周圍神經(jīng)病進展

1、周圍神經(jīng)病研究新熱點,第二軍醫(yī)大學(xué)上海長海醫(yī)院第二軍醫(yī)大學(xué)周圍神經(jīng)病診治中心管陽太,提要,小纖維神經(jīng)病診斷進展周圍神經(jīng)活檢技術(shù)進展痛性周圍神經(jīng)病診治規(guī)范化周圍神經(jīng)病專病中心建設(shè),小纖維神經(jīng)病診斷進展周圍神經(jīng)活檢技術(shù)進展痛性周圍神經(jīng)病診治規(guī)范化周圍神經(jīng)病專病中心建設(shè),1 Update on SFN,小纖維感覺神經(jīng)?。⊿FN）皮膚交感反應(yīng)（SSR）定量感覺試驗（QST）定量催汗軸突反射（QSART）皮

2、膚活檢（Skin Biospy）痛覺誘發(fā)電位（CHEP）角膜共焦顯微鏡（Corneal Confocal Microscopy，CCM）,1 Update on SFN,CCM原理,,,0 µm,10 µm,30 µm,40 µm,60 µm,100 µm,300 µm,550 µm,,,,,,,,,,,1 Update on SFN,上皮層基質(zhì)層

3、神經(jīng)纖維內(nèi)皮層,丁卡因麻醉鏡片消毒Viscotears眼膠覆蓋調(diào)節(jié)及觀察軟件處理,1 Update on SFN,反應(yīng)DM患者周圍神經(jīng)損害程度1,2,DM患者纖維長度（NFL）及密度（NFD）降低3T1DM患者也有類似改變4酶替代治療的Fabry患者及雜合子神經(jīng)纖維密度顯著降低5,1 Kallinikos P. Invest Ophthalmol Vis Sci 20042 Malik RA. Diabetolo

4、gia 20033 Elisabeth M. Graefes Arch Clin Exp Ophthalmol 20104 Hertz P. Diabetic Medicine 20115 Tavakoli M. Muscle&Nerve 2009,1 Update on SFN,對照,女性患者（ERT-F）,女性雜合子（ERT）,男性半和子（ERT）,Tavakoli M. Muscle&Nerve 2009,小

5、纖維神經(jīng)病診斷進展周圍神經(jīng)活檢技術(shù)進展痛性周圍神經(jīng)病診治規(guī)范化周圍神經(jīng)病專病中心建設(shè),2 Update on Nerve biopsy,傳統(tǒng)的神經(jīng)活檢腓腸神經(jīng)腓淺神經(jīng)橈淺神經(jīng)后骨間神經(jīng)（Posterior nterosseous nerve，PIN）,2 Update on Nerve biopsy,傳統(tǒng)神經(jīng)活檢的缺陷感染疼痛麻木感覺缺失,2 Update on Nerve biopsy,支配拇長伸?。‥P

6、L）和食指伸?。‥IP）后PIN遠端為純感覺支平均直徑1.8mm與前骨間動脈背支伴行,局部或區(qū)域麻醉消毒鋪巾橈尺骨正中線3-4cm切口顯露包含EIP和指總伸?。‥DC）肌腱的第四室鈍性分離骨間膜顯露PIN、伴行骨間動靜脈,2 Update on Nerve biopsy,2 Update on Nerve biopsy,對照,DM,DM,DM（sural）,小纖維神經(jīng)病診斷進展周圍神經(jīng)活檢技術(shù)進展痛性周圍神經(jīng)

7、病診治規(guī)范化周圍神經(jīng)病專病中心建設(shè),3 Update on Painful peripheral neuropathy,ToxicAlcoholMetals: Thallium; ArsenicMedications:cis-platinum; Disulfiram; Ifosfamide; Nitrofurantoin;Perhexiline; Taxol;&#

8、160;Thalidomide; Vinca alkaloidsMushroom: Clitocybe acromelalgaImmuneAcute onsetGuillain BarréDiffuse pain during acute courseDistal pain with incomplete recoveryImmobilityAcute panautonomicI

9、gM antibody associated polyneuropathiesTS-HDSSulfatideIgG antibody associated polyneuropathiesPotassium channel-complex antibodies: CASPR2 & OtherM-proteinGanglionopathiesSjögren'sParaneoplastic (Hu)

10、CryoglobulinemiaConnective tissue diseaseHIV (DILS)VasculitisHereditaryα-galactosidase (Fabry's)GM2 gangliosidosis: Late-onsetSensory Neuropathy I: Lancinating painsMotor-Sensory Neuropathy I & II: Foot

11、deformitySCN9ABurning feet syndromeSmall fiber neuropathyErythromelalgiaAmyloidosisPolyneuropathy: Pain & Paraesthesias in legsCarpal tunnel syndrome: Pain in handsPorphyriaEpisodic pain syndromes, Familial&

12、#160;(FEPS)Restless legs syndromes: Some with PNS involvementHereditary (Acromelalgia)  : 12qHSN 1HMSN 5CMT 2Huntington disease-like 1  Also: Renal failure,MetabolicDiabeticAcute: Lumbar or Thor

13、acic radiculopathy; Distal sensoryChronic: "Small fiber" neuropathyAlcoholic: AcutePellagra (niacin)Beriberi (thiamine)Strachan syndrome (Cuban neuropathy)Post gastroplastyHypertriglyceridemiaChronic mo

14、untain sicknessIdiopathicDistal sensory polyneuropathy syndromesErythromelalgiaMononeuritis multiplexMotor disorders with painDiabetic amyotrophyPolioInfectionsPolioHIVLocalized disordersNerve lesions:Trigem

15、inalBrachial plexusLumbosacral plexopathyThoracic outlet syndromeMedianPosterior tibial (Tarsal tunnel syndrome)Radiculopathies: Upper & Lower ExtremitiesBurning mouth syndromeDiabeti

16、c AmyotrophyReflex Sympathetic Dystrophy (Complex regional pain syndrome)Infections: Herpes zosterNeoplastic infiltration: Burning pain with dysesthesia in area of sensory lossPost surgical localized pain s

17、yndromesAcute onset of pain & paresthesiasAcute porphyriaToxic: Thallium; Arsenic; Perhexiline; Vinca alkaloidsGuillain-BarréAcute sensory neuropathiesSpinal: Myelopathy; Radiculo

18、pathy,neuromuscular.wustl.eduPAINFUL NEUROPATHIES: Differential Diagnosis,3 Update on Painful peripheral neuropathy,中華神經(jīng)科雜志，2012,3 Update on Painful peripheral neuropathy,病因分類代謝和營養(yǎng)障礙外傷和壓迫免疫介導(dǎo)感染藥物和中毒腫瘤相關(guān)隱源性,臨床表現(xiàn)癥

19、狀疼痛體征感覺障礙運動障礙腱反射異常自主神經(jīng)功能異常,3 Update on Painful peripheral neuropathy,評分VAS現(xiàn)時疼痛強度評分SF-MPQ臨床表現(xiàn)評定量表神經(jīng)經(jīng)病理性疼痛自評量表Leeds神經(jīng)病理性痛癥狀和體征評價表DN4,輔助檢查電生理NCVEMGSSRQST自主神經(jīng)功能活檢影像血液、CSF,3 Update on Painful peripheral

20、neuropathy,診斷流程,治療用藥,European Journal of Neurology2010,常見痛性周圍神經(jīng)病的用藥推薦,PAIN 2013,3 Update on Painful peripheral neuropathy,其它指南,http://guidance.nice.org.uk/CG173,Clin Proc. 2010,小纖維神經(jīng)病診斷進展周圍神經(jīng)活檢技術(shù)進展痛性周圍神經(jīng)病診治規(guī)范化周圍神經(jīng)病

21、專病中心建設(shè),4 周圍神經(jīng)病專病中心,不同人群發(fā)病率2-3%55歲以上發(fā)病率8%早期治療阻止疾病進展>100種病因診斷困難反復(fù)就診，增加就醫(yī)費用，浪費醫(yī)療資源,4 周圍神經(jīng)病專病中心,,4 周圍神經(jīng)病專病中心,國際市場占有率最高的電生理設(shè)備品牌丹迪Keypoint.net（左）光電MEB9002K（右）,檢測大纖維,以色列Medoc定量感覺分析儀TSA-II,檢測小纖維,4 周圍神經(jīng)病專病中心,神經(jīng)活檢,皮膚