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精神发育迟滞(MentalRetardation,MR),定义,精神发育迟滞是指个体在发育阶段(通常在18岁以前),由于先天的或后天的,生物学方面的或社会、心理方面的不利因素,使精神发育受阻或停滞,造成智力明显低于平均水平及社会适应困难。,Definitions,Mentalretardationaremanifestedbybehaviorsthatdeviatefromnormsfortheparticularsocioculturalgroupandage.Havesubnormalintellectualfunctioningandcommensuratedeficitsinadaptivefunctioningincludingcapacitiesforsocialandpersonalsufficiencyandindependence.,患病率,一般人群中,MR患病率1%3%WHO(1985):轻度MR3%,重度34中国8省市014岁儿童(1988):1.2%;农村城市,男女湖南416岁儿童(1990):2.22%,病因,遗传性异常:常染色体隐性遗传:如遗传代谢性疾病:苯丙酮尿症。常染色体显性遗传:结节性硬化症,神经纤维瘤病等。染色体畸变(结构和数目的改变)常染色体畸变:如DownSyndrome性染色体畸变:Klinefelter综合征,Turner综合征先天性颅脑畸形。,母孕期有害因素的影响:感染:如风疹病毒等药物与毒性物质放射线照射母体健康情况母亲情绪因素,围产期不利因素:早产、难产、产伤、窒息、颅内出血等出生后不良因素:中枢神经系统感染、外伤、缺氧、内分泌代谢疾病、婴幼儿期严重营养不良、缺乏文化教育机会,MentalRetardationCauses,EstimatedCauseExamplesFrequency(%),PrenatalGeneticdisorders4.8ChromosomalaberrationsDownsyndromeSingle-genemutationsTuberoussclerosis,phenylketonuriaandothermetabolicdisorders,fragileXsyndromeMultifactorialFamilialmentalretardationMalformationsyndromesPrader-Willi,Williams,andAngelmanduetomicrodeletionssyndromes,MentalRetardationCauses,Congenitalmalformations7.7MalformationsofthecentralNeuraltubedefectsnervoussystemMultiplemalformationCorneliadeLangesyndromesyndromes,EstimatedCauseExamplesFrequency(%),MentalRetardationCauses,EstimatedCauseExamplesFrequency(%),Exposure5.3MaternalinfectionsCongenitalrubella,HIVTeratogensFetalalcoholsyndromeToxemia,placentalPrematurityinsufficiencyOtherRadiation,trauma,MentalRetardationCauses,EstimatedCauseExamplesFrequency(%),Perinatal2.0InfectionsMeningitisDeliveryproblemsAsphyxiaOtherHyperbilirubinemiaPostnatal3.2InfectionsEncephalitisToxinsLeadpoisoningOtherpostnatalcausesTraumas,braintumorsPsychosocialproblemsPoverty,psychoticillnessUnknown30.0,分级,按IQ水平分:轻度:5069;中度:3549;重度:2034;极重度:IQ20。,轻度MR(7580%):IQ5069。语言功能较好,抽象思维、综合分析能力较差;勉强小学毕业。日常生活自理,可学会一技之长。中度(12%左右):IQ3549。语言发育较差词汇贫乏,不能完整表达思;数的概念模糊,不能计和点数;学习能力低下。学会简单的生活和工作技能,常需要别人帮助。常有体异常。,重度(78%):IQ2034。只能学会一些简单的语言,有的不会说话;经过训练,能学会自己吃饭及基本卫生习惯,只能在监护下生活,不能进行生产劳动。从小可发现躯体及神经系统异常。极重度(12%):IQ20以下。无语言能力;对周围环境及亲人不认识,仅有原始情绪反应,以哭闹尖叫表需求食物或不乐意;全部生活需要人照料,出生时即有明显的躯体畸形及神经系统异常。,诊断标准,智力明显低于同龄人的平均水平,在个别性智力测验时智商(IQ)低于人群均值2.0标准差,在70以下社会适应能力不足起病于18岁以前,DSM-IVDiagnosticCriteria,A.Significantlysubaverageintellectualfunctioning:anIQofapproximately70orbelowonanindividuallyadministeredIQtest(forinfants,aclinicaljudgmentofsignificantlysubaverageintellectualfunctioning).B.Concurrentdeficitsorimpairmentsinpresentadaptivefunctioning(i.e.,thepersonseffectivenessinmeetingthestandardsexpectedforhisorheragebyhisorherculturalgroup)inatleasttwoofthefollowingareas:communication,self-care,homeliving,social/interpersonalskills,useofcommunityresources,self-direction,functionalacademicskills,work,leisure,healthandsafety.C.Theonsetisbeforeage18years.Codebasedondegreeofseverityreflectinglevelofintellectualimpairment:Mildmentalretardation:IQlevel50.5toapproximately70Moderateretardation:IQlevel35.0to50.5Severementalretardation:IQlevel20.5to35.0Profoundmentalretardation:IQlevelbelow20or25Mentalretardation,severityunspecified:whenthereisastrongpresumptionofmentalretardationbutthepersonsintelligenceisuntestablebystandardtests,CASE,Fredwastheproductofanuncomplicatedpregnancyanddelivery.Developmentalmilestoneswereremarkableforwalkingat17monthsofage,butslownesstotalkandstammering,unclearspeech.Hewasdescribedasalittleslow,butat5yearsofagewhenverbalandlearningdelaysbecamemoreobvious,thefamilybecameconcerned.Atage6,Fredwasinjuredinanautomobileaccidentwithresultantcomafor5days.Hospitalrecordsatthetimesuggestedthepossibilityofslightneurologicalinjury,buthismotherattributedallofFredssubsequentproblemstothisaccident.Hewasunabletokeepupinschoolandfellintogreaterconflictwithhisyoungersiblings.At10yearsofagehedroppedoutofschool,CASE,and4yearslaterwasadmittedtotheDevelopmentalCenter.I.Q.testingovertheyearsconsistentlyplacedFredinthemoderaterange(middletolower50s).WhileintheinstitutionFredwasseldomintroubleandworkedonthewards,inthekitchen,andoccasionallyasamessenger.After3yearshewasdischargedtothecommunity,wheredespitenearlycontinuoussupervisionfromemployersandsocialworkers,hewassaidtobeinfrequenttrouble.Invirtuallyallofhismultipleplacesofemployment,primarilyasadishwasher,hisreputationwashighlightedbytemper,aggression,profanity,andboorishness.Hewasalsoinvolvedinseveralminordifficultieswithpoliceincludingseveralchargesofvagrancy.Ononeoccasionhetookanemployerscarwithoutpermissionanddroveitrecklessly.,CASE,Overtime,hisconductbecamemorecompetent,andattheageof32hepresentedasarobust-appearingmanperpetuallysmokingacigarwhogaveeveryappearanceofbeingahappy-go-lucky,easygoing,altogetherhappyman.Thereisnothinginhisappearancetosuggestthatheisanythinglessthananormalman,andhisspeechislikewiseunexceptionable?ItisonlyuponmuchcloserinspectionthatFredsintellectualdeficitbecomesapparent.Tothecasualobserver,heisanordinaryman,competenttolivewithinthenottoodemandingconstraintsofhislifecircumstances.,CognitiveandAdaptiveFunctioning,SpecifyanI.Q.of70orlessintheirdiagnosticcriteriaformentalretardation.Measurementsofadaptivebehavior,then,needtohaveadevelopmentalorientation,tobesociallyandculturallysensitive,andtorepresentthemanysettingsinwhichpeoplelive,work,andplay.,CommonMeasuresofAdaptiveBehavior,MeasureAgeRangeDomainsTestedVinelandAdaptiveBehaviorBirthto18Communication:receptive,expressive,Scales(Sparrow,Balla,andwritten;dailylivingskills:personal,Cicchetti,1984)domestic;communitysocialization:interpersonalrelations,playandleisure;copingskills;motorskills:fine,gross,CommonMeasuresofAdaptiveBehavior,ScalesofIndependentBirthto80+Motor:fine,gross;socialinteractionandBehaviorRevised(Bruininks,communication:socialinteraction,languageWoodcock,Weatherman,community,livingskills:timeandpunctuality,moneyandvalue,work,CommonMeasuresofAdaptiveBehavior,AAMRAdaptiveBehaviorScales3to18?Independentfunctioning,physicaldevelopment,(Lambert,Nihira,andLeland,1993)economicactivity,schoolKaufmanFunctionalAcademic15to58+Functionalmathandreadingskills,Skills(KaufmanandKaufman,administereddirectlytoperson1994)StreetSurvivalSkills9to40+Administeredtoperson;basicconcepts,Questionnai
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