PeripheralNeuropathy.ppt

周围神经病PeripheralNeuropathyDepartmentofNeurology,The2ndaffiliatedhospital,HarbinMedicalUniversity,PeripheralNeuropathy,OutlineGuillain-BarreSyndromeIdiopathicfacialpalsyTrigeminalneuralgia,OUTLINE,AnatomyPathologicalProcessesClinicalclassifationSymptomsInvestigation,Anatomy,Peripheralnervesaremadeupofnumerousaxonsboundtogetherbythreetypesofconnectivetissueendoneurium,perineurium,epineurium.Thevasalocatedintheepineuriumprovidesthebloodsupply.(fig.1)PeripheralnervetrunkscontainmyelinatedandunmyelinatedfibresCrinialnerveSpinalnerve,PathologicalProcesses,Walleriandegeneration(fig.2)AxonaldegenerationNeuronaldegenerationSegmentaldemyelination(fig.3),Symptoms,SensorydisturbanceMotordeficitsTendonreflexesAutonomicdisturbancesOthers,Investigation,Nerveconductionvelocity(NCV)Electromyography(EMG)NervebiopsyBloodtestsCSFexamination,TrigeminalNeuralgia三叉神经痛,EtiologyandPathology,Afacialpainsyndromeofunknowncause.Demyelination(fig.4),ClinicalFeatures,Itdevelopsinmiddletolatelife.Itconsistsofsevereparoxysmselectric-shock-likepain,usuallyintheV3andV2divisionofthetrigeminalnerve(fig.5),lastingforseveralsecondsorminuteseachtime.InvolvementofV1divisionorbilateraldiseaseoccursinlessthan5%ofcases.,ClinicalFeatures,Occurrenceduringsleepisrare.Painfreeintervalsmaylastforminutestoweeks,butlong-termspontaneousremissionisrare.Sensorystimulationoftriggerzonesaboutthecheek,nose,ormouthbytouch,cold,wind,talking,orchewingcanprecipitatethepain.,ClinicalFeatures,Ticdouloureux(痛性抽搐)CoursecouldbeperiodicPhysicalexaminationisnormal,DiagnosisandDifferentialDiagnosis,DiagnosisdependingontheclinicalfeaturesDifferentialDiagnosisSecondarytrigeminalneuralgiaToothacheGlosspharyngealneuralgia(舌咽神经痛),Treatment,DrugispreferentialAEDs:carbamazapine0.1bidpophenitoin,clonazepamBaclofen(氯苯氨丁酸)VitaminB12封闭疗法:无水酒精,甘油经皮半月神经节射频电凝疗法手术治疗,特发性面神经麻痹IdiopathicFacialPalsy(Bellspalsy),IdiopathicFacialPalsy(Bellspalsy),Anatomy(fig.6)EtiologyandpathogenesisClinicalfeaturesDiagnosisanddifferentialdiagnosisTreatment,EtiologyandPathology,尚未完全阐明，可有一些激发因素受冷、病毒感染和自主神经不稳神经营养血管收缩而毛细血管扩张组织水肿压迫面神经面神经水肿和脱髓鞘，严重者有轴突变性。,Clinicalfeatures,OnsetOccursinanyage,usuallyunilateral.Paralysis:Progressesover3to72hoursPain(50%):NearmastoidprocessExcesstearing(33%)Other:Hyperacusis;Dysgeusia,Clinicalfeatures,SignsFacialweakness(fig.7)UpperComplete(70%)Stapediusdysfunction(33%):HyperacusisLacrimation:mildlyaffectedinsomepatients,Taste:Noclinicallysignificantchangesinmostpatients.Thereshouldbenosensorylossintheface.Huntsyndrome.Noabnormalitiesbeyondtheterritoryofthefacialnerve.,Clinicalfeatures,PrognosisbetterIncompleteparalysisEarlyimprovementSlowprogressionYoungerage,Clinicalfeatures,PrognosisbetterNormaltasteElectrodiagnostictestsnormal病后10天面神经出现失神经，恢复时间延长,平均需要3个月。一般12月内可逐渐痊愈。,Diagnosisanddiffentialdiagnosis,诊断：根据急性起病的周围性面瘫即可诊断。鉴别：1格林-巴利综合征2各种耳源性面神经麻痹3颅后窝的肿瘤或脑膜炎引起的周围性面瘫,Treatment,原则：改善局部血液循环，减轻面神经水肿，促进功能恢复。Corticosteroids:prednisone60mgqdpodexamethasonVitaminB1,B12BaclofenRehabilitationPhysiotherapy,急性炎症性脱髓鞘性多发性神经病(acuteinflammatorydemyelinatingpolyneuropathies,AIDP),概念,急性炎症性脱髓鞘性多发性神经病(acuteinfla-mmatorydemyelinatingpolyneuropathies,AIDP)又称格林-巴利综合症(Guillain-BarrSyndromeGBS),是以周围神经和神经根的脱髓鞘及小血管周围淋巴细胞及巨噬细胞的炎性反应为病理特点的自身免疫病。,Epidemiology,Incidence:0.6to1.9/100,000/yearMale:Female=1.25:1Peakages:1625yearsold4560yearsoldCertainformsofGBSappeartooccurmorefrequentlyincertainareasofChina.,Etiologyandpathogenesis,Theprecisecauseisunclear.GBSoftenfollowsminorinfectiveillness,inoculationsorsurgicalprocedures.ClinicalandepidemiologicevidencesuggestanassociationwithprecedingCampylobacterJejuni(CJ)infection.(fig.8)ThepathogenesisresemblesEANMolecularmimicry,Clinicalfeatures,GBSProdromeItoftenfollows1-4weeksafterarespiratoryinfectionordiarrhea.Campylobacterjejuni(CJ)hasbeenparticularlyimplicatedasacauseofthediarrhea.,Clinicalfeatures,Weakness:MostoftensymptomaticinlegsDistribution:Proximal+Distal;SymmetricSeverity:Quadriplegiain30%;Bedboundanother30%Respiratoryfailure.,Clinicalfeatures,Sensory:ususllylessmarkedthanmotorsymptoms.Paraesthesias:Initialsymptomin50%;Eventuallyoccurin70%to90%PainLoss:withclassicglove-and-stockingpatternofsensoryloss,butrarelyoccurs.,Clinicalfeatures,Cranialnerve:,facialweaknessispresentin50%ofcases.Autonomicdysfunctiontachycardia,cardiacirregularitis,labilebloodpresure,disturbedsweatingandsoon.Monophasecourse,Clinicalfeatures,ClinicalclassificationAIDPAMANAMSANFishersyndromeUnclassifiableGBS,Investigations,CSF:acharacteristicabnormality,withincreasedproteinconcentrationbutanormalcellcount.Eletrophysiologicstudiesmarkedslowingofmotorandsensorycondu-ctionvelocity,evidenceofdenervationandaxonalloss.Fwavereflexisdelayedorabsent.Suralnervebiopsy:demyelination,DiagnosticcriteriaforGBS(1),RequiredfordiagnosisProgressiveweaknessofmorethanonelimb.Distalareflexiawithproximalareflexiaorhyporeflexia.,DiagnosticcriteriaforGBS(2),SupportiveofdiagnosisProgressionforupto4weaks.Relativelysymmetricdeficits.Mildsensoryinvolvement.Cranialnerve(especially)involvement.Recoverybeginningwithin4weeksafterprogressionstops.Autonomicdysfunction.Nofeveratonset.IncreasedCSFproteinafter1week.CSFwhitebloodcellcount10/l.Nerveconductionslowingorblockbyseveralweeks.,Differentialdiagnosis,Hypokalemicperiodicparalysis(Hopp)PoliomyelitisMyastheniagravis(MG),Treatment,Assistingrespiration:Patientswhoareseve-relyaffectedarebestmanagedinICUwherefacilitiesareavailableformonitoringandassis-tedrespirationifnecessary.Sometimesantibioticisnecessaryforpreventingrespiratorytractsinfection.肺活量2025ml/kg,动脉氧分压70mmHgSymptomatictherapy:Theaimistopreventsuchcomplicationsasrespiratoryfailureorvascularcollapse.,Treatment,Preventingcomplications:坠积性肺炎褥疮下肢深部静脉血栓、肺栓塞肢体挛缩、畸形吞咽麻痹尿潴留疼痛焦虑及抑郁,Treatment,Etiologicaltherapy:Plasmaexchange(plasmapheresis)Intravenous