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 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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