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1、精神发育迟滞（Mental Retardation, 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。DefinitionslMental retardation are manifested by behaviors that deviate from norms for the particular sociocultural group and age.Have subnormal intellectual functioning and comme
2、nsurate deficits in adaptive functioning including capacities for social and personal sufficiency and independence.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：l常染色体隐性遗传：如遗传代谢性疾病：苯丙酮尿症。l常染色体显性遗传：结节性硬化症，神经纤维瘤病等。l染色体畸变（结构和数目的改变） 常染色体畸变：如Down Synd
3、rome 性染色体畸变：Klinefelter综合征,Turner综合征l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 Mental Retardation Causes Estimated Cause Examples Frequency (%) Prenatal Genetic disorders Chromosomal aberrations Down syndrome
4、 Single-gene mutations Tuberous sclerosis, phenylketonuria and other metabolic disorders, fragile X syndrome Multifactorial Familial mental retardation Malformation syndromes Prader-Willi, Williams, and Angelman due to microdeletions syndromesMental Retardation CausesCongenital malformations Malform
5、ations of the central Neural tube defects nervous system Multiple malformation Cornelia de Lange syndrome syndromes Estimated Cause Examples Frequency (%)Mental Retardation Causes Estimated Cause Examples Frequency (%)Exposure Maternal infectionsCongenital rubella, HIV Teratogens Fetal alcohol syndr
6、ome Toxemia, placentalPrematurity insufficiency Other Radiation, traumaMental Retardation Causes Estimated Cause Examples Frequency (%) Infections Meningitis Delivery problems Asphyxia Other HyperbilirubinemiaPostnatal Infections Encephalitis Toxins Lead poisoning Other postnatal causes Traumas, bra
7、in tumors Psychosocial problems Poverty, psychotic illness分级 按IQ水平分：轻度：5069； 中度：3549； 重度：2034； 极重度：IQ20。轻度轻度MRMR（7580%7580%）：）：IQ5069IQ5069。语言功能较好，。语言功能较好， 抽象思维、综合分析能力抽象思维、综合分析能力 较差；勉强小学毕业。日较差；勉强小学毕业。日 常生活自理，可学会一技常生活自理，可学会一技 之长。之长。中度（中度（12%12%左右）：左右）：IQ3549IQ3549。语言发育较差词。语言发育较差词 汇贫乏，不能完整表达思；汇贫乏，不能完整
8、表达思； 数的概念模糊，不能计和数的概念模糊，不能计和 点数；学习能力低下。学点数；学习能力低下。学 会简单的生活和工作技能，会简单的生活和工作技能， 常需要别人帮助。常有体常需要别人帮助。常有体 异常。异常。 重度（重度（78%78%）：）：IQ2034IQ2034。只能学会一些简单的。只能学会一些简单的 语言，有的不会说话；经过训练，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，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产劳动。从小可发现躯体及神经 系统异常。系统异常。 极重度（极重度（12%12%
9、）：）：IQ20IQ20以下。无语言能力；对以下。无语言能力；对 周围环境及亲人不认识，仅周围环境及亲人不认识，仅 有原始情绪反应，以哭闹尖有原始情绪反应，以哭闹尖 叫表需求食物或不乐意；全叫表需求食物或不乐意；全 部生活需要人照料，出生时部生活需要人照料，出生时 即有明显的躯体畸形及神经即有明显的躯体畸形及神经 系统异常。系统异常。 诊 断 标 准l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）低于人群均值标准差，在70以下l社会适应能力不足l起病于18岁以前DSM-IV Diagnostic Criteria lA.Significantly subaverage intel
10、lectual functioning: an IQ of approximately 70 or below on an individually administered IQ test (for infants, a clinical judgment of significantly subaverage intellectual functioning).lB.Concurrent deficits or impairments in present adaptive functioning (i.e., the persons effectiveness in meeting th
11、e standards expected for his or her age by his or her cultural group) in at least two of the following areas: communication, self-care, home living, social/interpersonal skills, use of community resources, self-direction, functional academic skills, work, leisure, health and safety.lC.The onset is b
12、efore age 18 years.lCode based on degree of severity reflecting level of intellectual impairment:l Mild mental retardation: IQ level 50.5 to approximately 70l Profound mental retardation: IQ level below 20 or 25l Mental retardation, severity unspecified: when there is a strong presumption of mental
13、retardation but the persons intelligence is untestable by standard testsCASElFred was the product of an uncomplicated pregnancy and delivery. Developmental milestones were remarkable for walking at 17 months of age, but slowness to talk and stammering, unclear speech. He was described as a little sl
14、ow, but at 5 years of age when verbal and learning delays became more obvious, the family became concerned. At age 6, Fred was injured in an automobile accident with resultant coma for 5 days. Hospital records at the time suggested the possibility of slight neurological injury, but his mother attrib
15、uted all of Freds subsequent problems to this accident. He was unable to keep up in school and fell into greater conflict with his younger siblings. At 10 years of age he dropped out of school,CASEland 4 years later was admitted to the Developmental Center. I.Q. testing over the years consistently p
16、laced Fred in the moderate range (middle to lower 50s). While in the institution Fred was seldom in trouble and worked on the wards, in the kitchen, and occasionally as a messenger. After 3 years he was discharged to the community, where despite nearly continuous supervision from employers and socia
17、l workers, he was said to be in frequent trouble. In virtually all of his multiple places of employment, primarily as a dishwasher, his reputation was highlighted by temper, aggression, profanity, and boorishness. He was also involved in several minor difficulties with police including several charg
18、es of vagrancy. On one occasion he took an employers car without permission and drove it recklessly. CASElOver time, his conduct became more competent, and at the age of 32 he presented as a robust-appearing man perpetually smoking a cigar who gave every appearance of being a happy-go-lucky, easygoi
19、ng, altogether happy man. There is nothing in his appearance to suggest that he is anything less than a normal man, and his speech is likewise unexceptionable?It is only upon much closer inspection that Freds intellectual deficit becomes apparent. To the casual observer, he is an ordinary man, compe
20、tent to live within the not too demanding constraints of his life circumstances.Cognitive and Adaptive FunctioninglSpecify an I.Q. of 70 or less in their diagnostic criteria for mental retardation. lMeasurements of adaptive behavior, then, need to have a developmental orientation, to be socially and
21、 culturally sensitive, and to represent the many settings in which people live, work, and play. Common Measures of Adaptive BehaviorMeasure Age Range Domains TestedVineland Adaptive Behavior Birth to 18 Communication: receptive, expressive, Scales (Sparrow, Balla, and written; daily living skills: p
22、ersonal, Cicchetti, 1984) domestic; community socialization: interpersonal relations, play and leisure; coping skills; motor skills: fine, grossCommon Measures of Adaptive BehaviorCommon Measures of Adaptive BehaviorScales of Independent Birth to 80+ Motor: fine, gross; social interaction and Behavi
23、or Revised (Bruininks, communication: social interaction, language Woodcock, Weatherman, & comprehension, language expression; Hill, 1996) personal living skills: eating and meal reparation, toileting, dressing; community, living skills: time and punctuality, money and value, workCommon Measures of
24、Adaptive BehaviorCommon Measures of Adaptive BehaviorAAMR Adaptive Behavior Scales 3 to 18? Independent functioning, physical development, (Lambert, Nihira, and Leland, 1993) economic activity, schoolKaufman Functional Academic15 to 58+Functional math and reading skills, Skills (Kaufman and Kaufman,
25、 administered directly to person 1994)Street Survival Skills9 to 40+ Administered to person; basic concepts, Questionnaire (Linkenhoker and functional signs, tools, health McCarron, 1993)鉴 别 诊 断l儿童精神分裂症l儿童多动症l孤独症l视听障碍所致学习困难唐 氏 综 合 征（Down Syndrome, DS） 曾称先天愚型、21-三体综合征、伸舌样痴呆；是由于异常增多了一条21号染色体所致。 占新生儿活婴
26、的1，占MR患者的16%。 产妇年龄影响患病率：30岁左右1 ，40岁左右10 ，45岁左右为20。临 床 表 现l智力低下l语言发育障碍l行为障碍l运动生长发育障碍l躯体畸形脆性X染色体综合征 一种X染色体连锁的遗传性疾病， X染色体长臂具有脆性部分。主要表现为：大睾丸、身材较高、面长耳大、前额及颧骨突出、语言行为障碍、智力低下等。男性患病率1，女性携带者1/3有症状，占MR中2%6%。 叶酸治疗有效、幼年效果明显、青春期后不明显。苯 丙 酮 尿 症（Phenylketonuria, PKU） 苯丙酮尿症是一种先天性苯丙氨酸羟化酶缺乏而致的代谢性疾病。由于苯丙氨酸羟化为酪氨酸过程受阻，苯丙氨酸及其代谢产物在体内蓄积，造成一系列神经系统损害，并在尿中出现大量苯丙氨酸及苯丙酮酸等物质，故称苯丙酮尿症。 主要表现为智力低下、色素缺乏及脑电图异常。地方性克汀病（endemic cretinism） 又称地方性呆小病。发生在地方性甲状腺肿流行区。临床上有显著的精神发育迟滞和躯体发育延迟，病人身材矮小且不匀称，下部量短于上部量；骨骼发育迟缓，运动功能不良。 另有地方性亚临床克汀病较克汀病患病率更高。治 疗 与 预 防l病因治疗l改善脑细胞代谢的药物l对症治疗l康复训练
