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智能障礙與先天性動作協調障礙

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. 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 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 發生於 18 歲之前 AAMR, 2002. 概念 : 語言 ( 接受性與表達性 ) 讀和寫 金錢 概念 社會 : 人際 自尊 社會規範 責任感 應用 : 日常生活 職業技能. 智能障礙的定義.

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智能障礙與先天性動作協調障礙

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  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.

  2. 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種障礙 • 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 • 發生於 18歲之前 AAMR, 2002

  3. 概念: 語言(接受性與表達性) 讀和寫 金錢 概念 • 社會: 人際 自尊 社會規範 責任感 • 應用: 日常生活 職業技能

  4. 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二個標準差 • 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

  5. 智能障礙的原因 • 疾病感染和中毒 • 外傷造成 • 新陳代謝障礙或營養失調 • 產後原因 • 染色體異常 • 懷孕期間物質濫用

  6. What is Down syndrome • Down syndrome, also known as Trisomy 21, is the single most common genetic pattern of malformation in man. • Most text books quote the incidence of Down syndrome to be between one in 700 to 800 live births. • In 1866, John Langdon Haydon Down described the physical features and associated medical problems that have come to be known as Down syndrome. • In 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. • A normal human cell contains 23 pairs of chromosomes which carry all of a person's 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. • Advanced maternal age is associated with a high incidence of Trisomy 21, but even women of typical child bearing age can have affected babies. • While the diagnosis may be strongly suggested by characteristic physical findings, the final diagnosis is often made only after chromosome analysis, which includes a complete count and visualization under a microscope of the chromosomes taken from cells in the blood.

  7. Down syndrome

  8. Cri du Chat Syndrome • ("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 Lejeune, 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. • This cry, along with some of the other notable features, is so characteristic of the syndrome that a doctor can usually identify the condition before a chromosome analysis has taken place. • As 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 Cri du Chat is quite good.

  9. Cri du Chat Syndrome

  10. Cri du Chat Syndrome

  11. Fetal alcohol syndrome

  12. FAS-affected child’s brain

  13. Fragile-x Syndrome

  14. Hurler's syndrome • Hurler syndrome is an autosomal recessive lysosomal storage disorder affecting mucopolysaccharide metabolism, the underlying defect being a deficiency of alpha-L-iduronidase. Hurler syndrome is also called gargoylism. • The patient presents before the age of 2 with coarse facial features and mild developmental delay. The child's development ceases and a gradual intellectual decline occurs with death in the second decade.

  15. Hurler's syndrome

  16. Prader willi syndrome

  17. Lesch-Nyhan Syndrome • Lesch-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 sand) in the child’s diaper is usually the first symptom to appear in those affected with the syndrome. • LNS 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 purines. 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. • LNS has been reported to occur in 1 out 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 spontaneous (or new) mutation. • LNS 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 and colleagues in 1985.

  18. 智能障礙常伴隨的問題 • 先天性心臟疾病 • 心肺功能疾患 • 肌肉骨骼疾病

  19. 智能功能的評估 • 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 • 障礙類別的考量: 強調information processing capacity • 文化不利因素的考量: 尤其是語言的表現

  20. 常用的評估工具 • Stanford Binet Intelligence Scale • Welchsler Intelligence test for Children • Bayley Scale for Infant Development The sensorimotor-based infant assessments are poor predictors of mental ability at later ages • Peabody Picture Vocabulary

  21. 常用的評估工具 • Raven’s Progressive Matrices: selection of missing elements of abstract designs • Leiter International Performance scale non –language based test with minimal motor requirment

  22. 智能障礙的分類 • 極重度: 無自我照顧能力 須長期照護 • 重度: 同上 • 中度: 他人庇護下從事非技術性工作 部分自理能力 • 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

  23. 智能障礙的分類 • 極重度: 智商在平均值5個標準差以下 3歲以下(可訓練的) • 重度: 4~5個標準差 ; 3~6 歲(IQ25~40) • 中度: 3~4個標準差 ; 6~9 歲(IQ 40~55) (可教育的) • 輕度: 2~3個標準差 ; 9~12 歲(IQ 55~70)

  24. The mental retardation label is useful as a ‘passport’ to early intervention and special education. • Such label provides little insightinto the strengths of the individual or the services that are needed and may limit a child’s opportunities!

  25. 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期線索之一 • 減少功能的限制 • 避免二次損害 • 促進同儕的互動 • 家屬觀念的引導

  26. 復健的介入 • 直接服務 • 間接服務

  27. 介入的模式 • Top-Down approach theassessment identify means to achieve desired outcomes • Bottom-up approach theassessment results determine outcomes

  28. Top-down approach Desired outcome Strategies to bypass obstacles Intervention

  29. Bottom-up approach Identify strengths and weaknesses Determine goal Intervention plan and Strategies

  30. 發展協調障礙者 • 凡動作上有所缺失(motor impairment)而並未有任何醫學疾病(medical condition)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 • 美國精神醫學會 DSM-IV-TR

  31. DSM-IV-TR診斷標準 • 主要以敘述動作為主 • 診斷標準不以感覺, 知覺, 智力等問題存在 • 通常是6~12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

  32. 行為與情緒問題 • 易發脾氣 • 沮喪 • 低自尊 • 與疾病無關的頭痛及尿失禁

  33. 盛行率 • 美國精神醫學會: 5~11 歲 6% • 男與女比率約3/1 • 台灣地區七歲學童 4.81% ;八歲學童2.44%

  34. 感覺或動作技巧特徵 • 不正常的姿勢控制和精細動作的技巧 • 跑步笨拙經常跌倒無法做出連續的動作 • 反應慢動作時間長 • 較不會參與運動 肌力較弱 • 寫字 抓握及穿衣等動作計畫能力差

  35. 視覺特徵 • 無法精細估計物品大小 • 物品在空間中位置區辯困難 • 視覺注意力減少

  36. 本體覺或運動覺特徵 • 本體覺或運動覺的敏銳減少 • 視覺與本體覺整合有問題 • 肌肉收縮次序與力量控制是多變的 • 視動整合困難

  37. 心理社會 • 學習障礙或閱讀困難 • 缺乏同儕認同 • 低自我價值或更焦慮 • 動作協調問題持續至青少年以後 • 心理較無自信無能力有成就 • 越早發現治療效果越好

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