肌酸磷酸激酶增高的脊髓性肌萎缩症10例临床与肌电图
肌酸磷酸激酶增高的脊髓性肌萎缩症10例
临床与肌电图分析
seniledementia.However,curativeeffectofsingletherapeutic methodoftonifyingthekidneyisnotidealforexistingmanyetio- lo~calfactorscausingseniledementia.Patholo~calchangesnot onlyinvolvemanyorganssuchasspleen,heartandliver, etc.exceptbrain,butalsoafewpathogenicfactorssuchas bloodstasis,p~egmandtoxicheat,etc.Therefore,newwaysto anti-dementiamustbefoundsothatbreakthroughswiIIbe made.ResearcherswillpaystressonstudyonotherChinesedrugs whichmaybehaveanti-dementiaeffect,forinstance,thedrugsof strengtheningthespleen,drugsusedtopromotebloodcirculation andremovebloodstasis,andheat—clearinganddetoxifyingdrugs,
etc.Inrecentyears,someresearchershavefoundsomevaluable clues【16..".Inaddition,idealanimalmodelisaDrerequisitefor gettiIlgobjectiveconclusionsinthecurativeeffectevaluationof anti-dementiadrugs.?resuggestthatscreeningofanti—dementia
drugsinthe~turemustbeonthebasisofidealdementiaanimal model.11leindexes"ofmorphologyshouldalsobeobservedexcept usualindexessuchastheindexesoflearningandmemo~inaged animals,behaviorchangescausedbychemicalsandalterationof neurotransmissionrelatedtoseniledementia.
REFERENCEs
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di,ferentialdiagnosis,thelevelofplasmacreatinekinase(CK)isan imDonaIltbutnotas~cfficcriterion,andEMGandmusclebiopsy areofreferencevalue.TogetabetterunderstandingofSMA,we havecollectedandanalysedclinicaldata,EMG,andmusclebiopsy fmm10DatientswithSMAassociatedwithelevatedCKwhovisited SichuanPeople'sHospitalbetween1996and2003.
SIIBJECTSANDMETHODS
Subjects
Tenpatients(9malesand1female),aged10monthsto65years withtheaverageageof(34.3?21.9)yearsanddiagnosedasSMA
for3—36months,wererecruitedintothestudy.DiagnosisofSMA wasbasedonclinicalpresentations,EMG,neuroelectrophysiology, musclebiopsyandimaginganalysis.SMAtypeI—IVwasidentified
accordingtotlleclassmedcriteriamadebyWorldFederationof Neurology(WFN)in1994….
Methods
RoutineEMGandneuralevokedpotentialswereperformedoneach subiectbyusingaKeypointEMG/EvokedPotentialSystem(Dantec Company.USA)at18—22oC.Flastbloodwascollectedfrompe—
riDheralveinfortestingCKlevelbeforeperformingEMGandmuscle biopsy(normalvalue:30—140U/L).Musclebiopsiesweretaken
flmm6Datientsforpathologicalexamination,including4casesbe' foreEMG,1casewithoutEMGand1case4dafterEMG.
RESULTS,
ThediseaseisSMA—IVpredominantandaffectsmoremales(9cases) tllaJlfemales(1case)agedseveralmonthsto65yearsold?Muscle weaknesspresentsmoreinproximalmuscles.CKlevelwashigher thantllenonnal,especiallyhigherinSMA—IVgroup,amongwhich2
DatientsshowedextremehighCKlevel.
EMGwasperformedon48muscles,showingfibriHationpotentialin 85%.fasciculationpotentialin60%andpositivesharpwavesln 17%muscles.Slightmusclecontractioncanresuhinahighpoten—
tialwavein70%andpolyphasicunitsin62%muscles?Motorand sensoryconductionvelocitiesweremeasuredonleftupperlimbaJld rightlowerlimb.Therewere4caseswithlowercompoundmu00l. actionpotentials(CMAP)andslowconductionvelocity,although withinn0珊alrange.Amongthe4cases,1casewiththedisease courseofabevetwoyearshaddecreaseddistalnerveCMAP,along withDrolongedlatenciesofproximalmusculocutaneousnerveand axillarynerve.Fwavehadbeentestedon3ormorenen'esmcases
1,2,4,6andallresultswerenormal.
Musclebiopsiesfrom6patientsshowedneurogemcmuscleatrophY." 5casesch啪cterizedbygroupsofsmallatrophicfibersandrelatively increasednuclei,infiltrationofmatrixwithfatandconnectivetis—
sues.Thereweren0inflammatorycellsinvolvedintheatrophict.s—
sues.0nlv9casesrevealedmusclefiberdistortedwithsomehyaline degeneration,uncleal"striationandvacuolardegeneration,cateno.d arraJlgementofmnsclenuclearinfocallesionregaon,andwere ch啪cterizedbyinflammatorymyopathy,whichwasfoundmonocytes ttndmacrophagesintheatrophictissues?
DISCUSSl0N
SMAisarareautosomalrecessiveneuromusculardiseasecharac' terizedbvprogressiveweaknessandwastingofskeletalmusclesre' sultingfromanteriorhorncelldegeneration?Therewerenoclinical
signsofdisordersofpyramidalsystem.EMGshowedneurogemc myoDatllv.TherewerenormalperipheralnerveconductionVelocltY aJldF—wave.Musclebiopsyshowedneurogenlcmuscularat"' Dhv.Case1wasa11infantilepatientwithEMGdemonstratingneu—
mgenic?珂opathy.Therefore,thiscasewasdiagnosedasinfantile SM.A0rSMAtypeI.Cases2and3wereSMAtypeII.OnsetofSMA