lin22307 |
Wysłany: Śro 9:35, 02 Mar 2011 Temat postu: 肌酸磷酸激 |
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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
resuhinahighpoten—tialwavein70%andpolyphasicunitsin62%muscles·MotorandsensoryconductionvelocitiesweremeasuredonleftupperlimbaJldrightlowerlimb.Therewere4caseswithlowercompoundmu00l。actionpotentials(CMAP)andslowconductionvelocity,althoughwithinn0珊alrange.Amongthe4cases,1casewiththediseasecourseofabevetwoyearshaddecreaseddistalnerveCMAP,alongwithDrolongedlatenciesofproximalmusculocutaneousnerveandaxillarynerve.Fwavehadbeentestedon3ormorenen’esmcases1,2,4,6andallresultswerenormal.Musclebiopsiesfrom6patientsshowedneurogemcmuscleatrophY。“5casesch啪cterizedbygroupsofsmallatrophicfibersandrelativelyincreasednuclei,infiltrationofmatrixwithfatandconnectivetis—sues.Thereweren0inflammatorycellsinvolvedintheatrophict。s—sues.0nlv9casesrevealedmusclefiberdistortedwithsomehyalinedegeneration,uncleal"striationandvacuolardegeneration,cateno。darraJlgementofmnsclenuclearinfocallesionregaon,andwerech啪cterizedbyinflammatorymyopathy,whichwasfoundmonocytesttndmacrophagesintheatrophictissues·DISCUSSl0NSMAisarareautosomalrecessiveneuromusculardiseasecharac‘terizedbvprogressiveweaknessandwastingofskeletalmusclesre‘sultingfromanteriorhorncelldegeneration㈦Therewerenoclinicalsignsofdisordersofpyramidalsystem.EMGshowedneurogemcmyoDatllv.TherewerenormalperipheralnerveconductionVelocltYaJldF—wave.Musclebiopsyshowedneurogenlcmuscularat”‘Dhv.Case1wasa11infantilepatientwithEMGdemonstratingneu—mgenicⅡ珂opathy.Therefore,thiscasewasdiagnosedasinfantileSM。A0rSMAtypeI.Cases2and3wereSMAtypeII.OnsetofSMA
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