周围神经病PPT精品医学课件.ppt

周围神经病PeripheralNeuropathy Peripheralneuropathyisalargegroupofdiseases Damageofnervesoutsideofthespinalcordandbrain Dysfunctionofthesenerves Causedbyanyofriskfactors Anatomyandfunctionofperipheralnerve basictounderstandneuropathy Anatomycranialnerve olfactorynerveopticnerveoculomotornervetrochlearnervetrigeminalnerveabducentnervefacialnervevestibulocochlearnerveglossopharyngealnervevagusnerveaccessorynervehypoglossalnerve Spinalnerves Histology Electrophysiology UnmyelinatednervefiberMyelinatednervefiber Functions MotornerveSensorynerveAutonomicnervereflex Step1 Clinicalfeaturesofneuropathies historyandexaminationofpatientisthecluestodiagnosisofneuropathy Muscleweakness Weaknessinthearmsorlegs difficultywalking running climbstairs Stumblingortiringeasily Difficultieswithcarryingaloadofgroceries openingjars turningdoorknobs orcombinghair Cranialnerveparalysis ophthalmoplegia facialweakness dysarthria RespiratoryinsufficientMuscleatrophy Reflexlost tips LowermotorneurogenicdamageAnatomicdistributionSymmetricproximaldistalAsymmetricsinglenervemultiplenerveplexusnerveroot Sensoryabnormalities ParesthesiasSpontaneoussensations numbness tingling pinsandneedles prickling burning cold pinching sharpdeepstabs andelectricshocksorbuzzing DysesthesiasUnpleasantabnormalsensationsbroughtonbytouchingorotherstimuli Anesthesiaalackofsensation leadingtoinjuries cutsandburnsduetothelackofnormalwarningsensations alackofsensationofposition whichleadstouncoordinatedandunsteadywalking tips Hearingpatient sdescriptionAnatomicdistributionSymmetric GloveandStocking distributionAsymmetricsinglenervemultiplenerveplexusnerveroot AutonomicNerveDamage blurredvisiondecreasedabilitytosweat anhidrosis decreasedabilitytoregulatebodytemperaturedisturbancesofbladderfunctionorthostatichypotensionConstipationdiarrhea nauseaaftermeals abdominalbloatingsexualdysfunction impotence thinningoftheskin witheasybruisabilityandpoorhealing Step2 Laboratoryfindings nerveconductionstudycanconfirmneuropathyanddistinguishbetweendamagetoaxonsorthemyelinsheath electromyographydifferentiatesmyogenic muscletissue fromneurogenic nervetissue causesofweaknessandcanconfirmabnormalneuromuscularjunctions nervebiopsyisneededonlywhenothertestsareinconclusive Step3 causesDisease 炎症性 免疫介导型神经病中毒性神经病维生素缺乏遗传性神经病神经病伴肿瘤异常球蛋白血征相关的神经病感染相关的神经病系统性疾病外伤切割 挤压 嵌压 神经病伴肿瘤肿瘤的远隔效应肿瘤直接浸润异常球蛋白血征相关的神经病感染相关的神经病HIV麻风Lyme病带状疱疹 系统性疾病糖尿病神经病甲状腺功能低下肾脏 肺 肝脏衰竭危重病神经病器官移植相关神经病外伤切割 挤压 嵌压 Pathomechanism Walleriandegenerationaxonaldegenerationneuronaldegenerationsegmentaldemyelinationonion bulbformationInterstitialneuropathyVasculiticneuropathy 周围神经病的临床病理分类 对称性广泛性多神经病四肢远端对称性感觉运动障碍和皮肤植物神经功能异常 电生理检查 广泛神经传导异常和失神经电位 常见病因 中毒 代谢 遗传 免疫 结缔组织病等 单神经病 多发单神经病单个神经支配区域感觉异常 局部肌肉无力萎缩 非对称性 电生理检查 单神经常见原因 外伤 血管病 感染 肿瘤浸润 结缔组织病 甲低 等 吉兰 巴雷综合征Guillain Barresyndrome Guillain Barresyndrome 1859年Landry sAcendingparalysis1892年acutefebrilepolyneuroritis1918年acuteinfectivepolineuritis1916年Guillain Barre Strohlsyndrome PathologicchangesofAIDP Mechanism AntecedenteventsforGBS InfectionsvirusesE BvirusCytomegalovirusHIVInfuenzavirusCoxsackievirusesHerpessimplexHepatitisAandCvirusesOthers BacterialinfectionCampylobacterjejuniMycoplasmapneumoniacEscherichiacoliOthersParasticMarariaToxoplasmosis AntecedenteventsforGBS SystemicillnessesHodgkin sdiseaselymphocyticleukemiaHyperthoidismCollagenvasculardiseasesSarcoidosisRenaldisease OthermedicalconditionPregnancySurgicalproceduresBonemarrowtransplantationImmunizationsEnvenomizationDrugingestion ImmunopathologyMechanismmolecularmimicry ClassificationofGBS AIDPAcuteInflammatoryDemyelinatingPolyneuropathy MillerFishersyndromeAcutepanautonomicneuropathy APN acutesensoryneuropathy ASN AMAN AMSANAcuteMotor SensoryAxonalNeuropathy ConceptofGBS AcuteorsubacuteonsetPeripheralneuropathyalbuminocytologicdissociationinCSFImmune mediatedneuropathyspontaneousrecoveryinmostcasesMonophasiccourse Epidemiology Incidence1 2casesper100 000generalpopulationAllages meanageis40yAllracesandnationalitiesAslightmalepredominance ClinicalfeaturesofAIDP 1 50 haveparesthesiasandpainatbeginningfollowedbyweaknessmostbegininthelegs10 begininthearmrarelybeginsinthefaceweaknessmayinvolve proximaloflowerlimbsupperlimbs facialmuscle pharyngealandneckmuscle respiratorymuscles 1 3ofpatientsrequireventilator ClinicalfeaturesofAIDP 2 3 5 havecompleteophthalmoplegia15 haveautonomicmanifestationlabilebloodpressure cardiacarrhythmias bladderdysfunction constipation abdominaldistension bloating Neurologicexamination Quadriplegia MuscleweaknessandatrophyMusclestretchreflexesareabsentordepressedCranialnerveparalysisSensorylossinastocking glovedistributionPaininlowback buttocks thighExacerbatedbystraightlegraisingAutonomicdysfunction CSF 90 ofcases proteiniselevatedbythenadirofillnesswithoutleukocytosis 5 ofcases pleocytosis 10 10 20 cells mm3 OB oligoclonalbands IgG 24hincreasingMBPincreasingSpecialAntibodies GM1 Electro diagnosticstudies AIDP Demyelination Axonal AMAN AMSAN Axonal Demyelination Suralnervebiopsy demyelinatingandremyelinatingaxonaldegenerationInfiltrationofinflammatorycells lymphocytes macrophagesdepositionofIgandcomplements Course MostpatientsofAIDPbecomemaximallyweakwithin11 12daysofonset AMSNandAMANusuallyreachnadirwithin6days Mostpatientsprogresssteadily occasionally stutteringorstepwisecourse Theaveragetimetoonsetofrecoveryis4weeks Diagnosis Clinicalfeature acuteprogressivequadriplegiaCSF dissociationofprotein leukocytesF wave NCV EMG MEPSuralnervebiopsySpecialantibodiesinserumandCSF Differentialdiagnosis PeriodicparalysisPoliomyelitisMyelitisToxicneuropathyLyme sdiseaseMyositis TreatmentofGBS 1 SupportcarePreventcomplicationsRehabilitationPsychologysupportimmunotherapy TreatmentofGBS 2 ImmunotherapyPlasmaexchange PE IvIgPE IVIg Corticosteroid IVIg Corticosteroid Prognosis 80 recoverywithin6months15 havesevereresidualdisabilityMortalityrateis3 5 Causeofdeath ARDSwithorwithoutsepsis dysautonomia Chronicinflammatorydemyelinatingpolyradiculoneuritis CIDP SimilarwithAIDPCourses chronicprogressive 2monthsorrelapse remissionPathology demyelination axonaldegeneration onionbulbformationCauses Therapy IVIg plasmaexchange st