感覺自主神經(jīng)節(jié)神經(jīng)病課件

感覺自主神經(jīng)節(jié)神經(jīng)病Sensoryandautonomicganglionopathy神內(nèi)二病房神經(jīng)節(jié)的解剖學(xué)腦脊神經(jīng)節(jié)：感覺神經(jīng)節(jié)三叉神經(jīng)節(jié)前庭蝸神經(jīng)節(jié)后根神經(jīng)節(jié)植物神經(jīng)節(jié)交感神經(jīng)節(jié)副交感神經(jīng)節(jié)后根神經(jīng)節(jié)、后根后根神經(jīng)節(jié)SENSORYGANGLION(GASSERIAN)

Pseudounipolarneurons交感神經(jīng)節(jié)、交感干椎旁神經(jīng)節(jié)；位于脊柱旁，連成交感干椎前神經(jīng)節(jié)：位于脊柱前方，腹主動脈主要臟支的根部。腹腔神經(jīng)節(jié)主動脈腎神經(jīng)節(jié)腸系膜上神經(jīng)節(jié)腸系膜下神經(jīng)節(jié)副交感神經(jīng)節(jié)節(jié)前神經(jīng)元：腦干副交感核，脊髓S2～4節(jié)段，骶副交感核；節(jié)后神經(jīng)元：器官旁節(jié)、器官內(nèi)節(jié)SYMPATHETICGANGLIONCELLSHuman,Glees'method,162x.

命名Sensoryneuronopathies（ganglionopathy）Acute（subacute）sensoryataxicandautonomicneuronopathiesDorsalrootganglionitisAcute（subacute,chronic）sensoryganglionopathiesChronicidiopathicataxicneuropathyAcuteautonomicandsensoryneuropathyAcute（subacute）autonomicandsensoryganglionopathy病因?qū)W感染性：麻疹病毒，EB病毒，帶狀皰疹病毒，單純皰疹病毒感染，HIV感染，慢性活動性肝炎；副腫瘤綜合癥（抗Hu綜合癥）；干燥綜合癥；藥物和環(huán)境毒素（化療藥物，維生素B6，痢特靈）；單克隆丙球蛋白?。痪S生素E缺乏，脊髓小腦性共濟失調(diào)特發(fā)性病理學(xué)尸檢資料：脊髓后索髓鞘完全脫失，后角細胞減少，后根萎縮，側(cè)索和前索未見明確改變，頸胸腰骶節(jié)段后根神經(jīng)節(jié)和交感神經(jīng)節(jié)神經(jīng)元嚴重丟失，殘存神經(jīng)元非常稀少，伴Nageotte結(jié)節(jié)形成。免疫介導(dǎo)疾?。篊D8＋T細胞浸潤；周圍神經(jīng)活檢：大有髓纖維嚴重丟失，交感神經(jīng)節(jié)受累時亦見無髓纖維丟失。Subacuteinflammationinsympatheticanddorsalrootganglia.(A)Inflammatorymononuclearcellsdiffuselyinfiltratetheperivascularspace,theendoneurium,andaggregatearoundneurons.(Hematoxylinandeosin,originalmagnification×50.)(B)NumerousCD3+Tcellsinfiltratetheendoneurium(originalmagnification×50).(C)Anti-IgGlabelsnumerousendoneurialplasmacells,thecytoplasmandnucleiofneurons(arrows).Thearrowheadsindicatenegativeneurons(originalmagnification×50).(D)Double-immunolabelingforCD8(brownlabel)andIgG(bluelabel)demonstratesclosecontactofCD8+TcellstoIgGpositiveneurons(arrows)(originalmagnification×50).(E)Double-immunolabelingforIgG(brownlabel)andCD8(bluelabel)showscloseattachmentofaCD8+TcelltoanIgGnegativeneuron(arrow)(originalmagnification×125).(F)TwoCD8+Tcellsindenttheneuronalsurface(originalmagnification×250).(Allmagnificationsbefore34%reduction.).臨床表現(xiàn)開始常為四肢遠端麻木、感覺異常；病程可為急性、亞急性和慢性，或者為隱襲性；后根神經(jīng)節(jié)受累表現(xiàn)：深感覺障礙（振動覺和關(guān)節(jié)位置覺突出），步態(tài)不穩(wěn)，步基寬，感覺性共濟失調(diào)，假性手足徐動癥，深反射降低或消失。交感、副交感神經(jīng)節(jié)受累表現(xiàn)：頑固性肢體疼痛，多汗或無汗，Adie瞳孔，性功能障礙，體位性低血壓，假性腸梗阻。MRI發(fā)現(xiàn)軸位和矢狀位可見后索長T2信號后根神經(jīng)節(jié)病變，其中樞突和周圍突均發(fā)生華勒變性診斷各種年齡均可發(fā)病，病程呈急性、亞急性或者慢性過程；感覺性共濟失調(diào)，振動覺和關(guān)節(jié)位置覺降低或消失，而運動癥狀和痛溫覺相對較輕或正常，假性手足徐動癥，提示后根神經(jīng)節(jié)受累；常常伴有自主神經(jīng)節(jié)受累表現(xiàn)：頑固性疼痛，多汗，體位性低血壓，瞳孔改變，假性腸梗阻等肌電圖提示為單純相，感覺神經(jīng)動作電位波幅降低或消失，符合軸索性神經(jīng)病；誘發(fā)電位：外周誘發(fā)潛伏期電位波幅降低；腓腸神經(jīng)活檢提示大有髓纖維嚴重丟失，小有髓纖維保存良好。鑒別診斷純感覺性GBS：起病快，感覺功能異常分布廣泛且對稱，腦脊液蛋白高而細胞數(shù)少或無，電生理檢查符合脫髓鞘性周圍神經(jīng)病，恢復(fù)可較完全。（MirallesF,MonteroJ,ReneR,etal.PuresensoryGuillain-Barresyndrome.JNeurol

NeurosurgPsychiatry,1992,55:411-12）Miller-Fis