(医药卫生)儿童神经肌肉疾病及其复健

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1、兒童神經肌肉疾病及其復健天主教台東聖母醫院物理治療師陳志軒今日主題何謂神經肌肉疾病?基本解剖學介紹臨床上常見的兒童神經肌肉病變復健的處理中樞神經系統人體的運動單位人體的運動單位前角細胞(ant.horncell)脊髓神經根(spinalroots)週邊神經(peripheralnerve)神經肌肉交界處(neuromuscularjunction)肌肉(muscle)神經肌肉病變泛指運動單位(motorunit)的某一部分因病變的影響,而造成臨床上肌肉無力張力過低基腱反射降低肌肉萎縮等下運動神經元症候群的症狀兒童常見

2、的神經肌肉病變肌肉疾病裘馨氏肌肉萎縮症貝克型肌肉萎縮症運動神經元疾病小兒麻痺脊髓性肌肉萎縮症脊髓膨出症週邊神經臂神經叢受損裘馨氏肌肉萎縮症Duchennemusculardystrophy(DMD)Duchennemusculardystrophy(DMD)iscausedbyanalteredgenefoundontheX-chromosome,sotheconditionusuallyaffectsonlyboys.Insomecases,thedisorderappears'outoftheblue',foll

3、owinganewmutationintheunfertilisedegg.Inothers,DMDisinheritedfromacarriermother,whoisusuallyunaffectedherself.Acarriermotherhasaone-in-fourchanceofhavinganaffectedsonandaone-in-fourchanceofhavingacarrierdaughter.學齡前可發現的現象Gower’ssign骨盆及軀幹肌肉無力的現象常出現在DMD的小孩肌肉組織切片脊

4、椎變形貝克型肌肉萎縮症BeckerMuscularDystrophySymptoms:Generalizedweaknessandmusclewastingaffectinglimbandtrunkmusclesfirst.Calvesoftenenlarged.AlmostidenticaltoDuchennebutoftenmuchlesssevere.Canbesignificantheartinvolvements.Onset:AdolescenceoradulthoodProgression:Disease

5、progressesslowlyandwithvariabilitybutwillaffectallvoluntarymuscles.SurvivalwellintomidtolateadulthoodCalfmusclesinitiallyenlarge(anattemptbythebodytocompensateforlossofmusclestrength),buttheenlargedmuscletissueiseventuallyreplacedbyfatandconnectivetissue(pseudo

6、hypertrophy).Cardiomyopathymayoccur,butthedevelopmentofcongestiveheartfailureorarrhythmias(irregularheartbeats)israre.X-linkedrecessive(femalesarecarriersBeckerMuscularDystrophy(BMD)isoneoftheninetypesofmusculardystrophyandisconsideredthemoremildcousinofDuchenn

7、eMuscularDystrophy,themostcommoninheritedchildhoodlethaldisorderofhumankind.BMDdiseaseaffectsapproximately1in30,000malebirthsregardlessofraceorethnicity,andthereareanestimated100,000BMDpatientsworldwide.HowisBMDdifferentfromDMD?BMDislessseverelydisablingthenDMD

8、.Anarbitarymeansofdistinguishingthetwodisordersdependsonwhethertheaffectedpersoncanstillwalkatage16years.Musclebiopsytendstoshowmoreorlessseverechanges,relatedtotheseverityo