第五章 神经发育障碍
第一节 智力障碍
The neurodevelopmental disorders are agroup of conditions with onset in the developmental period. The disorders typically manifest early in development (发育早期), often before the child enters grade school (入学前), and are characterized by developmental deficits that produce impairments of personal, social, academic, or occupational functioning (个人、社交、学业和职业上都存在功能受损).
•Intellectual disability (intellectual developmental disorder)：智力障碍
•The communication disorders：交流障碍
•Autism spectrum disorder：自闭症谱系障碍
•ADHD：注意缺陷与多动障碍
•The neurodevelopmental motor disorders：神经发育性运动障碍
•Specific learning disorders：特异性学习障碍
一、智力障碍的定义
•Intellectual disability (intellectualdevelopmental disorder) is characterized by deficits in general mental abilities, such as reasoning, problem solving, planning, abstract thinking, judgment, academic learning, and learning from experience. The deficits resultin impairments of adaptive functioning, such that the individual fails to meet standards of personal independence and social responsibility in one or more aspects of daily life, including communication, social participation, academic or occupational functioning, and personal independence at home or in community settings. Global developmental delay, as its name implies, is diagnosed when an individual fails to meet expected developmental milestones in several areas of intellectual functioning.
专题：Rosa's Law
二、智力障碍的诊断标准（Diagnostic Criteria of Intellectual Disability）
A. 智力缺陷（deficits inintellectual functions）
B. 适应能力缺陷（deficits inadaptive functioning）
C. 起病于发育期 （onset duringdevelopmental period）
按严重程度区分为如下四类：
•Mild 轻度
•Moderate 中度
•Severe 重度
•Profound 极重度
适应能力(Adaptive functioning)主要从以下三个方面的推理能力来进行评估:
•Conceptual - 概念理解能力
•Social - 社交能力
•Practical- 实践能力
•适应能力（诊断标准B）指的是与其他相同年龄组和社会文化环境下的人相比，个体多大程度上能够具备个人独立生存能力和承担一定的社会责任（有一系列标准需要符合）。
三、诊断特点（Diagnostic Features）
1. 单纯的IQ评估并不可靠
2. 沟通技巧的缺乏，会导致情绪调节能力低，进而导致破坏性和攻击性行为
3. 患者易受骗
4. 患者容易遭遇意外伤害
四、发病率（prevalence）
•总体发病率：约1%
•严重病例的发病率：约 6‰
五、与诊断相关的性别因素（Gender-Related Diagnostic issues）
•Mild：male vs female-1.6 vs 1
•Severe：male vs female -1.2 vs 1
•Male vulnerability to brain insult may account for some of the gender differences.
六、病程发展（ Development and Course ）
•Delayed motor, language, and social milestones may be identifiable within the first 2 years of life among those with more severe intellectual disability, while mild levels may not be identifiable until school age when difficulty with academic learning becomes apparent.
•When intellectual disability is associated with a genetic syndrome, there may be a characteristic physical appearance (as in, e.g.. Down syndrome).
•Although intellectual disability is generally nonprogressive, in certain genetic disorders (e.g., Rett syndrome) there are periods of worsening. After early childhood, the disorder is generally lifelong.
•Early and ongoing interventions may improve adaptive functioning throughout childhood and adulthood.
七、致病风险和预后（ Risk and Prognostic Factors ）
•主要是基因和生理原因（genetic and physiological）
•产前：DNA序列变异；染色体疾病
•围产期：过度劳累；新生儿脑炎
•产后：缺氧缺血性损伤；脑外伤；感染；癫痫；中毒（铅、水银）
•环境因素：酒精、药物、中毒等
•其他：先天代谢错误；脑结构异常；母亲疾病