1. Developmental and Cognitive Disorders
Chapter 13

2. What are developmental disorders?
Disorders that usually first appear in childhood or adolescence (onset)
Note: most of these conditions persist into adulthood

5. ICA D/O
As you answer the questions below, you will be better able to choose between the four categories introduced earlier.
1. Is the predominant sx an impairment of learning or intellectual function?
2. Is the predominant sx abnormal motor activity?
3. Is the predominant sx socially inappropriate or self injurious behavior?
4. Is the predominant sx an impairment in the ability to communicate or exchange meaningful information?

6. Perspectives on Developmental Disorders
Normal vs. Abnormal Development
Developmental Psychopathology
Study of how disorders arise and change with time
Childhood is associated with significant developmental changes
Disruption of early skills will likely disrupt development of later skills

7. Outline Common developmental disorders (ADHD & Learning Disorders)
Pervasive Developmental disorders (autism)
Mental Retardation
Cognitive Disorders (dementia)

8. Attention Deficit Hyperactivity Disorder (ADHD): An Overview
Nature of ADHD
Central features – Inattention, overactivity, and impulsivity
Difficulty w sustained attn (can’t finish games, watch TV)
Constant motion, fidgeting
Blurt out answers, act without thinking
Associated with behavioral, cognitive, social, and academic problems

9. Attention Deficit Hyperactivity Disorder (ADHD): An Overview
DSM-IV and DSM-IV-TR Symptom Clusters
Cluster 1 – Symptoms of inattention
Cluster 2 – Symptoms of hyperactivity and impulsivity cluster
Either cluster 1 or 2 must be present for a diagnosis

10. ADHD: Facts and Statistics
Prevalence
Occurs in 4%-12% of children who are 6 to 12 years of age
Symptoms are usually present around age 3 or 4
68% of children with ADHD have problems as adults
Impulsive component decreases over time
Gender Differences
Boys outnumber girls 4 to 1

11. ADHD: Overdiagnosis? Cultural Factors
Probability of ADHD diagnosis is greatest in the United States
Studies show more school-aged kids on stimulants than prevalence of Dx
College-aged people often present for assessment when academic problems emerge

12. ADHD: Biological Contributions
Genetic Contributions
ADHD runs in families
Some studies suggest High heritability
Familial ADHD may involve deficits on chromosome 20
The D4 receptor gene is more common in ADHD children (dopamine dysregulation?)

13. ADHD: Biological Contributions
Neurobiological Contributions: Brain Dysfunction and Damage
Used to be called “minimal brain dysfunction”
Inactivity of the frontal cortex and basal ganglia
Right hemisphere malfunction
Abnormal frontal lobe development and functioning
Yet to identify a precise neurobiological mechanism for ADHD

14. ADHD: Biological Contributions
The Role of Toxins
Allergens and food additives do not appear to cause ADHD
Maternal smoking increases risk of having a child with ADHD

15. ADHD: Psychosocial Contributions
Psychosocial Factors appear to Influence (not cause) the Disorder
Constant negative feedback from teachers, parents, and peers
Peer rejection and resulting social isolation
Such factors foster low self-image

16. Biological Treatment of ADHD
Goal of Biological Treatments
To reduce impulsivity/hyperactivity and to improve attention
Stimulant Medications
Reduce the core symptoms of ADHD in 70% of cases
Examples include Ritalin, Dexedrine, Cylert
Other Medications
Imipramine and Clonidine (antihypertensive) have some efficacy

17. Biological Treatment of ADHD
Effects of Medications
Improve compliance and decrease negative behaviors in many children
Not clear that academic performance or social skills are improved in the long-term
Beneficial effects are not lasting following drug discontinuation
Negative side effects include insomnia, drowsiness, and irritability

18. Behavioral and Combined Treatment of ADHD
Behavioral Treatment
Involve reinforcement programs
Aim to increase appropriate behaviors and decrease inappropriate behaviors
May also involve parent training
Combined Bio-Psycho-Social Treatments
Are highly recommended - it appears however that long-term psychosocial Tx is necessary to maintain gains

19. Learning Disorders Scope of Learning Disorders
Problems related to academic performance in reading, mathematics, and writing
Performance is substantially below what would be expected (IQ - Ach discrepancy)
DSM-IV and DSM-IV-TR Reading, Mathematics, Written Expression Disorders
Performance is at a level significantly below that of a typical person of the same age
Problem cannot be caused by sensory deficits (e.g., poor vision)

20. Learning Disorders: Some Facts and Statistics
Incidence and Prevalence of Learning Disorders
1% to 3% incidence of learning disorders in the United States
Prevalence is highest in wealthier regions of the United States
Prevalence rate is 10% to 15% among school age children
Reading difficulties are the most common of the learning disorders
School experience for such persons tends to be quite negative
About 32% of students with learning disabilities drop out of school

21. Half of school children classified as disabled have learning disabilities. Twenty years ago the proportion was 50% lower
Figure 13.1

22. Biological and Psychosocial Causes of Learning Disorders
Genetic and Neurobiological Contributions
Reading disorder runs in families, with 100% concordance rate for identical twins
Evidence for subtle forms of brain damage is inconclusive
Overall, genetic and neurobiological contributions are unclear
Psychological and motivational factors seem to affect eventual outcome

23. Treatment of Learning Disorders
Medications not typically used
Requires Intense Educational Interventions
Remediation of basic processing problems (e.g., teaching visual skills)
Efforts to improve of cognitive skills (e.g., instruction in listening)
Targeting behavioral skills to compensate for problem areas
Data Support Behavioral Educational Interventions for Learning Disorders

24. Pervasive Developmental Disorders
PDD is an umbrella term
severe and pervasive impairments in several areas of development: reciprocal social interaction skills, communication skills, presence of stereotyped behavior, interests, and activities
Symptoms are on a continuum
5 PDD’s: autistic disorder, Asperger’s disorder, Rett’s disorder, Childhood Disintegrative Disorder, PDD/NOS (distinctions among these not particularly clear)

25. Autistic Disorder Approximately 10 cases per 10,000 individuals
More common in males (4-5:1)
Impairment in Social Interactions
Impairment in Communication
Restricted, Repetitive and Stereotyped Behaviors, Interests, and Activities
Onset of delays prior to age 3 years

26. Autistic Disorder (cont.)
Qualitative Impairment in Social Interaction (needs at least 2)
marked impairment in the use of nonverbal behaviors
Failure to develop peer relationships appropriate to developmental level
A lack of spontaneous seeking to share enjoyment, interests, or achievements
lack of social or emotional reciprocity

27. Autistic Disorder (cont.)
Qualitative impairment in Communication (needs a lest one)
delay or total lack of development of spoken language (no compensation)
marked impairment in the ability to initiate or sustain a conversation with others in individuals that can speak
stereotyped and repetitive use of language or idiosyncratic language
lack of carried , spontaneous make-believe play or social imitative play appropriate to developmental level.

28. Autistic Disorder (cont.)
Restricted, Repetitive and Stereotyped Behaviors, Interests, and Activities (needs at least one)
encompassing preoccupation with one or more stereotyped and restricted patterns of interest that is abnormal either in intensity or focus
apparently inflexible adherence to specific, nonfunctional routines or rituals
stereotyped mannerisms
persistent preoccupation with parts of objects

29. Other issues related to autism
Sensory issues (e.g., high threshold for pain, oversensitivity to sounds, fascination with touch or smell)
Abnormalities in mood and affect
Feeding issues (limited diet, pica)
Behavior difficulties (SIB, Tantrums, short attention span, hyperactivity, sleep problems)

30. Phenomenology of Autism
20/20 Video
What is it like to be autistic?

31. Autism and Intellectual Functioning
75% of individuals have mental retardation
50% have IQs in the severe-to-profound range of mental retardation
25% test in the mild-to-moderate IQ range (i.e., IQ of 50 to 70)
Remaining people display abilities in the borderline-to-average IQ range
Better language skills and IQ test performance predicts better lifetime prognosis

32. Etiology of Autism Psychosocial Contributions Are Unclear
Autism has a genetic component that is largely unclear
Neurobiological evidence for brain damage – Link with mental retardation
Cerebellum size – Substantially reduced in persons with autism

33. Asperger’s Disorder Qualitative impairment in social interaction
Restrictive Repetitive and stereotyped patterns of behavior, interest, and activities
Disturbance causes clinically significant impairment
no clinically significant general delay in language
no clinically significant delay in cognitive or adaptive functioning

34. Rett’s Disorder Only reported in females
Apparent normal pre and perinatal development
Normal head circumference at birth
Deceleration of head growth between 5 and 48 months
Loss of hand skills between 5 and 30 months with development of stereotyped hand movement, loss of social engagement, poorly coordinated gait or trunk movements, severely impaired expressive and receptive language development with severe psychomotor retardation
Severe or Profound MR
6-7 cases per

35. Applied Behavior Analysis
Breaks down autism into separate behavioral problems and attempts to treat as many of these problems as possible.
By far the most well documented treatment approach with hundreds of studies on behavioral treatment for children with autism.
Goal is remediation (recovery) from the disorder to the point that children are indistinguishable from their peers.

36. UCLA Young Autism Project (Lovaas)
Intensive one-on-one training for app. 40 hours a week.
Training prg carried out in homes, school, community
commences at age no older than 46 months
1st phase, focuses on teaching compliance, simple imitation, appropriate play, suppress self-stimulation and non-compliant behavior
2nd phase teaching expressive skills, early abstract language, social play with peers
Third phase teaching expression of emotions, functional academics and more complex cognitive abilities (e.g., cause-to-effect relationships)

37. UCLA Young Autism Project - study design
Initial 2 year treatment
N = 59 children
3 groups:
(a) Experimental group (N=19) - 40 hours a week of treatment for 2 years
Control group # 1 (N=19) - 10 hours of treatment for 2 years on average
Control group #2 (N=21) - No treatment provided by UCLA
Blind Evaluations pre & post treatment

38. UCLA Young Autism Project pre-treatment
Exp Group Control Group # 1
Treatment: 40 hrs/week hrs/week
Pre Treatment Measures:
Age at Dx 32 months 34 months
Beg. trt 35 months 40 months IQ

39. UCLA Young Autism Project pre-treatment
Exp Gr Control Group # 1
(N=19) (N=19)
Mute 58% 47%
Reject Adults 63% 42%
Not Toilet Trained 68% 63%
Gross Inattention 89% 74%
Tantrums 89% 79%
Absent Toy Play 53% 63%
Self Stimulation 95% 89%
Absent Peer Play 100% 100%

40. UCLA Young Autism Project- Results
Blind evaluation: pre treatment & 2 years later (Age 6-7)
Experimental Group Results:
** Average IQ Gain = 20 points
** 9/19 (47%) Completed first grade in regular class
** IQ Gain for these 9 subjects = 37 points
8/19 (42%) Continued in a learning disabled class
2/19 (11%) Severe MR / autistic classroom
Control Group Results: Group # Group # 2
IQ no significant changes
Completed first grade in regular class: 0/19 ( 0%) /21 (5%)
Continued in a learning disabled class: 8/19 (42%) /21 (48%)
Continued in a Severe MR Class: /19 (58%) /21 (48%)

41. UCLA Young Autism Project- Results 1993 Follow Up Study Results
Blind Evaluations of the 9 children in the “Best Outcome” Group
Age at follow up evaluation - 13 years old.
IQ gains remained.
Normal functioning on tests of:
emotional functioning;
social functioning;
intellectual functioning.
8/9 (88%) Remained in typical classrooms.
1/9 was in an LD classroom.

42. Autism - Pharmacotherapy
No known effective medications

43. Mental Retardation Nature of Mental Retardation
Disorder of childhood
Below-average intellectual and adaptive functioning
Range of impairment varies greatly across persons
Mental Retardation and the DSM-IV and DSM-IV-TR
Significantly subaverage intellectual functioning (IQ below 70 - about 2-3% of the population)
Concurrent deficits or impairments two or more areas of adaptive functioning
MR must be evident before the person is 18 years of age

44. Levels of MR Mild MR Moderate MR Severe MR Profound MR
Includes persons with an IQ score between 50 or 55 and 70 (often can lead independent lives)
Moderate MR
Includes persons in the IQ range of to
Severe MR
Includes people with IQs ranging from up to 35-40
Profound MR
Includes people with IQ scores below (typically require complete assistance)

45. Other Classification Systems for Mental Retardation (MR)
American Association of Mental Retardation (AAMR)
Defines MR based on levels of assistance required
Examples of levels include intermittent, limited, extensive, or pervasive assistance
Not that widely adopted
Classification of MR in Educational Systems
Based on whether person is “Educable”
Stigmatizing system

46. Mental Retardation (MR): Some Facts and Statistics
Prevalence
About 1% to 3% of the general population
90% of MR persons are labeled with mild mental retardation
Gender Differences
MR occurs more often in males, male-to-female ratio of about 1.6:1
Course of MR
Tends to be chronic, but prognosis varies greatly from person to person

47. Mental Retardation (MR): Psychosocial Contributions
Cultural-Familial Retardation
Believed to cause about 75% of MR cases and is the least understood
Believed to result from combination of biological (low IQ) and social factors
Neglect, abuse, poor nutrition
Associated with mild levels of retardation on IQ tests and good adaptive skills
Lower end of distribution but probably distinct etiology from those w clear organic causes

48. Mental Retardation (MR): Biological Contributions
Genetic Research
MR involves multiple genes, and at times single genes
Chromosomal Abnormalities and Other Forms of MR
Down syndrome – Trisomy 21
Fragile X syndrome – Abnormality on X chromosome
Maternal Age and Risk of Having a Down’s Baby
Nearly 75% of cases cannot be attributed to any known biological cause

49. Specific genetic syndromes associated with MR
Down Syndrome
Fragile X syndrome
Types of chromosomal abnormalities (trisomies - a third copy of the chromosomes instead of only two) Down, Klinfelter XXY. Monosomies is the absence of one of the two chromosomes. Structural abnormalities: deletions, translocations -. Thus, a part of chromosome 11 might swith with chromosome 22 - mosaicism(usually chromosome abnormalities start off with the egg ot the sperm and every cell is affected. With mosaicism the baby is conceived with the normally expected chromosome patterns but somewhere during the process of embryonic cell dicision a chromosome abnormality accidentilly occurs in one of the dividing cells. So some cells are normal, others are not and the child develops with two populations of of cells p.39 in Dykens,.

50. Down Syndrome

51. Down Syndrome Most common chromosomal form of MR
Prevalence: about 1 out of every 700 live births
As many of 75% of trisomy 21 result in miscarriages or stillbirths.
The older the mother, the higher the (p) of DS (e.g., Maternal age of 20 = 1 in 2000; 35 = 1 in 500; 45 = 1 in 18)
Theory - ova (eggs) produced in youth are exposed to toxins, radiation - damaged
Most prevalent form of mental retardation. Has as many studies as all the other 750 genetic mr syndromes together,
no distinction in race and SE background

52. Down Syndrome (cont.)
Health issues: Congenital heart defects (50), hearing loss (66-89), ophthalmic conditions (60), endocrine conditions (e.g., hypothyroidism) (50-90), obesity (50-60), dental problems (60-100), seizure disorders (6-13), high risk of Alzheimer's disease.
High risk of Alzeimer’s disease (127 per typical adults older than 60 - nearly all people with DS show the neuropathology of Alzheimer;safter age 30 (15-40% show the behavioral signs age 40 and older)

53. Down Syndrome (cont.)
Adaptive behavior: In general children with DS show higher AB levels than intelligence
Personality: Sociable and pleasant.
Dual DX: Less often and less severe maladaptive behavior and psychopathology

54. Fragile X Syndrome

55. Fragile X syndrome Most common known inherited form of MR
Prevalence = 1 in 4000 males and at least half that in females.
The marker was an X chromosome with a small, pinched-off piece of genetic material
Mothers often have learning disabilities
males only have one X and are more vulnerable to many disorders because females have two X chromosomes. So a recessive X linked disorder cannot be buffered by the other X chromosome as in females.
Martin and Bell (1943) described a large family in which multiple males were affected and all had the same characterisitcs.

56. Fragile X syndrome (cont.)
Physical features: 80% of post pubertal boys and men with this disorder have enlarged testicules (about twice the size).
Other physical features: long narrow face and prominent ears, flat feet, hyper extensible finger joints, soft skin
Features become more pronounced with age and are subtle in childhood
Few significant medical problems are associated with fragile X (seizures in about 20%)

57. Fragile X syndrome (cont.)
IQ levels vary from moderate levels of MR to the average range of functioning (varies with genetic status, gender, and age)
In general, females are less impaired than males
Many males seem to show declines in their IQ scores over time.
(3 last one more likely to be seen in higher functioning individuals)

58. Treatment of MR
For mild MR, tx is similar to that for learning disabilities
For more severe MR, treatment is similar to that for PDD
Goals include communication, social development, independent living and job skills
People with MR often Benefit from Such Interventions