周围神经病进展2

周围神经病研究新热点,第二军医大学上海长海医院第二军医大学周围神经病诊治中心管阳太,提要,小纤维神经病诊断进展周围神经活检技术进展痛性周围神经病诊治规范化周围神经病专病中心建设,小纤维神经病诊断进展周围神经活检技术进展痛性周围神经病诊治规范化周围神经病专病中心建设,1 Update on SFN,小纤维感觉神经病（SFN）皮肤交感反应（SSR）定量感觉试验（QST）定量催汗轴突反射（QSART）皮肤活检（Skin Biospy）痛觉诱发电位（CHEP）角膜共焦显微镜（Corneal Confocal Microscopy，CCM）,1 Update on SFN,CCM原理,0 m,10 m,30 m,40 m,60 m,100 m,300 m,550 m,1 Update on SFN,上皮层基质层神经纤维内皮层,丁卡因麻醉镜片消毒Viscotears眼胶覆盖调节及观察软件处理,1 Update on SFN,反应DM患者周围神经损害程度1,2,DM患者纤维长度（NFL）及密度（NFD）降低3T1DM患者也有类似改变4酶替代治疗的Fabry患者及杂合子神经纤维密度显著降低5,1 Kallinikos P. Invest Ophthalmol Vis Sci 20042 Malik RA. Diabetologia 20033 Elisabeth M. Graefes Arch Clin Exp Ophthalmol 20104 Hertz P. Diabetic Medicine 20115 Tavakoli M. Muscle&Nerve 2009,1 Update on SFN,对照,女性患者（ERT-F）,女性杂合子（ERT）,男性半和子（ERT）,Tavakoli M. Muscle&Nerve 2009,小纤维神经病诊断进展周围神经活检技术进展痛性周围神经病诊治规范化周围神经病专病中心建设,2 Update on Nerve biopsy,传统的神经活检腓肠神经腓浅神经桡浅神经后骨间神经（Posterior nterosseous nerve，PIN）,2 Update on Nerve biopsy,传统神经活检的缺陷感染疼痛麻木感觉缺失,2 Update on Nerve biopsy,支配拇长伸肌（EPL）和食指伸肌（EIP）后PIN远端为纯感觉支平均直径1.8mm与前骨间动脉背支伴行,局部或区域麻醉消毒铺巾桡尺骨正中线3-4cm切口显露包含EIP和指总伸肌（EDC）肌腱的第四室钝性分离骨间膜显露PIN、伴行骨间动静脉,2 Update on Nerve biopsy,2 Update on Nerve biopsy,对照,DM,DM,DM（sural）,小纤维神经病诊断进展周围神经活检技术进展痛性周围神经病诊治规范化周围神经病专病中心建设,3 Update on Painful peripheral neuropathy,ToxicAlcoholMetals:Thallium;ArsenicMedications:cis-platinum;Disulfiram;Ifosfamide;Nitrofurantoin;Perhexiline;Taxol;Thalidomide;Vinca alkaloidsMushroom:Clitocybe acromelalgaImmuneAcute onsetGuillain BarrDiffuse pain during acute courseDistal pain with incomplete recoveryImmobilityAcutepanautonomicIgM antibody associated polyneuropathiesTS-HDSSulfatideIgG antibody associated polyneuropathiesPotassium channel-complex antibodies: CASPR2 & OtherM-proteinGanglionopathiesSjgrensParaneoplastic (Hu)CryoglobulinemiaConnective tissue diseaseHIV (DILS)VasculitisHereditary-galactosidase (Fabrys)GM2 gangliosidosis: Late-onsetSensory Neuropathy I: Lancinating painsMotor-Sensory Neuropathy I & II: Foot deformitySCN9ABurning feet syndromeSmall fiber neuropathyErythromelalgiaAmyloidosisPolyneuropathy: Pain & Paraesthesias in legsCarpal tunnel syndrome: Pain in handsPorphyriaEpisodic pain syndromes, Familial(FEPS)Restless legs syndromes: Some with PNS involvementHereditary (Acromelalgia) : 12qHSN 1HMSN 5CMT 2Huntington disease-like 1 Also:Renal failure,MetabolicDiabeticAcute: Lumbar or Thoracic radiculopathy; Distal sensoryChronic: Small fiber neuropathyAlcoholic: AcutePellagra (niacin)Beriberi (thiamine)Strachan syndrome (Cuban neuropathy)Post gastroplastyHypertriglyceridemiaChronic mountain sicknessIdiopathicDistal sensory polyneuropathy syndromesErythromelalgiaMononeuritis multiplexMotor disorders with painDiabetic amyotrophyPolioInfectionsPolioHIVLocalized disordersNerve lesions:TrigeminalBrachial plexusLumbosacral plexopathyThoracic outlet syndromeMedianPosterior tibial(Tarsal tunnel syndrome)Radiculopathies:Upper Radiculopathy,PAINFUL NEUROPATHIES: Differential Diagnosis,3 Update on Painful peripheral neuropathy,中华神经科杂志，2012,3 Update on Painful peripheral neuropathy,病因分类代谢和营养障碍外伤和压迫免疫介导感染药物和中毒肿瘤相关隐源性,临床表现症状疼痛体征感觉障碍运动障碍腱反射异常自主神经功能异常,3 Update on Painful peripheral neuropathy,评分VAS现时疼痛强度评分SF-MPQ临床表现评定量表神经经病理性疼痛自评量表Leeds神经病理性痛症状和体征评价表DN4,辅助检查电生理NCVEMGSSRQST自主神经功能活检影像血液、CSF,3 Update on Painful peripheral neuropathy,诊断流程,治疗用药,European Journal of Neurology2010,常见痛性周围神经病的用药推荐,PAIN 2013,3 Update on Painful peripheral neuropathy,其它指南,.uk/CG173,Clin Proc. 2010,小纤维神经病诊断进展周围神经活检技术进展痛性周围神经病诊治规范化周围神经病专病中心建设,4 周围神经病专病中心,不同人群发病率2-3%55岁以上发病率8%早期治疗阻止疾病进展100种病因诊断困难反复就诊，增加就医费用，浪费医