智能障碍与先天性动作协调障碍课件.ppt

1、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,2002l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

2、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What is Down syndromelDown syndrome,also known as Trisomy 21,is the single most common genetic pattern of malformation in man.lMost text books quote the incidence of Down syndrome to be between one in 700 to 800 live births.lIn 1866,John Langdon Haydon

3、Down described the physical features and associated medical problems that have come to be known as Down syndrome.lIn the 1930s,physicians established a relationship between advanced maternal age and Down syndrome.The chromosomal,or genetic,basis of Down syndrome was not established until 1959.lA nor

4、mal human cell contains 23 pairs of chromosomes which carry all of a persons genetic information.Due to several possible abnormal mechanisms of cell reproduction,patients with Down syndrome have an extra(third)copy of the 21st chromosome.Thus,Down syndrome is also called Trisomy 21.lAdvanced materna

5、l age is associated with a high incidence of Trisomy 21,but even women of typical child bearing age can have affected babies.lWhile the diagnosis may be strongly suggested by characteristic physical findings,the final diagnosis is often made only after chromosome analysis,which includes a complete c

6、ount and visualization under a microscope of the chromosomes taken from cells in the blood.Down syndromeCri du Chat Syndromel(Cry of the cat in French)is a genetic disorder caused by the loss or misplacement of genetic material from the fifth chromosome.It was first identified in 1963 by Professor L

7、ejeune,who also identified the genetic cause of Downs Syndrome.He described the syndrome after the sound that many of the babies and young children make when crying.lThis cry,along with some of the other notable features,is so characteristic of the syndrome that a doctor can usually identify the con

8、dition before a chromosome analysis has taken place.lAs well as the physical features,Cri du Chat causes a varied level of mental handicap.There are a few children who attend mainstream education,but the majority of the children need more specialised education.The long term outlook for children with

9、 Cri du Chat is quite good.Cri du Chat SyndromeCri du Chat SyndromeFetal alcohol syndromeFAS-affected childs brainFragile-x SyndromeHurlers syndromelHurler syndrome is an autosomal recessive lysosomal storage disorder affecting mucopolysaccharide metabolism,the underlying defect being a deficiency o

10、f alpha-L-iduronidase.Hurler syndrome is also called gargoylism.lThe patient presents before the age of 2 with coarse facial features and mild developmental delay.The childs development ceases and a gradual intellectual decline occurs with death in the second decade.Hurlers syndromePrader willi synd

11、romeLesch-Nyhan SyndromelLesch-Nyhan syndrome(LNS)is a rare genetic disorder characterized by an overproduction of uric acid,neurological disability,and behavioral problems.The symptoms of LNS typically appear between ages 3 and 6 months;the presence of orange-colored crystal-like deposits(orange sa

12、nd)in the childs diaper is usually the first symptom to appear in those affected with the syndrome.lLNS is caused by a mutation in the HPRT gene on the X-chromosome,resulting in a deficiency of the enzyme hypoxanthine-guanine phosphoribosyltransferase(HPRT).HPRT is involved in the recycling of purin

13、es.When the body is unable to recycle these purines,there is a dramatic overproduction of uric acid,which then leads to hyperuricemia.Hyperuricemia can result in gouty arthritis,tophi(lumpy deposits of uric acid crystals just under the skin)and kidney stones.lLNS has been reported to occur in 1 out

14、of every 100,000 live births.It is estimated that there are only several hundred individuals with the disorder in the United States.LNS has been found equally among all races and ethnic groups,however as an X-linked disorder,nearly all cases are male.LNS can either be inherited or it can occur as a

15、spontaneous(or new)mutation.lLNS was first described by Michael Lesch,M.D.and William Nyhan,M.D.,Ph.D.in 1964 when they reported two affected brothers.The enzymatic defect was discovered by Seegmiller and colleagues in 1967.Finally,the gene responsible for LNS was cloned and sequenced by Friedmann a

16、nd colleagues in 1985.l 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information processing capacityl文化不利因素的考量文化不利因素的考量:尤其是語言的表現常用的評估工具lStanford Binet Intelligence ScalelWelchsler Intelligence test for ChildrenlBayley Scale for Infant Develo

17、pment The sensorimotor-based infant assessments are poor predictors of mental ability at later ageslPeabody Picture Vocabulary常用的評估工具lRavens Progressive Matrices:selection of missing elements of abstract designslLeiter International Performance scale non language based test with minimal motor requir

18、ment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(IQ 4055)(可教育的)l輕度:23個標準差;912 歲(IQ 5570)lThe mental retardation label is useful as a passport to early intervention and special education

19、.lSuch label provides little insight into the strengths of the individual or the services that are needed and may limit a childs opportunities!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l直接服務l間接服務介入的模式lTop-Down approach the assessment identify means to achieve desired ou

20、tcomeslBottom-up approach the assessment results determine outcomesTop-down approachDesired outcomeStrategies to bypass obstaclesInterventionBottom-up approachIdentify strengths and weaknessesDetermine goalIntervention plan and Strategies 發展協調障礙者l凡動作上有所缺失(motor impairment)而並未有任何醫學疾病(medical conditio

21、n)或是低智商的診斷(low IQ)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-TRDSM-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%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