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20070919 Development Delay In Neuropsychological Function洪焜隆院長
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20070919 Development Delay In Neuropsychological Function洪焜隆院長

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    20070919 Development Delay In Neuropsychological Function洪焜隆院長 20070919 Development Delay In Neuropsychological Function洪焜隆院長 Presentation Transcript

    • Growth & Developmental delay in children 兒童生長與發展遲緩 洪焜隆 國泰綜合醫院小兒科 台北醫學大學兼任教授
    • Fetal growth
    • Normal children stages Normal children stages o         Neonate (newborn) (0-30 days) o         Infant (baby) (1 month-1 year) o         Toddler (1-4) o         Primary school age (4-12) o         Adolescence and puberty (teenage) (13-19) o         Young adult (19-25 ) Normal children stages o         Neonate (newborn) (0-30 days) o         Infant (baby) (1 month-1 year) o         Toddler (1-4) o         Primary school age (4-12) o         Adolescence and puberty (teenage) (13-19) o         Young adult (19-25 ) Normal children stages o         Neonate (newborn) (0-30 days) o         Infant (baby) (1 month-1 year) o         Toddler (1-4) o         Primary school age (4-12) o         Adolescence and puberty (teenage) (13-19) o         Young adult (19-25 ) Normal children stages o         Neonate (newborn) (0-30 days) o         Infant (baby) (1 month-1 year) o         Toddler (1-4) o         Primary school age (4-12) o         Adolescence and puberty (teenage) (13-19) o         Young adult (19-25 ) o         Neonate (newborn) (0-30 days) o         Infant (baby) (1 month-1 year) o         Toddler (1-4) o           Prmary school age (4-12) o         Adolescence and puberty (teenage) (13-19) o         Young adult (19-25)
    • Growth formula (average) Weight Birth 3-12mo 1-6yr 7-12yr Kg 3.25 Age(mo) + 9 2 Age(yr) × 2 + 8 Age(yr) × 7 - 5 Height Birth 1yr 2-12 yr cm 50 75 Age(yr) × 6 + 77
    • Normal growth in children
      • Growth in 1st year: rapid, discontinuous burst in physical, motor, cognitive development
      • Growth in 2nd year: slow progress in physical, cognitive, emotional, linguistic development
      • Growth in preschool year: slow in physical, rapid in language, complex in cognition & play
      • Growth in school year: slow in physical, central issue-- self-esteem & peer grouping
      • Growth in adolescence: rapid growth, second ary sex characteristics, asking independence
    • 0-6 歲男生身高生長曲線 (1999 衛生署資料 )
    • (1999 衛生署資料 )
    • (1999 衛生署資料 )
    • (1999 衛生署資料 )
    • Body proportional change
    • Sex maturation rate (SMR)
    • Sex maturation rate (SMR)
    •  
    • Growth retardation: etiology
      • 1.  Constitutional growth delay
      • 2.   Endocrine disorder: hypopituitarism, hypothyroidism
      • 3.    Genetic disease: Turner syndrome, Prader-Willi syndrome
      • 4.    Precocious puberty
    • Human brain development
      • Developmental event Time Main function
      • 1 .Induction( 誘導期 )
      •   Dorsal induction 3 ~ 4w Neural tube formation
      • Ventral induction 5 ~ 6w Prosencephalic development
      • 2 .Cellular proliferation 2 ~ 4m Neuron, glia cell
      • ( 細胞增生期 )
      • 3 .Migration( 移行期 ) 3 ~ 5m Cerebral cortex, cerebellum
      • 4 . Organization 5m ~ NB Lamination
      • ( 器官形成期 ) Neurite & synapses formation
      • 5 . Myelination 6m ~ Oligodendroglial differentiation
      • ( 髓鞘化 )   postnatal years Myelin maturation
    • Timetable of CNS development
      • Neural plate and groove 18 days
      • Anterior pore closure 24-26 days
      • Posterior pore closure 26-28 days
      • Five cerebral vesicles 35 (5W) days
      • CSF circulation 56 days
      • Corpus callosum 70-100 days
      • Primary fissure 70-150 days
      • 6-layered cortex 6-7 months
      • Secondary & tertiary fissure 7-9 months
      • Myelination of spinal cord 11 weeks
      • Myelination of brainstem 5 months
      • Myelination of cortical area 2nd decade postnatal
    • 嬰幼兒期,突觸成長最快 初生時 六歲 十四歲
    • Developmental milestones
    • Normal neuropsychological development
      • 年齡 粗 動 作 細 動 作 語言發育 身邊處理及社會性
      • 3 m 抬頭 握拳、張手 哭、咕聲 笑、視物移動
      • 6m 翻身、扶坐 抓物、合掌 尋聲源、牙牙語 認人
      • 9m 坐、爬 換手、 雙 手握物 答答聲 身邊玩具
      • 12m 站、扶走 拍手、撕紙 叫爸、媽 躲躲貓、脫帽
      • 1.5y 行走 塗鴉、翻書 單字、片語 摹仿動作、拿杯喝水
      • 2y 跑跳、上下樓 堆積木 (2-3) 10 個單字 用叉匙、脫衣
      • 3y 單腳跳 畫圓圈 句子 認出部份身體
    • Developmental neurological tests in newborns & infants
      • 1) 身體姿勢 (Posture)
      • 仰臥 (Supine)
      • 俯臥 (Prone)
      • 腹部懸抱 (Ventral suspension)
      • 坐 (Sitting)
      • 站 (Standing)
      • 2) 神經反射 (Reflex)
      • 3) 肌肉張力 (Muscle tone)
      • 主動張力
      • 被動張力
    • Prone posture NB 2m 4m 6m
    • Primitive reflex & protective reflex
      • 擁抱反射 (Moro reflex) birth~6m
      • 吸吮反射 (Sucking reflex) birth~3m
      • 尋奶反射 (Rooting reflex) birth~3m
      • 抓握反射 (Grasp reflex) birth~6m
      • 頸部強直反射 (Tonic neck reflex) 1m~6m
      • Landau reflex 5m~24m
      • 降落反射 (Parachute reflex) 9m~
      • Traction response 3m~
    • Early Reflex Pattern Moro reflex
    • 吸吮反射 (Sucking reflex) 抓握反射 (Grasp reflex)
    • Early Reflex Pattern Tonic neck reflex
    • Landau reflex Postural activities in 7m/o infant supported in ventral suspension
    • Parachute reflex
    • Traction response
    • 台灣目前 常 用 發展測試 工具 (1) Bayley Scales of Infant Development-II ( 貝莉氏嬰兒發展量表 )
      • 內容 : 包括 心理、動作、行為 三個分測驗。
      • 適用年齡: 出生至 42 個月
      • 1. 心理分量表 (Mental scale): 評估認知學習有關能力 =>
      • 心理發展指標 --Mental Index (MDI= 100  15S.D.)
      • 2. 動作分量表 (Motor scale): 評估肢體控制功能 =>
      • 動作發展指標 --Motor Index (PDI=100  15 S.D. )
      • 3. 行為分量表 : 評估人際關係、對環境的覺察、注意力和 行為控制等特質 => 結果以 百分位數 (percentile) 代表。
      • 發展年齡 (developmental age: DQ) 和層面分析 ( Facet analysis)
    • 台灣目前 常 用 發展測試 工具 (1I) Denver Developmental Screening Test ( 丹佛嬰幼兒發展測驗 , Denver II)
      • 粗動作
      • 精細動作及適應能力
      • 語言
      • 身邊處理及社會性
      • 適用年齡:出生至六歲
    • 教育積木
    • 圖形 (1)
    • Picture
    • Draw
    • Tower
      • Chinese Child Development Inventory (CCDI, 學齡前兒童行為發展量表) -- 照顧者填答
      • 適用年齡: 6 個月至六歲
      • 內容:包括粗動作、精細動作、溝通表達、概念理解、環境理解、生活自理、人際社會、綜合發展,推估各範疇之發展年齡
      台灣目前 常 用 發展測試 工具 (1II) Chinese Child Development Inventory (CCDI, 學齡前兒童行為發展量表)
    •  
    • Developmental delay
      • A child with developmental delay is a child who is not achieving certain skills as quickly as expected. In other words, the child is not reaching developmental milestones at the usual age (>20% of normal age) .
      • Cognitive development ( 認知發展 )
      • Social and emotional development ( 社會及適應行為發展 )
      • Speech and language development ( 語言發展 )
      • Gross & fine motor skill development ( 運動能力發展 )
      • Sensory function development ( 知 覺 發展 )
    • Causes of developmental delay
      • 1) Prenatal: chromosomal abnormalities, congenital dysmorphic syndromes, inherited disorders and intrauterine injuries to fetus
      • 2) Perinatal: birth trauma, hyperbilirubinemia, asphyxia, metabolic disorders, infections, intracranial hemorrhage and neonatal seizures
      • 3) Postnatal: infection, head trauma, intoxication, encephalopathy
      • Environmental factors : abuse, neglect and deprivation
    • 致病機轉
      • 致病時間 產前 週產期 產後
      •  
      • 傷害 發展中的中樞神經系統與肌肉骨骼系統等
      •  
      • 功能 運動 認知 語言 行為
      •  
      • 疾病 腦性麻痺 智能障礙 發展性語言障礙 行為障礙
    • 腦源性的功能異常可以造成許多種障礙 腦功能異常 抽痙藥 僵化 營養不良 學習 障礙 萎縮 感染 吞嚥異常 便秘 膀胱功能 自閉症 精神異常 攻擊 副作用 巨結腸 尿道炎 胃炎 / 肺炎 尿失禁 骨質疏鬆 腸阻塞等 精神性藥物 多種外傷 骨折 貧血 / 慢性肺病等 之副作用 癲癇 運動障礙 餵食障礙 平滑肌功能障 認知異常 行為障礙
    • What is Early Intervention and Who Benefits From it? *Source: U.S. Department of Education
      • Early intervention applies to children of school age or younger who are discovered to have or be at risk of developing a handicapping condition or other special need that may affect their development.
      • Early intervention consists in the provision of services such children and their families for the purpose of lessening the effects of the condition.
      • Early intervention can be remedial or preventive in nature--remediating existing developmental problems or preventing their occurrence.
      • Early intervention may focus on the child alone or on the child and the family together.
      • Early intervention programs may be center-based, home-based, hospital-based , or a combination. Services range from identification--that is, hospital or school screening and referral services--to diagnostic and direct intervention programs.
      • Early intervention may begin at any time between birth and school age; however, there are many reasons for it to begin as early as possible .
    • 早期療育之重要性
      • 據聯合國世界衛生組織的估計,發展遲緩兒童的發生率約為 6%~8%
      • 台灣每年出生的嬰兒約二十萬人,則其中一萬二千人可能就是發展遲緩兒童,若能及早發現、及早治療,其中有一部份可完全趕上正常同齡兒童,有些雖不能完全趕上,卻可減輕殘障程度,而能發揮較多的潛力。
      • 根據歐美先進國家多年的經驗及研究顯示:早期療育不但可 減少社會成本 ,也 減少未來醫療、教育及機構成本 ,同時也可 提升未來社會公民的素質 。根據聯合國世界衛生組織之統計,每投入 1 元於早期療育工作中,可節省特殊教育 3 元的成本;也有專家學者認為 3 歲以前作早期療育一年的功效是 3 歲以後 10 倍的功效,故療育愈早愈好,尤其要把握 0 至 6 歲的黃金療育期。
    • 如何及早發現幼兒 有發展遲緩情形?
      • 一般六歲以下健康兒童可配合兒童健康手冊於一個月、三個月、五個月、七個月、十個月、一歲、一歲六個月 ~ 二歲、三歲 ~ 四歲、五歲 ~ 六歲至小兒科醫師或家醫科醫師處 定期做健康檢查 ,任何時期如果檢查出有生長發育 ( 如:生理、運動、視力、聽力、認知、智能、語言溝通、感覺、情緒、學習、社會行為、環境適應等方面 ) 異常,可由醫師轉介至相關醫療專家做進一步聯合鑑定。
      • 如果屬於 高危險群兒童 ,如早產兒、低體重兒、多胞胎兒、有先天疾病… . 等,因日後發育過程中出現發展遲緩之機率比一般兒童高,更需經常做發展評估。
      • 此外 幼稚園、托兒所、保母 等相關照顧者也應常注意孩子是否比其他同年齡的孩子發展較慢,如對外界的刺激沒有反應、到一歲了還不會爬、兩歲還不會模仿說單字… . 等。
    • Service of Early Intervention
      •   Assistive technology (devices a child might need)
      •   Audiology or hearing services
      •   Counseling and training for a family
      •   Educational programs
      •   Medical services
      •   Nursing services
      •   Nutrition services
      •   Occupational therapy
      •   Physical therapy
      •   Psychological services
      •  Respite services
      • (Multidisciplinary program)
    • 發展遲緩兒童早期療育服務實施方案
      • ( 中華民國八十六年五月廿九日台 (86) 內社字第八六七五七八二號函頒 ,
      • 中華民國八十九年十二月十二日台 (89) 內童字第八九九 00 六一號函修正 )
      • 壹、依據:    一、 兒童福利法    二、 特殊教育法    三、 身心障礙者保護法
      • 貳、目標: 一、結合社會福利、衛生、教育等專業人員,以團 隊合作方式,提供發展遲緩兒童早期療育服務。
      •     二、建立發展遲緩兒童早期療育發現、通報流程及 服務模式確立各相關單位分工權責。
      • 參、策劃單位: 肆、主辦單位:
      •     內政部 台北市政府
      •   教育部 高雄市政府
      • 行政院衛生署 台灣省各縣市
      •   福建省金門縣、連江縣政府
    • 早期療育服務的範疇
      • 從發展遲緩者的需求立場來看,早期療育服務至少包括以下六個步驟中所提供的各項協助︰
      • 發現
      • 通報
      • 轉介
      • 評估
      • 療育服務
      • 追蹤與結案
    •  
    • Diagram of migration disorder
      • 1. Normal cortex
      • 2. Lissencephaly
      • 3. Pachygyria
      • 4. Schizencephaly
      • open
      • Schizencephaly
      • closed (fused)
      • 6. Heterotopia
      • 7. Polymicrogyria
    • Peri-Sylvian polymicrogyria (cortical dysplasia) 7 m/o seizure, microcephaly and developmental delay T1: Rt hemiatrophy & with malformation of the operculum. T2: Thick cortical ribbons over Rt operculum Rt insula exposed due to under-and/or maldeveloped operculum
    • 何貫儀 1.5y/o M Lissencephaly 1.5 y/o M seizure & developmental delay
    • 邱浩安 2m/o Male DCC 2m/o Male DCC Pachygyria with dysgenesis of corpus callosum 2 m/o male Hypotonia
    • What’s wrong with 6/m child?
    • What’s wrong with 5 y/o child?
    • What’s wrong with 10 y/o child?