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1UpdateonSFN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（SkinBiospy）痛觉诱发电位（CHEP）角膜共焦显微镜（CornealConfocalMicroscopy，CCM）1UpdateonSFNCCM原理1UpdateonSFN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1UpdateonSFN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51KallinikosP.InvestOphthalmolVisSci20042MalikRA.Diabetologia20033ElisabethM.GraefesArchClinExpOphthalmol20104HertzP.DiabeticMedicine20115TavakoliM.Muscle&Nerve20091UpdateonSFN对照女性患者（ERT-F）女性杂合子（ERT）男性半和子（ERT）TavakoliM.Muscle&Nerve2009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2UpdateonNervebiopsy传统的神经活检腓肠神经腓浅神经桡浅神经后骨间神经（Posteriornterosseousnerve，PIN）2UpdateonNervebiopsy传统神经活检的缺陷感染疼痛麻木感觉缺失2UpdateonNervebiopsy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2UpdateonNervebiopsy2UpdateonNervebiopsy对照DMDMDM（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3UpdateonPainfulperipheralneuropathyToxicAlcoholMetals:

Thallium;

ArsenicMedications:cis-platinum;

Disulfiram;

Ifosfamide;

Nitrofurantoin;

Perhexiline;

Taxol;

Thalidomide;

VincaalkaloidsMushroom:

ClitocybeacromelalgaImmuneAcuteonsetGuillainBarréDiffusepainduringacutecourseDistalpainwithincompleterecoveryImmobilityAcute

panautonomicIgMantibodyassociatedpolyneuropathiesTS-HDSSulfatideIgGantibodyassociatedpolyneuropathiesPotassiumchannel-complexantibodies:CASPR2&OtherM-proteinGanglionopathiesSjögren'sParaneoplastic(Hu)CryoglobulinemiaConnectivetissuediseaseHIV(DILS)VasculitisHereditaryα-galactosidase(Fabry's)GM2gangliosidosis:Late-onsetSensoryNeuropathyI:LancinatingpainsMotor-SensoryNeuropathyI&II:FootdeformitySCN9ABurningfeetsyndromeSmallfiberneuropathyErythromelalgiaAmyloidosisPolyneuropathy:Pain&ParaesthesiasinlegsCarpaltunnelsyndrome:PaininhandsPorphyriaEpisodicpainsyndromes,Familial

(FEPS)Restlesslegssyndromes:SomewithPNSinvolvementHereditary(Acromelalgia)

:12qHSN1HMSN5CMT2Huntingtondisease-like1

Also:

RenalfailureMetabolicDiabeticAcute:LumbarorThoracicradiculopathy;DistalsensoryChronic:"Smallfiber"neuropathyAlcoholic:AcutePellagra(niacin)Beriberi(thiamine)Strachansyndrome(Cubanneuropathy)PostgastroplastyHypertriglyceridemiaChronicmountainsicknessIdiopathicDistalsensorypolyneuropathysyndromesErythromelalgiaMononeuritismultiplexMotordisorderswithpainDiabeticamyotrophyPolioInfectionsPolioHIVLocalizeddisordersNervelesions:TrigeminalBrachialplexusLumbosacralplexopathyThoracicoutletsyndromeMedianPosteriortibial

(Tarsaltunnelsyndrome)Radiculopathies:

Upper

&

Lower

ExtremitiesBurningmouthsyndromeDiabeticAmyotrophyReflexSympatheticDystrophy

(Complexregionalpainsyndrome)Infections:

HerpeszosterNeoplasticinfiltration:BurningpainwithdysesthesiainareaofsensorylossPostsurgicallocalizedpainsyndromesAcuteonsetofpain&paresthesiasAcuteporphyriaToxic:

Thallium;

Arsenic;

Perhexiline;

VincaalkaloidsGuillain-BarréAcutesensoryneuropathies

Spinal:

Myelopathy;RadiculopathyPAINFULNEUROPATHIES:DifferentialDiagnosis3UpdateonPainfulperipheralneuropathy中华神经科杂志，20123UpdateonPainfulperipheralneuropathy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3UpdateonPainfulperipheralneuropathy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、CSF3UpdateonPainfulperipheralneuropathy诊断流程治疗用药EuropeanJournalofNeurology2010常见痛性周围神经病的用药推荐PAIN20133UpdateonPainfulperipheralneuropathy其它指南.uk/CG173ClinProc.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，浪费医疗资源4周围神经病专病中心周围神经病临床神经电生理神经病理神经免疫基因诊断神经活检皮肤活检神经传导速度针极肌电图特殊检查项目4周围神经病专病中心国际市场占有率最高的电生理设备品牌丹迪K（左）光电MEB9002K（右）检测大纤维以色列Medoc定量感觉分析仪TSA-II检测小纤维4周围神经病专病中心神经活检皮肤活检