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.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
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