriteriafordemyelinatingpolyneuropathyassociatedwithMGUS,Acausalrelationisunlikelywhenatleastthreeofthefollowingarepresentinapatientwithoutanti-MAGantibodies:(1)Mediantimetopeakoftheneuropathyiswithin1year(2)Clinicalcourseisrelapsingandremittingormonophasic(3)Cranialnervesareinvolved(4)Neuropathyisasymmetrical(5)Motorsymptomsandsignspredominate(6)HistoryofprecedinginfectionPresenceofabnormalmedianSNAPincombinationwithnormalsuralSNAP.,CIDP诊断标准(Barohn,1989年),必须标准进行性肌无力(缓慢进展,阶梯性或复发)2个月；对称性上肢或下肢的近端和远端肌无力；腱反射降低或消失。必须排除标准，病人必