七年制医学课件 神经病学 13Peripheral neuropathy

PeripheralNeuropathy

周围神经病

SeeP116-118,P127-129YANYONGDep.ofNeurology,the1stHospital,ChongqingUniversityofMedicalSciencePREFACE

PeripheralNeuropathy(PN)

Cranialn.Spinaln.(Tenpairs)Mononeuropathy

Polyneuropathy

asingleseveralPN

numerousPN

ulnarn.ulnarn.+terminalradicalradialn.radialn.Nervesmedialn.（末梢性)(根性)

peronealn.

tibialisn.

PeripheralNeuropathy(PN)脑神经Cranialnerves.

除嗅、视神经外的十对脑神经，最常见的有

三叉神经、眼球运动神经、面神经、舌咽和舌下神经脊神经Spinaln.

单神经病Mononeuropathy(symplex/multiplex)

：

尺神经、桡神经、正中神经、腓总神经、胫神经…

多发性神经病Polyneuropathy

：

Terminalnerves末梢神经炎、周围神经炎,感染、中毒…各种原因

感染后免疫介导性病变：

急性炎症性脱髓鞘性多发性神经病（Guillain-Barresyndrome,GBS/AIDP）;

慢性炎症性脱髓鞘性多发性神经病（CIDP）;Distalaxonopathies远端轴索病；

Myelinopathies髓鞘病

CausesofperipheralneuropathyIdiopathicinflammatoryneuropathiesAcuteinflammatorydemyelinating

polyneuropathy(AIDP,oracuteIdiopathicpolyneuropathy,Guillain-Barresyndrome),Chronicinflammatorydemyelinating

polyneuropathy(CIDP)MetabolicandnutritionalneuropathiesDiabetes;Otherendocrinopathies:hypothyroidism,acromegaly肢端肥大Uremia;Liverdisease;VitaminB12deficiencyInfectiveandgranulomatous肉芽肿

neuropathiesAIDS;Leprosy麻风;Diphtheria白喉Sarcoidosis结节病;Sepses脓毒症

andmultiorganfailureVasculiticneuropathiesPolyarteritis

nodosa结节性多动脉炎;Rheumatoidarthritis;Systemiclupuserythematousus系统性红斑狼疮(SLE)NeoplasticandparaproteinemicneuropathiesCompressionandinfiltrationbytumor;Paraneoplasticsyndromes副肿瘤综合征;paraproteinemiasAmyloidosis淀粉样变性Drug-inducedandtoxicneuropathiesAlcohol;otherdrugsToxins:Organiccompounds:Hexacarbons;organophosphates;Heavymetals:Arsenic;lead;Thallium;Gold;Platinum

Tryptophan(contaminant)HereditaryneuropathiesIdiopathic:

Hereditarymotorandsensoryneuropathies;Hereditarysensoryneuropathies遗传性感觉神经病

Friedreich’sataxia;Famillial

amyloidosis淀粉样变性

Metabolic:

Porphyria卟啉病

Metachromatic

leukodystrophy

Krabbe’sdiseaseAbetalipoproteinemia

Refsum’sdiseaseFabry’sdiseaseEntrapment压迫或嵌压性

neuropathiescarpaltunnelsyndrome腕管综合征

PATHOLOGYofPND1.Walleriandegeneration华勒变性2.Axonaldegeneration轴突变性3.Segmentaldemyelination节段性脱髓鞘ThefeaturesofPNdamagearethedysfunctionofmotor,sensoryandautonomicnerves.motor(weakness

or

paralysis)sensory

(pain,paresthesiaoranesthesiawithstocking-and-glovepatternindistaloflimbs)autonomic(tachycardia,cardiacIrregu-larities,labilebloodpressure,disturbedsweating)

canbeseeninPNdisorders,

butsphincterdisturbancearerare.

Acuteinflammatorydemyelinating

polyradiculoneuritis

(AIDP)(急性炎症性脱髓鞘性多发性神经病，

P127)Anothernames:Acuteinflammatorypolyneuropathy(急性炎症性多发性神经病)Guillain-Barresyndrome(GBS

)

(吉兰-巴雷、格林-巴利综合征)Acuteidiopathicpolyneuropathy(急性特发性多发性神经病)

GBSisanacuteorsubacuteonsetof

generallysymmetricalandprogressive

lowermotorneuronsparalysis(LMNP)oflimbsandcranialnerves.It’sarealradicalneuropathies.itcanfollowminorinfectiveillnesses,inoculations接种,orsurgicalproceduresormayoccurwithoutobviousprecipitants.

A.EtiologyofGBSItsprecisecauseisunclear,butitappearstohaveanimmunologicbasis.B.PathologyofGBSBothdemyelinating

脱髓鞘(onanteriorrootsandperipheralnerves)andaxonalforms轴索型(somewithaxonaldegenerationinCNS).

C.Clinicalmanifestations

1.Weaknessissymmetricallyusuallybeginsinthelegs,isoftenmoremarkedproximallythandistallylowermotorneuronslesion(hypotonia,hyporeflexia,wastingofaffectedmuscles),between10and14days.Thedeeptendonreflexesaretypicallyabsent.Thesevercasesisthemusclesofrespiratoryareinvolved.Therespiratorymusclespalsymaycauserespiratoryfailurethatislifethreatening!

2.Cranialnervesinvolvement:TheⅦth,oculomotornerves(Ⅲ,ⅣandⅥ),Ⅸ,ⅩandⅪmaybeinvolvedandproducedfacialpalsy,ophthalmologic,andbulbarpalsywhichpredisposetoaspirationpneumonia.

oculomotor动眼神经,trochlear滑车,abducent外展,

glossopharygeal舌咽,vagus迷走,accessory副神经

3.Autonomic

dysfunction自主神经功能障碍:

Theremaybemarkedautonomicdysfunction,withtachycardia心动过速,cardiacirregularities,labilebloodpressure,disturbedsweating,butsphincterdisorder(fecalorurinaryincontinence)arerare.

4.Sensoryinvolvement:

Distalandsymmetrical,asglove-and-stockingsensoryloss,itusuallylessmarkedthanmotorsymptoms,butfrequentalso.

5.OtherclinicaltypesMiller-Fishersyndrome

It’sasubtypeofGBS.

Ataxia共济失调,

hyporeflexiaandoculomotorparalysis;notpalsyoflimbs;increasedproteinconcentrationinCSF;slowlywithNCV神经传导速度.

三个主要特征;共济失调、反射降低和眼球运动障碍。肢体无瘫痪。脑脊液蛋白增高。神经传导速度降低。Acuteaxonmotorneuropathy

It’saspecialsubtypeofGBS.InnorthernChinaarelatedaxonalformoccursfrequentlyandhasbeendesignatedacutemotoraxonalneuropathy.

Thereareweaknessoflimbs(LMN)andwastingofaffectedmusclesintheearlystageofcourse.TheNCVarenormalusually.

D.Investigativestudies

1.CSF:

A

characteristicabnormalitywithincreasedproteinconcentrationafter1week,butobviousin2-3weeks；CSFwhitebloodcellcount≤10/mm3）(ie,CSFalbuminocytologicdissociation

蛋白-细胞分离);

2.Electrophysiologic神经电生理studiesmayrevealmarkedslowingofmotorconductionvelocity(MCV

运动传导速度)andsensoryconductionvelocity(SCV

感觉传导速度),orevidenceofdenervation失神经

andaxonalloss.

TherearesomereducedorlosswithFwavesandHreflex

intheearlystageofcourse

在疾病的早期阶段就可出现F波和H反射消失或减慢.ThemainclinicalfeaturesofGBS(AIDP):①acuteorsubacuteonset;②Generallysymmetrical

progressive

lowermotorneuronsparalysis(LMNP)oflimbsandcranialnerves;

③Weaknessusuallybeginsinthelegs,moremarkedproximallythandistallylowerneuronslesion(hypotonia,hyporeflexia,wastingofaffectedmuscles);

④TheⅦth,oculormotornerves(Ⅲ,ⅣandⅥ),Ⅸ,ⅩandⅪ

cranialnervesmaybeinvolved;⑤TheFwavesandHreflex

reducedorloss,andslowingofMCVandSCV,

buttheNCVmaynotrevealmarkedslowingintheearlystageofcourse⑥CSFalbuminocytologicdissociation.

E.DiagnosisThefeaturesusefulfordiagnosingGBSaresummarizedinbelowtable.

1.Requiredfordiagnosis

Progressiveweaknessofmorethanonelimb；一个以上肢体进行性无力

Distalareflexiawithproximalareflexiaor

hyporeflexia

腱反射消失或降低(肢体远端和近端2.SupportiveofdiagnosisProgressiveforupto4weeks;

病情进展可达4周Relativelysymmetricdeficits;症状和体征相对对称Mildsensoryinvolvement;轻度感觉障碍Cranialn.(especiallyⅦ)involvement;颅神经(尤以面神经)麻痹Recoverybeginningwithin4wksafterprogressivestops

病情停止进展后在4周内开始恢复Autonomicdysfunction;植物神经功能紊乱Nofeveratonset

发病时无发热IncreasedCSFproteinafter1week,butobviousin3weeksCSFwhitebloodcellcount≤10/mm3

脑脊液蛋白1周后增高但3周后达高峰,白细胞数≤10/mm3Nerveconductionslowingorblockbyseveralweeks.

神经传导速度减慢或在数周内消失AgainstdiagnosisMarkedlyasymmetricweakness

显著的不对称性Bowelorbladderdysfunction(atonsetorPersistent)直肠或膀胱功能紊乱(发病时或病程中)CSFwhitebloodcellcount>50

脑脊液中白细胞大于50Well-demarcatedsensorylevel

明显的感觉平面ExcludingdiagnosisIsolatedsensoryinvolvement

单独的感觉受损Anotherpolyneuropathythatexplainsclinicalpicture

Differentialdiagnosis

1.MG重症肌无力:

ThemostimportantsymptomofMGisthefatigability,causingfluctuatingweaknesswhichisworseafterexerciseandusuallyattheendoftheday.Edrophonium(Tensilon)testispositive.2.Hypokalemicperiodicparalysis(低钾性周期性麻痹SeeP346):Periodicepisode,

hypokalemia

andabnormalofECG心电图.3.CIDP慢性炎症性脱髓鞘性多发性神经病

(SeeP129):

CIDPisclinicallysimilartoGBSexceptthatitfollowsachronicprogressivecourseover2monthsoracoursecharacterizedbyrelapses,andnoimprovementisapparentwithinthe6monthsafteronset.

F.

TreatmentIntravenousimmunoglobulin

(IVIG,静脉用免疫球蛋白)

appearstobeequallyeffectiveandshouldbeusedinpreferencetoplasmapheresisinadultswithcardiovascularinstabilityandinchildren;thetwotherapiesarenotadditive.2.

Plasmapheresis(血浆置换)

appearstoreducethetimerequiredforrecoveryandmaydecreasethelikelihoodofresidualneurologicdeficits.

3.Symptomatictherapy对症治疗

isneed.

VitamineB1,B12,VitamineC,Topreventrespiratoryfailureandvascularcollapse,whenthepatienthasshortbreath,whoareseverelyaffectedarebestmanagedintheintensivecareunits(ICU),orthebloodoxygensaturationdeclinessometimestracheotomy气管切开isnecessaryforthepatientwithrespiratorycanalblotchedbysecretionorsputum,andtheassistantrespiratorymachineisnecessaryforthepatientwithrespiratoryfailure呼吸衰竭

.

Corticosteroids

arenotindicated

becauseitmayaffecttheoutcomeadverselyordelayrecovery.G.Prognosis

Itisself-limitingandceasetoprogressbyabout4weeks,improvementoccursoverthewksormonsfollowingonset.

AtypicalcaseAwoman,30yrsold.Shewasadmittedinhospitalwithsymmetricallyweaknessandparesthesiaofarmsandlegsforoneday.It’snormalofsphincter.TheexaminationofNSshowedthat:wakefulness,alitterdysarthria,bilateralperipheralfacialpalsy.Therearelow-gradeofmuscletoneandlossoftendonreflexonfourlimbs.Thereareanalgesiaanddysaphiabelowthewristsandanklesalso.Themuscularstrengthofarmsis2gradeand1gradeonlegs.Thepathologicreflexesaredeficit.Therearemusclepainortendernessofthebilateralcalves.