免疫介導(dǎo)的周圍神經(jīng)病

免疫介導(dǎo)的周圍神經(jīng)病病例特點(diǎn)老年男性，慢性病程，反復(fù)緩解復(fù)發(fā)；長(zhǎng)期慢性腹瀉病史；自1998年2月開始雙上肢麻木無力，1999年出現(xiàn)四肢麻木活動(dòng)無力，癥狀反復(fù)遷延復(fù)發(fā)，無明確完全緩解期，使用激素和環(huán)磷酰胺等有效；查體可見雙上肢伸肌肌力Ⅴ-級(jí)，雙下肢肌力Ⅴ級(jí)。四肢肌張力和腱反射低下，雙手掌指關(guān)節(jié)以下、雙踝關(guān)節(jié)以下針刺覺、音叉震動(dòng)覺減退

肌電圖：右脛前肌、右拇短外展肌可見巨大電位，運(yùn)動(dòng)和感覺神經(jīng)傳導(dǎo)速度減慢

誘發(fā)電位：VEP左側(cè)各波潛伏期延長(zhǎng)，BAEP左耳Ⅰ波未引出，SEP雙側(cè)P15至N20潛伏期延長(zhǎng)，左側(cè)C7、Erb’s點(diǎn)及右側(cè)Erb’s點(diǎn)波形未引出。雙下肢SEP摑窩未引出波形。

病例特點(diǎn)腦脊液檢查：細(xì)胞數(shù)2600/mm3,WBC2/mm3，生化：Pro76mg/dl,C-Glu3.1mmol/L,C-Cl124mmol/L；GM1-IgM(+),GM1-IgG(-)。

血GM1-IgM1：200（參考值≤800），GM1-IgG1：50（參考值≤200）。

尿本－周蛋白陰性

病例特點(diǎn)血免疫全項(xiàng)均未見異常；

頸MRI：C5－6間盤后突，后緣骨刺壓迫脊髓，髓內(nèi)可見長(zhǎng)T2信號(hào)

病例特點(diǎn)定位診斷周圍神經(jīng)：四肢麻木，針刺覺減退，提示感覺神經(jīng)小纖維受累；四肢遠(yuǎn)端音叉覺減退，提示感覺神經(jīng)大纖維受累；電生理檢查出現(xiàn)感覺神經(jīng)傳導(dǎo)速度慢、傳導(dǎo)阻滯。四肢以遠(yuǎn)端為重的運(yùn)動(dòng)功能下降，腱反射低下，無病理征，肌電圖運(yùn)動(dòng)神經(jīng)傳導(dǎo)速度慢，提示運(yùn)動(dòng)神經(jīng)纖維受累。運(yùn)動(dòng)神經(jīng)元或神經(jīng)根：電生理檢查右脛前肌可見巨大電位，多相電位增多，右拇短外展肌可見巨大電位。定位診斷定性診斷免疫介導(dǎo)的周圍神經(jīng)病IgMmonoclonalgammopathyofundeterminedsignificanceChronicinflammatorydemyelinatingpolyneuropathyParaneoplasticneuropathies未明意義的單克隆丙球蛋白病

（IgMmonoclonalgammopathyofundeterminedsignificance〕發(fā)病時(shí)間大于2年，慢性遷延病程；遠(yuǎn)端對(duì)稱性感覺運(yùn)動(dòng)神經(jīng)??；肌電圖脫髓鞘改變與運(yùn)動(dòng)神經(jīng)元受累表現(xiàn)；腦脊液GM1-IgM(+),GM1-IgG(-)激素和環(huán)磷酰胺治療有效。腓腸神經(jīng)活檢：脫髓鞘改變，部分軸索變性，血管周圍少量淋巴細(xì)胞AcausalrelationbetweendemyelinatingpolyneuropathyandMGUSshouldbeconsideredinapatientwith:(1)DemyelinatingpolyneuropathyaccordingtotheelectrodiagnosticANNcriteriaforidiopathicCIDP(2)PresenceofanMprotein(IgM,IgG,orIgA),withoutevidenceofmalignantplasmacelldyscrasiaslikemultiplemyeloma,lymphoma,Waldenstrom’smacroglobulinemia,oramyloidosis.(3)

Familyhistorynegativeforneuropathy.(4)

Age>30yearsProposalforcriteriafordemyelinatingpolyneuropathyassociatedwithMGUS

Therelationisdefinitewhenthefollowingispresent:(1)

IgMMproteinwithanti-MAGantibodiesTherelationisprobablewhenatleastthreeofthefollowingarepresentinapatientwithoutanti-MAGantibodies:(1)

Timetopeakoftheneuropathy>2years(2)

Chronicslowlyprogressivecoursewithoutrelapsingorremittingperiods(3)

Symmetricaldistalpolyneuropathy(4)

SensorysymptomsandsignspredominateovermotorfeaturesProposalforcriteriafordemyelinatingpolyneuropathyassociatedwithMGUS

ProposalforcriteriafordemyelinatingpolyneuropathyassociatedwithMGUSAcausalrelationisunlikelywhenatleastthreeofthefollowingarepresentinapatientwithoutanti-MAGantibodies:(1)

Mediantimetopeakoftheneuropathyiswithin1year(2)

Clinicalcourseisrelapsingandremittingormonophasic(3)

Cranialnervesareinvolved(4)

Neuropathyisasymmetrical(5)

Motorsymptomsandsignspredominate(6)

HistoryofprecedinginfectionPresenceofabnormalmedianSNAPincombinationwithnormalsuralSNAP.CIDP診斷標(biāo)準(zhǔn)(Barohn,1989年)必須標(biāo)準(zhǔn)進(jìn)行性肌無力(緩慢進(jìn)展,階梯性或復(fù)發(fā))2個(gè)月；對(duì)稱性上肢或下肢的近端和遠(yuǎn)端肌無力；腱反射降低或消失。必須排除標(biāo)準(zhǔn)，病人必須無：(