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

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 1FengBH,LiWX,LuoJ,et一.EffectofGynosaponinXUlLonglutamicacidand GABAinmicebrainZhongguoYaolixueTongbao1998;14(3):234—6(China) 2HuoHR,WangTY,YuSR,eta1.ProtectiveeffectofsaponinsofGynostemma Pubescensonneuronalinjuryinculturedcorticalnellroll$ofmicezh鸣gu0 YaolixueTongbao1998;14(2):120—2(China) 3GuoSR.LuoWF.UuTP.EffectofBerberineonlearningandmemory, of mice.zb'增y?YaoliYu厶M^?增1997;13(2):17—9(China) 4XuJZ,LuM,ZhaoYG.et一.TheeffectofsapogeninfromZhimu(ZMS)onthe learningabilityandmemoryinagedrats-批ngy?YaoliYuLinchuang1995;11 (3):18—21(China) 5HuM,HuYE,ZhangW,eta1.TheeffectofZMSonbrainMreceptorinaged rats.踟,讲?脚?Zazhi2001;21(3):158—161(China) 6DaiXM,ZhangZX,FuZX,et—ThefacilitationeffectofFmctusbroussonetiaeon learningandmemoryinmicezb,.舻YaoliYuLinchuang1997;13(5):27—9 (China) 7YangWM,ZhouYX,HanMX,et—Experimentalresearchoffacilitationeffectof Rhizomapelygona,tionlearningandmemoryofmiceZhongyiyao,injiu2000: 16(3):45—7,53(China) 8LiLJ.HanCJ,CuiST.et—TheeffectofRhizomacureumaeonlearningand memoryandlipidperoxideinmice.Zhongyaocai1998;21(10):522—3(China) 9MaA,GuoH,EffectofRadixachyranthisbidentataeonmemo~anden— 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