多灶性运动神经病讲解.docx

多灶性运动神经病讲解.docx

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多灶性运动神经病讲解

Multifocalmotorneuropathy

1.ClinNeuroradiol.2015Dec;25（4）:

423-5.doi:

10.1007/s00062-014-0364-9.Epub2015

Jan3.

UltrasonographyofMultifocalAcquiredDemyelinatingSensoryandMotorNeuropathy

（MADSAM）.

NeubauerC

（1）,GruberH

（2）,BäuerleJ（3）,EggerK（4）.

Authorinformation:

（1）DepartmentofRadiology,UniversityMedicalCenterFreiburg,HugstetterStr.

55,79106,Freiburg,Germany.

（2）DepartmentofRadiology,InnsbruckMedical

University,Anichstraße35,6020,Innsbruck,Austria.（3）DepartmentofNeurology,

UniversityMedicalCenterFreiburg,BreisacherStr.64,79106,Freiburg,Germany.

（4）DepartmentofNeuroradiology,UniversityMedicalCenterFreiburg,Breisacher

Str.64,79106,Freiburg,Germany.karl.egger@uniklinik-freiburg.de.

PMID:

25556187[PubMed-inprocess]

2.JCrohnsColitis.2015Dec;9（12）:

1174-5.doi:

10.1093/ecco-jcc/jjv137.Epub2015

Jul29.

MultifocalMotorNeuropathyAssociatedwithInfliximab.

RowanCR

（1）,TubridyN

（2）,CullenG

（2）.

Authorinformation:

（1）CentreforColorectalDisease,StVincent'sUniversityHospital,Dublin,

IrelandandSchoolofMedicineandMedicalScience,UniversityCollegeDublin,

Dublin,Ireland.c.rowan2@st-vincents.ie.

（2）CentreforColorectalDisease,St

Vincent'sUniversityHospital,Dublin,IrelandandSchoolofMedicineandMedical

Science,UniversityCollegeDublin,Dublin,Ireland.

BACKGROUND:

Theanti-tumournecrosisfactor[TNF]monoclonalantibody,

infliximab,iscommonlyprescribedinbothulcerativecolitisandCrohn's

disease.Neurologicalsideeffectssuchasopticneuritisarewellrecognised,

althoughnotasfrequentlyseenashypersensitivityandseriousinfections.

CASE:

Wepresentacaseofperipheralneuropathyinayoungmanoninfliximab

therapyforulcerativecolitis.Thispresentedasanasymmetricalandslowly

progressiveweaknessinhisrightupperlimb,severelyimpactingonfunction.

Investigationsconfirmedadiagnosisofmultifocalmotorneuropathy[MMN].This

hasbeenpreviouslydescribedinpatientsreceivinginfliximabfor

rheumatologicalconditions.Theexactmechanismisunclear,buttheneuropathy

respondswelltointravenousimmunoglobulin.Inourcase,infliximabwas

discontinued.Thepatientwastreatedwithimmunoglobinfor5daysandrecovered

rapidly.Mercaptopurinewasinstitutedasmaintanencetherapy,withgoodeffect.

CONCLUSION:

Gastroenterologistsprescribinginfliximabshouldbecognisantof

bothperipheralandcentralneurologicalcomplications,ensuringprompt

withdrawaloftheoffendingagentandappropriatealternativetreatment.

Copyright©2015EuropeanCrohn’sandColitisOrganisation（ECCO）.Publishedby

OxfordUniversityPress.Allrightsreserved.Forpermissions,pleaseemail:

journals.permissions@.

PMID:

26223843[PubMed-inprocess]

3.JAMANeurol.2015Dec1;72（12）:

1510-8.doi:

10.1001/jamaneurol.2015.2347.

TheImportanceofRareSubtypesinDiagnosisandTreatmentofPeripheral

Neuropathy:

AReview.

CallaghanBC

（1）,PriceRS

（2）,ChenKS（3）,FeldmanEL

（1）.

Authorinformation:

（1）DepartmentofNeurology,UniversityofMichigan,AnnArbor.

（2）Departmentof

Neurology,UniversityofPennsylvania,Philadelphia.（3）Departmentof

Neurosurgery,UniversityofMichigan,AnnArbor.

IMPORTANCE:

Peripheralneuropathyisaprevalentconditionthatusuallywarrants

athoroughhistoryandexaminationbuthaslimiteddiagnosticevaluation.

However,rarelocalizationsofperipheralneuropathyoftenrequiremoreextensive

diagnostictestinganddifferenttreatments.

OBJECTIVE:

Todescriberarelocalizationsofperipheralneuropathy,includingthe

appropriatediagnosticevaluationandavailabletreatments.

EVIDENCEREVIEW:

ReferenceswereidentifiedfromPubMedsearchesconductedonMay

29,2015,withanemphasisonsystematicreviewsandrandomizedclinicaltrials.

Articleswerealsoidentifiedthroughtheuseoftheauthors'ownfiles.Search

termsincludedcommonrareneuropathylocalizationsandtheircauses,aswellas

epidemiology,pathophysiology,diagnosis,andtreatment.

FINDINGS:

Diffuse,nonlength-dependentneuropathies,multiplemononeuropathies,

polyradiculopathies,plexopathies,andradiculoplexusneuropathiesarerare

peripheralneuropathylocalizationsthatoftenrequireextensivediagnostic

testing.Atypicalneuropathyfeatures,suchasacute/subacuteonset,asymmetry,

and/ormotorpredominantsigns,arefrequentlypresent.Themostcommondiffuse,

nonlength-dependentneuropathiesareGuillain-Barrésyndrome,chronic

inflammatorydemyelinatingpolyneuropathy,multifocalmotorneuropathy,and

amyotrophiclateralsclerosis.Effectivedisease-modifyingtherapiesexistfor

manydiffuse,nonlength-dependentneuropathiesincludingGuillain-Barrésyndrome,

chronicinflammatorydemyelinatingpolyneuropathy,multifocalmotorneuropathy,

andsomeparaprotein-associateddemyelinatingneuropathies.Vasculiticneuropathy

（multiplemononeuropathy）alsohasefficacioustreatmentoptions,butdefinitive

evidenceofatreatmenteffectforIgManti-MAGneuropathyanddiabetic

amyotrophy（radiculoplexusneuropathy）islacking.

CONCLUSIONSANDRELEVANCE:

Recognitionofrarelocalizationsofperipheral

neuropathyisessentialgiventheimplicationsfordiagnostictestingand

treatment.Electrodiagnosticstudiesareanimportantearlystepinthe

diagnosticevaluationandprovideinformationonthelocalizationand

pathophysiologyofnerveinjury.

PMID:

26437251[PubMed-inprocess]

4.Neurotherapeutics.2015Nov24.[Epubaheadofprint]

ImmunotherapyinPeripheralNeuropathies.

LégerJM

（1）,Guimarães-CostaR

（2）,MunteanC

（2）.

Authorinformation:

（1）NationalReferralCenterforRareNeuromuscularDiseases,Institut

Hospitalo-UniversitairedeNeurosciences,UniversityHospitalPitié-Salpêtrière

andUniversityPierreetMarieCurie（ParisVI）,Paris,France.

jean-marc.leger@aphp.fr.

（2）NationalReferralCenterforRareNeuromuscular

Diseases,InstitutHospitalo-UniversitairedeNeurosciences,UniversityHospital

Pitié-SalpêtrièreandUniversityPierreetMarieCurie（ParisVI）,Paris,France.

Immunotherapyhasbeeninvestigatedinasmallsubsetofperipheralneuropathies,

includinganacuteone,Guillain-Barrésyndrome,and3chronicforms:

chronic

inflammatorydemyelinatingpolyradiculoneuropathy,multifocalmotorneuropathy,

andneuropathyassociatedwithIgManti-myelin-associatedglycoprotein.Several

experimentalstudiesandclinicaldataarestronglysuggestiveofan

immune-mediatedpathogenesis.Eithercell-mediatedmechanismsorantibody

responsestoSchwanncell,compactmyelin,ornodalantigensareconsideredto

acttogetherinanaberrantimmuneresponsetocausedamagetoperipheralnerves.

Immunomodulatorytreatmentsusedintheseneuropathiesaimtoactatvarious

stepsofthispathogenicprocess.However,therearemanyphenotypicvariants

and,consequently,thereisasignificantdifferenceintheresponseto

immunotherapybetweentheseneuropathies,aswellasaneedtoimproveour

knowledgeandlong-termmanagementofchronicforms.

PMID:

26602549[PubMed-assuppliedbypublisher]

5.MuscleNerve.2015Nov12.doi:

10.1002/mus.24968.[Epubaheadofprint]

Multifocalmotorneuropathy:

30yearsfromonsettodiagnosis.

Hobson-WebbLD

（1）,DonahueSN

（1）,BeyRD

（2）.

Authorinformation:

（1）DepartmentofNeurology,DukeUniversityMedicalCenter,Durham,NC.

（2）NovantHealthWinstonNeurology,Winston-Salem,NC.

PMID:

26562827[PubMed-assuppliedbypublisher]

6.JNeurolNeurosurgPsychiatry.2015Nov;86（11）:

1234-9.doi:

10.1136/jnnp-2014-308589.Epub2014Dec24.

Diagnosticaccuracyofelectricallyelicitedmultipletdischargesinpatients

withmotorneurondisease.

SleutjesBT

（1）,MontfoortI

（1）,vanDoornPA

（2）,VisserGH（3）,BlokJH（4）.

Authorinformation:

（1）DepartmentofClinicalNeurophysiology,ErasmusMC,UniversityMedicalCentre

Rotterdam,Rotterdam,TheNetherlands.

（2）DepartmentofNeurology,ErasmusMC,

UniversityMedicalCentreRotterdam,Rotterdam,TheNetherlands.（3）Departmentof

ClinicalNeurophysiology,ErasmusMC,UniversityMedicalCentreRotterdam,

Rotterdam,TheNetherlandsDepartmentofClinicalNeurophysiology,SEIN

Heemstede,Heemstede,TheNetherlands.（4）DepartmentofClinicalNeurophysiology,

ErasmusMC,UniversityMedicalCentreRotterdam,Rotterdam,TheNetherlands

DepartmentofClinicalPhysics,MáximaMedicalCentre,Veldhoven,The

Netherlands.

OBJECTIVE:

Todetermineandcomparethediagnosticaccuracyofelectrically

elicitedmultipletdischarges（MDs）andfasciculationpotentials（FPs）inmotor

neurondisease（MND）.

METHODS:

PatientswereeligiblewhentheyhadMNDintheirdifferentialdiagnosis

andwerereferredforelectromyogram（EMG）.Stimulatedhigh-densitysurfaceEMG

ofthethenarmuscleswasperformedonthesamedayasstandardEMGexamination.

High-densityrecordingswereanalysedforpresenceofMDsandneedleEMGofany

muscleinvestigatedinthecervicalregionforpresenceofFPs.

RESULTS:

Ofthe61patientsenrolledinthisdiagnosticstudy,24patientswere

clinicallydiagnosedwithamyotrophiclateralsclerosis（ALS）and11patients

withprogressivemuscularatrophy（PMA）.Anotherdiagnosiswasmadein26

patients.SixteenpatientsinwhomMDsweredetectedwerediagnosedwitheither

ALS（n=11）orPMA（n=5;sensitivity=47.1%,PPV=94.1%）.MDsweredetectedinonly

onepatientinitiallydiagnosedwithPMA,butinwhomlateron,multifocalmotor

neuropathycouldnotbeexcluded（specificity=96.2%）.ElectricallyelicitedMDs

hadahigherspecificitythanFPs（96.2%vs53.9%,p<0.001,n=26）andlower

sensitivity（47.1%vs85.3%,p=0.002,n=34）.WhenconsideringpresenceofMDsin

MNDasneurogenicEMGabnormality,lowermotorneuroninvolvementof≥1E