智能障碍与先天性动作协调障碍课件

智能障礙與先天性動作協調障礙天主教台東聖母醫院物理治療師陳志軒2005.4.20.智能障礙與先天性動作協調障礙天主教台東聖母醫院1智能障礙的定義l智力功能和適應行為上存有顯著之限制而表現出的一種障礙l所謂適應行為指的是概念(conceptual)社會(social)及應用(practical)三方面的技能l發生於18歲之前AAMR,2002智能障礙的定義智力功能和適應行為上存有顯著之限制而表現出的一2l概念:語言(接受性與表達性)讀和寫金錢概念l社會:人際自尊社會規範責任感l應用:日常生活職業技能概念:語言(接受性與表達性)讀和寫金錢3智能障礙的定義l心智功能明顯低下或個別智力測驗結果未達平均數負二個標準差l學生在自我照顧動作溝通社會情緒或學科學習較同年齡有顯著困難身心障礙及資賦優異學生鑑定原則基準第三條智能障礙的定義心智功能明顯低下或個別智力測驗結果未達平均數負4智能障礙的原因l疾病感染和中毒l外傷造成l新陳代謝障礙或營養失調l產後原因l染色體異常l懷孕期間物質濫用智能障礙的原因疾病感染和中毒5What is Down syndromelDownsyndrome,alsoknownasTrisomy21,isthesinglemostcommongeneticpatternofmalformationinman.lMosttextbooksquotetheincidenceofDownsyndrometobebetweenonein700to800livebirths.lIn1866,JohnLangdonHaydonDowndescribedthephysicalfeaturesandassociatedmedicalproblemsthathavecometobeknownasDownsyndrome.lInthe1930s,physiciansestablishedarelationshipbetweenadvancedmaternalageandDownsyndrome.Thechromosomal,orgenetic,basisofDownsyndromewasnotestablisheduntil1959.lAnormalhumancellcontains23pairsofchromosomeswhichcarryallofapersonsgeneticinformation.Duetoseveralpossibleabnormalmechanismsofcellreproduction,patientswithDownsyndromehaveanextra(third)copyofthe21stchromosome.Thus,DownsyndromeisalsocalledTrisomy21.lAdvancedmaternalageisassociatedwithahighincidenceofTrisomy21,butevenwomenoftypicalchildbearingagecanhaveaffectedbabies.lWhilethediagnosismaybestronglysuggestedbycharacteristicphysicalfindings,thefinaldiagnosisisoftenmadeonlyafterchromosomeanalysis,whichincludesacompletecountandvisualizationunderamicroscopeofthechromosomestakenfromcellsintheblood.WhatisDownsyndrome6Down syndromeDownsyndrome7CriduChatSyndromel(CryofthecatinFrench)isageneticdisordercausedbythelossormisplacementofgeneticmaterialfromthefifthchromosome.Itwasfirstidentifiedin1963byProfessorLejeune,whoalsoidentifiedthegeneticcauseofDownsSyndrome.Hedescribedthesyndromeafterthesoundthatmanyofthebabiesandyoungchildrenmakewhencrying.lThiscry,alongwithsomeoftheothernotablefeatures,issocharacteristicofthesyndromethatadoctorcanusuallyidentifytheconditionbeforeachromosomeanalysishastakenplace.lAswellasthephysicalfeatures,CriduChatcausesavariedlevelofmentalhandicap.Thereareafewchildrenwhoattendmainstreameducation,butthemajorityofthechildrenneedmorespecialisededucation.ThelongtermoutlookforchildrenwithCriduChatisquitegood.CriduChatSyndrome(Cryoft8CriduChatSyndromeCriduChatSyndrome9CriduChatSyndromeCriduChatSyndrome10FetalalcoholsyndromeFetalalcoholsyndrome11智能障碍与先天性动作协调障碍课件12FAS-affectedchildsbrainFAS-affectedchildsbrain13Fragile-xSyndromeFragile-xSyndrome14Hurlers syndromelHurlersyndromeisanautosomalrecessivelysosomalstoragedisorderaffectingmucopolysaccharidemetabolism,theunderlyingdefectbeingadeficiencyofalpha-L-iduronidase.Hurlersyndromeisalsocalledgargoylism.lThepatientpresentsbeforetheageof2withcoarsefacialfeaturesandmilddevelopmentaldelay.Thechildsdevelopmentceasesandagradualintellectualdeclineoccurswithdeathintheseconddecade.HurlerssyndromeHurlersyndr15Hurlers syndromeHurlerssyndrome16PraderwillisyndromePraderwillisyndrome17Lesch-Nyhan SyndromelLesch-Nyhansyndrome(LNS)isararegeneticdisordercharacterizedbyanoverproductionofuricacid,neurologicaldisability,andbehavioralproblems.ThesymptomsofLNStypicallyappearbetweenages3and6months;thepresenceoforange-coloredcrystal-likedeposits(orangesand)inthechildsdiaperisusuallythefirstsymptomtoappearinthoseaffectedwiththesyndrome.lLNSiscausedbyamutationintheHPRTgeneontheX-chromosome,resultinginadeficiencyoftheenzymehypoxanthine-guaninephosphoribosyltransferase(HPRT).HPRTisinvolvedintherecyclingofpurines.Whenthebodyisunabletorecyclethesepurines,thereisadramaticoverproductionofuricacid,whichthenleadstohyperuricemia.Hyperuricemiacanresultingoutyarthritis,tophi(lumpydepositsofuricacidcrystalsjustundertheskin)andkidneystones.lLNShasbeenreportedtooccurin1outofevery100,000livebirths.ItisestimatedthatthereareonlyseveralhundredindividualswiththedisorderintheUnitedStates.LNShasbeenfoundequallyamongallracesandethnicgroups,howeverasanX-linkeddisorder,nearlyallcasesaremale.LNScaneitherbeinheritedoritcanoccurasaspontaneous(ornew)mutation.lLNSwasfirstdescribedbyMichaelLesch,M.D.andWilliamNyhan,M.D.,Ph.D.in1964whentheyreportedtwoaffectedbrothers.TheenzymaticdefectwasdiscoveredbySeegmillerandcolleaguesin1967.Finally,thegeneresponsibleforLNSwasclonedandsequencedbyFriedmannandcolleaguesin1985.lLesch-NyhanSyndromeLesch-Nyha18智能障礙常伴隨的問題l先天性心臟疾病l心肺功能疾患l肌肉骨骼疾病智能障礙常伴隨的問題先天性心臟疾病19智能功能的評估l年齡大小的考量年齡大小的考量:年齡太小語言表達仍有問題不能以知覺動作的發展來評估智能的高低l障礙類別的考量障礙類別的考量:強調informationprocessingcapacityl文化不利因素的考量文化不利因素的考量:尤其是語言的表現智能功能的評估年齡大小的考量:20常用的評估工具lStanfordBinetIntelligenceScalelWelchslerIntelligencetestforChildrenlBayleyScaleforInfantDevelopmentThesensorimotor-basedinfantassessmentsarepoorpredictorsofmentalabilityatlaterageslPeabodyPictureVocabulary常用的評估工具StanfordBinetIntellig21常用的評估工具lRavensProgressiveMatrices:selectionofmissingelementsofabstractdesignslLeiterInternationalPerformancescalenonlanguagebasedtestwithminimalmotorrequirment常用的評估工具RavensProgressiveMat22智能障礙的分類l極重度:無自我照顧能力須長期照護l重度:同上l中度:他人庇護下從事非技術性工作部分自理能力l輕度:特殊教育下從事半技術或簡單技術工作及部分自理能力智能障礙的分類極重度:無自我照顧能力23智能障礙的分類l極重度:智商在平均值5個標準差以下3歲以下(可訓練的)l重度:45個標準差;36歲(IQ2540)l中度:34個標準差;69歲(IQ4055)(可教育的)l輕度:23個標準差;912歲(IQ5570)智能障礙的分類極重度:智商在平均值5個標準差以下24lThementalretardationlabelisusefulasapassporttoearlyinterventionandspecialeducation.lSuchlabelprovideslittleinsightintothestrengthsoftheindividualortheservicesthatareneededandmaylimitachildsopportunities!Thementalretardationlabeli25復健的介入l語言及動作發展遲緩經常是兒童被診斷是智能障礙的最初期線索之一l減少功能的限制l避免二次損害l促進同儕的互動l家屬觀念的引導復健的介入語言及動作發展遲緩經常是兒童被診斷是智能障礙的最初26復健的介入l直接服務l間接服務復健的介入直接服務27介入的模式lTop-DownapproachtheassessmentidentifymeanstoachievedesiredoutcomeslBottom-upapproachtheassessmentresultsdetermineoutcomes介入的模式Top-Downapproach28Top-downapproachDesiredoutcomeStrategiestobypassobstaclesInterventionTop-downapproachDesiredoutco29Bottom-upapproachIdentifystrengthsandweaknessesDeterminegoalInterventionplanandStrategiesBottom-upapproachIdentifystr30發展協調障礙者l凡動作上有所缺失(motorimpairment)而並未有任何醫學疾病(medicalcondition)或是低智商的診斷(lowIQ)且缺乏處理每天日常生活作息活學業學習所需的動作能力l美國精神醫學會DSM-IV-TR發展協調障礙者凡動作上有所缺失(motorimpairme31DSM-IV-TR診斷標準l主要以敘述動作為主l診斷標準不以感覺,知覺,智力等問題存在l通常是612歲時發展協調障礙兒童才會因功能上出現問題而被診斷出來DSM-IV-TR診斷標準主要以敘述動作為主32行為與情緒問題l易發脾氣l沮喪l低自尊l與疾病無關的頭痛及尿失禁行為與情緒問題易發脾氣33盛行率l美國精神醫學會:511歲6%l男與女比率約3/1l台灣地區七歲學童4.81%;八歲學童2.44%盛行率美國精神醫學會:511歲6%34感覺或動作技巧特徵l不正常的姿勢控制和精細動作的技巧l跑步笨拙經常跌倒無法做出連續的動作l反應慢動作時間長l較不會參與運動肌力較弱l寫字抓握及穿衣等動作計畫能力差感覺或動作技巧特徵35視覺特徵l無法精細估計物品大小l物品在空間中位置區辯困難l視覺注意力減少視覺特徵無法精細估計物品大小36本體覺或運動覺特徵l本體覺或運動覺的敏銳減少l視覺與本體覺整合有問題l肌肉收縮次序與力量控制是多變的l視動整合困難本體覺或運動覺特徵本體覺或運動覺的敏銳減少37心理社會l學習障礙或閱讀困難l缺乏同儕認同l低自我價值或更焦慮l動作協調問題持續至青少年以後l心理較無自信無能力有成就l越早發現治療效果越好心理社會學習障礙或閱讀困難38