PeripheralNeuropathy

周圍神經病

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

周圍神1PREFACE

PeripheralNeuropathy(PN)

Cranialn.Spinaln.(Tenpairs)Mononeuropathy

Polyneuropathy

asingleseveralPN

numerousPN

ulnarn.ulnarn.+terminalradicalradialn.radialn.Nervesmedialn.（末梢性)(根性)peronealn.tibialisn.PREFACEPeripher2PeripheralNeuropathy(PN)腦神經Cranialnerves.

除嗅、視神經外的十對腦神經，最常見的有三叉神經、眼球運動神經、面神經、舌咽和舌下神經脊神經Spinaln.

單神經病Mononeuropathy(symplex/multiplex)

：尺神經、橈神經、正中神經、腓總神經、脛神經…

多發性神經病Polyneuropathy

：

Terminalnerves末梢神經炎、周圍神經炎,感染、中毒…各種原因

感染后免疫介導性病變：

急性炎癥性脫髓鞘性多發性神經病（Guillain-Barresyndrome,GBS/AIDP）;

慢性炎癥性脫髓鞘性多發性神經病（CIDP）;Distalaxonopathies遠端軸索病；Myelinopathies髓鞘病Peripheral3CausesofperipheralneuropathyIdiopathicinflammatoryneuropathiesAcuteinflammatorydemyelinatingpolyneuropathy(AIDP,oracuteIdiopathicpolyneuropathy,Guillain-Barresyndrome),Chronicinflammatorydemyelinatingpolyneuropathy(CIDP)MetabolicandnutritionalneuropathiesDiabetes;Otherendocrinopathies:hypothyroidism,acromegaly肢端肥大Uremia;Liverdisease;VitaminB12deficiencyInfectiveandgranulomatous肉芽腫neuropathiesAIDS;Leprosy麻風;Diphtheria白喉Sarcoidosis結節病;Sepses膿毒癥andmultiorganfailureCausesofperipheralneuropa4VasculiticneuropathiesPolyarteritisnodosa結節性多動脈炎;Rheumatoidarthritis;Systemiclupuserythematousus系統性紅斑狼瘡(SLE)NeoplasticandparaproteinemicneuropathiesCompressionandinfiltrationbytumor;Paraneoplasticsyndromes副腫瘤綜合征;paraproteinemiasAmyloidosis淀粉樣變性Drug-inducedandtoxicneuropathiesAlcohol;otherdrugsToxins:Organiccompounds:Hexacarbons;organophosphates;Heavymetals:Arsenic;lead;Thallium;Gold;PlatinumTryptophan(contaminant)Vasculiticneuropathies5HereditaryneuropathiesIdiopathic:

Hereditarymotorandsensoryneuropathies;Hereditarysensoryneuropathies遺傳性感覺神經病Friedreich’sataxia;Famillialamyloidosis淀粉樣變性Metabolic:Porphyria卟啉病MetachromaticleukodystrophyKrabbe’sdiseaseAbetalipoproteinemiaRefsum’sdiseaseFabry’sdiseaseEntrapment壓迫或嵌壓性neuropathiescarpaltunnelsyndrome腕管綜合征Hereditaryneuropathies6

PATHOLOGYofPND1.Walleriandegeneration華勒變性2.Axonaldegeneration軸突變性3.Segmentaldemyelination節段性脫髓鞘PATHOLOGYofPND7ThefeaturesofPNdamagearethedysfunctionofmotor,sensoryandautonomicnerves.motor(weakness

or

paralysis)sensory

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

canbeseeninPNdisorders,

butsphincterdisturbancearerare.ThefeaturesofPNdamageare8

Acuteinflammatorydemyelinatingpolyradiculoneuritis

(AIDP)(急性炎癥性脫髓鞘性多發性神經病，

P127)Anothernames:Acuteinflammatorypolyneuropathy(急性炎癥性多發性神經病)Guillain-Barresyndrome(GBS

)

(吉蘭-巴雷、格林-巴利綜合征)Acuteidiopathicpolyneuropathy(急性特發性多發性神經病)

Acuteinflammatorydemyelinat9GBSisanacuteorsubacuteonsetof

generallysymmetricalandprogressive

lowermotorneuronsparalysis(LMNP)oflimbsandcranialnerves.It’sarealradicalneuropathies.itcanfollowminorinfectiveillnesses,inoculations接種,orsurgicalproceduresormayoccurwithoutobviousprecipitants.GBSisanacuteorsu10

A.EtiologyofGBSItsprecisecauseisunclear,butitappearstohaveanimmunologicbasis.B.PathologyofGBSBothdemyelinating

脫髓鞘(onanteriorrootsandperipheralnerves)andaxonalforms軸索型(somewithaxonaldegenerationinCNS).

11

C.Clinicalmanifestations

1.Weaknessissymmetricallyusuallybeginsinthelegs,isoftenmoremarkedproximallythandistallylowermotorneuronslesion(hypotonia,hyporeflexia,wastingofaffectedmuscles),between10and14days.Thedeeptendonreflexesaretypicallyabsent.Thesevercasesisthemusclesofrespiratoryareinvolved.Therespiratorymusclespalsymaycauserespiratoryfailurethatislifethreatening!C.Clinicalmanifestatio12

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

oculomotor動眼神經,trochlear滑車,abducent外展,glossopharygeal舌咽,vagus迷走,accessory副神經2.Cranialnervesinvolvemen13

3.Autonomic

dysfunction自主神經功能障礙:

Theremaybemarkedautonomicdysfunction,withtachycardia心動過速,cardiacirregularities,labilebloodpressure,disturbedsweating,butsphincterdisorder(fecalorurinaryincontinence)arerare.3.Autonomicdysfunction自主神144.Sensoryinvolvement:

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

4.Sensoryinvolvement:155.OtherclinicaltypesMiller-Fishersyndrome

It’sasubtypeofGBS.

Ataxia共濟失調,

hyporeflexiaandoculomotorparalysis;notpalsyoflimbs;increasedproteinconcentrationinCSF;slowlywithNCV神經傳導速度.

三個主要特征;共濟失調、反射降低和眼球運動障礙。肢體無癱瘓。腦脊液蛋白增高。神經傳導速度降低。5.Otherclinicaltypes16Acuteaxonmotorneuropathy

It’saspecialsubtypeofGBS.InnorthernChinaarelatedaxonalformoccursfrequentlyandhasbeendesignatedacutemotoraxonalneuropathy.

Thereareweaknessoflimbs(LMN)andwastingofaffectedmusclesintheearlystageofcourse.TheNCVarenormalusually.Acuteaxonmotorneuropathy17

D.Investigativestudies

1.CSF:

A

characteristicabnormalitywithincreasedproteinconcentrationafter1week,butobviousin2-3weeks；CSFwhitebloodcellcount≤10/mm3）(ie,CSFalbuminocytologicdissociation蛋白-細胞分離);D.Investigativestudie182.Electrophysiologic神經電生理studiesmayrevealmarkedslowingofmotorconductionvelocity(MCV運動傳導速度)andsensoryconductionvelocity(SCV感覺傳導速度),orevidenceofdenervation失神經

andaxonalloss.TherearesomereducedorlosswithFwavesandHreflex

intheearlystageofcourse

在疾病的早期階段就可出現F波和H反射消失或減慢.2.Electrophysiologic神經電19ThemainclinicalfeaturesofGBS(AIDP):①acuteorsubacuteonset;②Generallysymmetrical

progressive

lowermotorneuronsparalysis(LMNP)oflimbsandcranialnerves;

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

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

cranialnervesmaybeinvolved;⑤TheFwavesandHreflex

reducedorloss,andslowingofMCVandSCV,

buttheNCVmaynotrevealmarkedslowingintheearlystageofcourse⑥CSFalbuminocytologicdissociation.

Themainclinicalfeaturesof20

E.DiagnosisThefeaturesusefulfordiagnosingGBSaresummarizedinbelowtable.

1.Requiredfordiagnosis

Progressiveweaknessofmorethanonelimb；一個以上肢體進行性無力

Distalareflexiawithproximalareflexiaorhyporeflexia

腱反射消失或降低(肢體遠端和近端E.Diagnosis212.SupportiveofdiagnosisProgressiveforupto4weeks;

病情進展可達4周Relativelysymmetricdeficits;癥狀和體征相對對稱Mildsensoryinvolvement;輕度感覺障礙Cranialn.(especiallyⅦ)involvement;顱神經(尤以面神經)麻痹Recoverybeginningwithin4wksafterprogressivestops

病情停止進展后在4周內開始恢復Autonomicdysfunction;植物神經功能紊亂Nofeveratonset

發病時無發熱IncreasedCSFproteinafter1week,butobviousin3weeksCSFwhitebloodcellcount≤10/mm3

腦脊液蛋白1周后增高但3周后達高峰,白細胞數≤10/mm3Nerveconductionslowingorblockbyseveralweeks.神經傳導速度減慢或在數周內消失2.Supportiveofdiagnosis22AgainstdiagnosisMarkedlyasymmetricweakness

顯著的不對稱性Bowelorbladderdysfunction(atonsetorPersistent)直腸或膀胱功能紊亂(發病時或病程中)CSFwhitebloodcellcount>50

腦脊液中白細胞大于50Well-demarcatedsensorylevel

明顯的感覺平面ExcludingdiagnosisIsolatedsensoryinvolvement

單獨的感覺受損AnotherpolyneuropathythatexplainsclinicalpictureAgainstdiagnosis23

Differentialdiagnosis

1.MG重癥肌無力:

ThemostimportantsymptomofMGisthefatigability,causingfluctuatingweaknesswhichisworseafterexerciseandusuallyattheendoftheday.Edrophonium(Tensilon)testispositive.Differential242.Hypokalemicperiodicparalysis(低鉀性周期性麻痹SeeP346):Periodicepisode,hypokalemia

andabnormalofECG心電圖.3.CIDP慢性炎癥性脫髓鞘性多發性神經病(SeeP129):

CIDPisclinicallysimilartoGBSexceptthatitfollowsachronicprogressivecourseover2monthsoracoursecharacterizedbyrelapses,andnoimprovementisapparentwithinthe6monthsafteronset.2.Hypokalemicperiodicparalys25

F.

TreatmentIntravenousimmunoglobulin

(IVIG,靜脈用免疫球蛋白)

appearstobeequallyeffectiveandshouldbeusedinpreferencetoplasmapheresisinadultswithcardiovascularinstabilityandinchildren;thetwotherapiesarenotadditive.2.Plasmapheresis(血漿置換)

appearstoreducethetimerequiredforrecoveryandmaydecreasethelikelihoodofresidualneurologicdeficits.F.Treatment26

3.Symptomatictherapy對癥治療

isneed.

VitamineB1,B12,VitamineC,Topreventrespiratoryfailureandvascularcollapse,whenthepatienthasshortbreath,whoareseverelyaffectedarebestmanagedintheintensivecareunits(ICU),orthebloodoxygensaturationdeclinessometimestracheotomy氣管切開isnecessaryforthepatientwithrespiratorycanalblotchedbysecretionorsputum,andtheassistantrespiratorymachineisnecessaryforthepatientwithrespiratoryfailure呼吸衰竭.3.Symptomatictherapy對癥治療i27Corticosteroids

arenotindicated

becauseitmayaffecttheoutcomeadverselyordelayrecovery.G.Prognosis

Itisself-limitingandceasetoprogressbyabout4weeks,improvementoccursoverthewksormonsfollowingonset.Corticosteroidsarenotind28

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

典型病例

某女，30歲，因四肢無力、手和足麻木1天入院，大小便正常。神經系統檢查：神志清楚，語言稍含糊。雙側周圍性面神經麻痹。四肢肌張力低，腱反射消失，雙上肢肌力2級、雙下肢肌力1級。雙上肢腕以下和雙下肢踝以下痛覺觸覺輕度減退。病理反射（－）。雙側腓腸肌壓痛。對此患者，①請你判斷是什么性質的癱瘓（上運動神經元或下運動神經元性癱瘓）？②提出應該完成那些主要的檢查？③最可能的診斷是什么？④應主要與哪些疾病鑒別？endAtypicalcase29

Keypoints1.AlbuminocytologicdissociationinCSF蛋白-細胞分離2.themainclinicalfeaturesofGBS(AIDP):①acuteorsubacuteonset;②Generallysymmetrical

progressive

lowermotorneuronsparalysis(LMNP)oflimbsandcranialnerves;③Weaknessusuallybeginsinthelegs,moremarkedproximallythandistallylowerneuronslesion(hypotonia,hyporeflexia,wastingofaffectedmuscles);④TheⅦth,oculomotornerves(Ⅲ,Ⅳ

andⅥ),Ⅸ,Ⅹ

andⅪ

cranialnervesmaybeinvolved;⑤Therespiratorymusclesinvolvedandpalsymaycauserespiratoryfailureinthesevercases.3.WhatchangesofelectrophysiologicintheGBS(AIDP)?TheFwavesandHreflex

reducedorlossintheearlystageofcoursebuttheNCVmaynotrevealmarkedslowing.4.HowdodifferentialdiagnosiswithotherLMNP?Keypoints30PeripheralNeuropathy

周圍神經病

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

周圍神31PREFACE

PeripheralNeuropathy(PN)

Cranialn.Spinaln.(Tenpairs)Mononeuropathy

Polyneuropathy

asingleseveralPN

numerousPN

ulnarn.ulnarn.+terminalradicalradialn.radialn.Nervesmedialn.（末梢性)(根性)peronealn.tibialisn.PREFACEPeripher32PeripheralNeuropathy(PN)腦神經Cranialnerves.

除嗅、視神經外的十對腦神經，最常見的有三叉神經、眼球運動神經、面神經、舌咽和舌下神經脊神經Spinaln.

單神經病Mononeuropathy(symplex/multiplex)

：尺神經、橈神經、正中神經、腓總神經、脛神經…

多發性神經病Polyneuropathy

：

Terminalnerves末梢神經炎、周圍神經炎,感染、中毒…各種原因

感染后免疫介導性病變：

急性炎癥性脫髓鞘性多發性神經?。℅uillain-Barresyndrome,GBS/AIDP）;

慢性炎癥性脫髓鞘性多發性神經?。–IDP）;Distalaxonopathies遠端軸索病；Myelinopathies髓鞘病Peripheral33CausesofperipheralneuropathyIdiopathicinflammatoryneuropathiesAcuteinflammatorydemyelinatingpolyneuropathy(AIDP,oracuteIdiopathicpolyneuropathy,Guillain-Barresyndrome),Chronicinflammatorydemyelinatingpolyneuropathy(CIDP)MetabolicandnutritionalneuropathiesDiabetes;Otherendocrinopathies:hypothyroidism,acromegaly肢端肥大Uremia;Liverdisease;VitaminB12deficiencyInfectiveandgranulomatous肉芽腫neuropathiesAIDS;Leprosy麻風;Diphtheria白喉Sarcoidosis結節病;Sepses膿毒癥andmultiorganfailureCausesofperipheralneuropa34VasculiticneuropathiesPolyarteritisnodosa結節性多動脈炎;Rheumatoidarthritis;Systemiclupuserythematousus系統性紅斑狼瘡(SLE)NeoplasticandparaproteinemicneuropathiesCompressionandinfiltrationbytumor;Paraneoplasticsyndromes副腫瘤綜合征;paraproteinemiasAmyloidosis淀粉樣變性Drug-inducedandtoxicneuropathiesAlcohol;otherdrugsToxins:Organiccompounds:Hexacarbons;organophosphates;Heavymetals:Arsenic;lead;Thallium;Gold;PlatinumTryptophan(contaminant)Vasculiticneuropathies35HereditaryneuropathiesIdiopathic:

Hereditarymotorandsensoryneuropathies;Hereditarysensoryneuropathies遺傳性感覺神經病Friedreich’sataxia;Famillialamyloidosis淀粉樣變性Metabolic:Porphyria卟啉病MetachromaticleukodystrophyKrabbe’sdiseaseAbetalipoproteinemiaRefsum’sdiseaseFabry’sdiseaseEntrapment壓迫或嵌壓性neuropathiescarpaltunnelsyndrome腕管綜合征Hereditaryneuropathies36

PATHOLOGYofPND1.Walleriandegeneration華勒變性2.Axonaldegeneration軸突變性3.Segmentaldemyelination節段性脫髓鞘PATHOLOGYofPND37ThefeaturesofPNdamagearethedysfunctionofmotor,sensoryandautonomicnerves.motor(weakness

or

paralysis)sensory

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

canbeseeninPNdisorders,

butsphincterdisturbancearerare.ThefeaturesofPNdamageare38

Acuteinflammatorydemyelinatingpolyradiculoneuritis

(AIDP)(急性炎癥性脫髓鞘性多發性神經病，

P127)Anothernames:Acuteinflammatorypolyneuropathy(急性炎癥性多發性神經病)Guillain-Barresyndrome(GBS

)

(吉蘭-巴雷、格林-巴利綜合征)Acuteidiopathicpolyneuropathy(急性特發性多發性神經病)

Acuteinflammatorydemyelinat39GBSisanacuteorsubacuteonsetof

generallysymmetricalandprogressive

lowermotorneuronsparalysis(LMNP)oflimbsandcranialnerves.It’sarealradicalneuropathies.itcanfollowminorinfectiveillnesses,inoculations接種,orsurgicalproceduresormayoccurwithoutobviousprecipitants.GBSisanacuteorsu40

A.EtiologyofGBSItsprecisecauseisunclear,butitappearstohaveanimmunologicbasis.B.PathologyofGBSBothdemyelinating

脫髓鞘(onanteriorrootsandperipheralnerves)andaxonalforms軸索型(somewithaxonaldegenerationinCNS).

41

C.Clinicalmanifestations

1.Weaknessissymmetricallyusuallybeginsinthelegs,isoftenmoremarkedproximallythandistallylowermotorneuronslesion(hypotonia,hyporeflexia,wastingofaffectedmuscles),between10and14days.Thedeeptendonreflexesaretypicallyabsent.Thesevercasesisthemusclesofrespiratoryareinvolved.Therespiratorymusclespalsymaycauserespiratoryfailurethatislifethreatening!C.Clinicalmanifestatio42

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

oculomotor動眼神經,trochlear滑車,abducent外展,glossopharygeal舌咽,vagus迷走,accessory副神經2.Cranialnervesinvolvemen43

3.Autonomic

dysfunction自主神經功能障礙:

Theremaybemarkedautonomicdysfunction,withtachycardia心動過速,cardiacirregularities,labilebloodpressure,disturbedsweating,butsphincterdisorder(fecalorurinaryincontinence)arerare.3.Autonomicdysfunction自主神444.Sensoryinvolvement:

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

4.Sensoryinvolvement:455.OtherclinicaltypesMiller-Fishersyndrome

It’sasubtypeofGBS.

Ataxia共濟失調,

hyporeflexiaandoculomotorparalysis;notpalsyoflimbs;increasedproteinconcentrationinCSF;slowlywithNCV神經傳導速度.

三個主要特征;共濟失調、反射降低和眼球運動障礙。肢體無癱瘓。腦脊液蛋白增高。神經傳導速度降低。5.Otherclinicaltypes46Acuteaxonmotorneuropathy

It’saspecialsubtypeofGBS.InnorthernChinaarelatedaxonalformoccursfrequentlyandhasbeendesignatedacutemotoraxonalneuropathy.

Thereareweaknessoflimbs(LMN)andwastingofaffectedmusclesintheearlystageofcourse.TheNCVarenormalusually.Acuteaxonmotorneuropathy47

D.Investigativestudies

1.CSF:

A

characteristicabnormalitywithincreasedproteinconcentrationafter1week,butobviousin2-3weeks；CSFwhitebloodcellcount≤10/mm3）(ie,CSFalbuminocytologicdissociation蛋白-細胞分離);D.Investigativestudie482.Electrophysiologic神經電生理studiesmayrevealmarkedslowingofmotorconductionvelocity(MCV運動傳導速度)andsensoryconductionvelocity(SCV感覺傳導速度),orevidenceofdenervation失神經

andaxonalloss.TherearesomereducedorlosswithFwavesandHreflex

intheearlystageofcourse

在疾病的早期階段就可出現F波和H反射消失或減慢.2.Electrophysiologic神經電49ThemainclinicalfeaturesofGBS(AIDP):①acuteorsubacuteonset;②Generallysymmetrical

progressive

lowermotorneuronsparalysis(LMNP)oflimbsandcranialnerves;

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

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

cranialnervesmaybeinvolved;⑤TheFwavesandHreflex

reducedorloss,andslowingofMCVandSCV,

buttheNCVmaynotrevealmarkedslowingintheearlystageofcourse⑥CSFalbuminocytologicdissociation.

Themainclinicalfeaturesof50

E.DiagnosisThefeaturesusefulfordiagnosingGBSaresummarizedinbelowtable.

1.Requiredfordiagnosis

Progressiveweaknessofmorethanonelimb；一個以上肢體進行性無力

Distalareflexiawithproximalareflexiaorhyporeflexia

腱反射消失或降低(肢體遠端和近端E.Diagnosis512.SupportiveofdiagnosisProgressiveforupto4weeks;

病情進展可達4周Relativelysymmetricdeficits;癥狀和體征相對對稱Mildsensoryinvolvement;輕度感覺障礙Cranialn.(especiallyⅦ)involvement;顱神經(尤以面神經)麻痹Recoverybeginningwithin4wksafterprogressivestops

病情停止進展后在4周內開始恢復Autonomicdysfunction;植物神經功能紊亂Nofeveratonset

發病時無發熱IncreasedCSFproteinafter1week,butobviousin3weeksCSFwhitebloodcellcount≤10/mm3

腦脊液蛋白1周后增高但3周后達高峰,白細胞數≤10/mm3Nerveconductionslowingorblockbyseveralweeks.神經傳導速度減慢或在數周內消失2.Supportiveofdiagnosis52AgainstdiagnosisMarkedlyasymmetricweakness

顯著的不對稱性Bowelorbladderdysfunction(atonsetorPersistent)直腸或膀胱功能紊亂(發病時或病程中)CSFwhitebloodcellcount>50

腦脊液中白細胞大于50Well-demarcatedsensorylevel

明顯的感覺平面ExcludingdiagnosisIsolatedsensoryinvolvement

單獨的感覺受損AnotherpolyneuropathythatexplainsclinicalpictureAgainstdiagnosis53

Differentialdiagnosis

1.MG重癥肌無力:

ThemostimportantsymptomofMGisthefatigability,causingfluctuatingweaknesswhichisworseafterexerciseandusuallyattheendoftheday.Edrophonium(Tensilon)testispositive.Differential542.Hypokalemicperiodicparalysis(低鉀性周期性麻痹SeeP346):Periodicepisode,hypokalemia

andabnormalofECG心電圖.3.CIDP慢性炎癥性脫髓鞘性多發性神經病(SeeP129):

CIDPisclinicallysimilartoGBSexceptthatitfollowsachronicprogressivecourseover2monthsoracoursecharacterizedbyrelapses,andnoimprovementisapparentwithinthe6monthsafteronset.2.Hypokalemicperiodicparalys55

F.

TreatmentIntravenousimmunoglobulin

(IVIG,靜脈用免疫球蛋白)

appearstobeequallyeffectiveandshouldbeusedinpreferencetoplasmapheresisinadultswithcardiovascularinstabilityandinchildren;thetwotherapiesarenotadditive.2.Plasmapheresis(血漿置換)

appearstoreducethetimerequiredforrecoveryandmaydecreasethelikelihoodofresidualneurologicdeficits.F.Treatment56

3.Symptomatictherapy對癥治療

isneed.

Vi