Chapter 5: Intellectual and

Developmental Disabilities
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Topics

Definition
Prevalence
Causes
Identification
Psychological and behavioral characteristics
Educational considerations
Assessment of progress
Early intervention
Transition to adulthood

Whats in a name?
Idiot, moron, imbecile
Feeble-minded
Mental retardation
Still the classification used in Pennsylvania
today

Intellectual and developmental disabilities

Definition
Reasons for caution

Concern about misdiagnosis of ethnic

minorities
Possible stigma associated with the
diagnosis
Belief that intellectual disability is a socially
constructed condition

2002 AAIDD definition

Mental retardation is a disability
characterized by significant limitations
both in intellectual functioning and in
adaptive behavior as expressed in
conceptual, social, and practical adaptive
skills. This disability originates before
age 18.
IQ less than ____/75
Need to have both low IQ and limited
adaptive behavior

5 Assumptions of the AAIDD

Definition

Must consider context, age, and community

Assessment must be valid and consider
culture; language; sensory, motor, and
behavioral issues; and differences in
communication
Individuals have both limitations and
strengths
Should develop profile of needed supports
Life functioning can improve over time with
use of appropriate supports

Supports
Supports
Resources and strategies that promote development,
education, interests, and well being

Levels of support (1992 AAMR)

Intermittentas _________
Limiteddaily or weekly for _____ period of time
Extensivedaily or weekly for _____ period of time
Pervasivehourly or __________

Types of support
Natural
Service-based
Technological

Classification of Mental Retardation

(American Psychological Association)

IQ Score

Mild
Moderate
Severe
Profound

50-70
35-50
20-35
00-20

2002 Pennsylvania Definition

Mental retardation--"significantly subaverage
intellectual functioning that is accompanied by
significant limitations in adaptive functioning in at
least two of the following areas: Communcation,
self-care, home living, social/interpersonal skills,
use of community resources, self-direction,
functional academic skills, work, leisure, health,
and safety. The onset must occur before the
individuals 22nd birthday. (4210.101a)

IQ below 70

Types of Support in PA
Learning
Services for students with a disability who
require services primarily in the areas of
___________, ___________, __________, or
speaking or listening skills related to academic
performance

Life skills
"Services for students with a disability who
require service primarily in the areas of
academic, functional or vocational skills
necessary for __________ living.

Prevalence of Intellectual Impairments

Theoretical prevalence of intellectual impairment

based on normal curve = 2.27%

Prevalence of Intellectual Impairments

Actual prevalence =

1%

Discrepancy between theoretical and

actual prevalence may be due to:
consideration of adaptive behavior
classification as learning disabled for
those with higher IQs
classification as autistic

Prevalence of People with Mental

Retardation by Classification
Severe
3%

Profound
2%

Moderate
10%

Mild
85%

Causes
Prenatal causes (before ______)
Chromosomal disorders

Down syndrome
Williams syndrome
Fragile X syndrome
Prader-Willi syndrome
Inborn errors of metabolism
PKU

Developmental disorders of brain formation

Microcephalus
Hydrocephalus

Environmental influences
Fetal alcohol spectrum disorders
Rubella

Down Syndrome
Three types
Trisomy 21
Mosaicism
Translocation

Trisomy 21
Down Syndrome
Trisomy 21
47 chromosomes in all
cells due to trisomy of
21st chromosome
92% of all cases of
Down Syndrome
Related to increased
parental age

Maternal age and Trisomy 21 Down

Syndrome

Characteristics of
Down Syndrome

Physical Characteristics
Short stature
Obesity
Hyperflexibility of joints
Hypotonicity (low _____ _______)

Characteristics of
Down Syndrome
Distinctive facial features
Folds of skin around eyes
Small ears with very small lobes
Flat appearance to face; small nose
with small bridge
Protruding tongue due to:
small mouth
short roof of mouth
low muscle tone in tongue

Sparse, fine hair

Characteristics of
Down Syndrome
Physical Characteristics
Distinct hands and feet
simian crease across palms
small hands with short stubby
fingers
little finger curves in instead of
being straight
short stubby toes with wide
space between big and 2nd toe

Characteristics of
Down Syndrome
Health Characteristics

Myopia ( ____ sightedness) and/or strabismus ( _____ eyes) in 60%

Heart defects in 50%
Respiratory problems
Higher rate of leukemia
Higher rate of Alzheimer's
Conductive hearing loss in 66-89%
Hypothyroidism in 50-90%
Eczema and/or dry skin in 50%
Generally sterile
Shorter life span
50 years average life expectancy

Characteristics of
Down Syndrome
Cognitive characteristics
Better on visual-spatial tasks than verbal-auditory tasks
Girls are about 5-10 IQ points higher than boys
Declining IQ as person ages

highest IQs in infancy and early childhood

development plateaus in middle childhood
most adults have moderate MR
deterioration of adaptive skills beginning at 40

Language characteristics

Grammar is akin to that of 3 year old

Receptive language is better than expressive language
90% have articulation problems
May see more gesturing than actual language use

Characteristics of
Down Syndrome
Sociobehavioral characteristics
Generally are very sociable and friendly
outgoing
likely to use sociability to get out
of difficult tasks
may do better in inclusive settings
may prefer playing with younger
children

75% described as stubborn

increased depression with age

Williams Syndrome
Caused by microdeletion
of the 7th chromosome
(7q11.2)
Characteristics:
mild mental retardation in
75%
attention deficit disorder
and hyperactivity
overly friendly with adults
incessant chatter
inappropriate and
repetitive speech
excessive anxiety & worry

7q11.2

Williams Syndrome
Characteristics:
strengths in auditory rote
memory and language
extreme weakness in
perceptual and motor
auditory and textile
hypersensitivity
high incidence of absolute
pitch
cardiovascular disease
narrowing of the main
arteries leading from the
heart

Middle two rows are copied spatial designs (top row) by

two 11-year-old children with Williams Syndrome. Their
copies distort spatial properties while preserving other
design aspects, such as color. Bottom row drawings are by
a six-year-old child without Williams syndrome.

Williams Syndrome
Characteristics:
connective tissue abnormalities
joint limitation or laxity, and soft,
lax skin
hoarse voice
bowel/bladder diverticulae and
rectal prolapse

delayed rate of growth, including

low stature and a slight build
distinctive facial characteristics

Characteristics of Williams
Syndrome
________- like facial
features
broad brow
short nose, full nasal
tip,
long philtrum
full lips
wide mouth
small jaw
prominent earlobes

Fragile X
aka Martin-Bell Syndrome or Marker X syndrome
caused by a trinucleotide repeat expansion of the FMR1
gene on the _____ chromosome

Trinucleotides on the
X chromosome
expand
from the usual 6-50
repetitions to more
than 200 repetitions

Fragile X
symptoms
become
apparent @ 200
repeats;
The more
repetitions,
the more
symptoms
we see

Characteristics of
Fragile X
distinct face
long narrow face
prominent jaw
large protruding ears
high arched palate

Characteristics of
Fragile X

long hands and palms

large testes after puberty
seizures in 20%
autism-like features in 7-25% (Hatton, et al., 2003)
normal life expectancy
ADHD
mildmoderate intellectual impairment
in boys; borderline IQ in girls
decline in IQs over time

perseverative speech
generally better verbal skills than one
would expect given IQ
good adaptive behavior

Prader Willi Syndrome

Caused by deletion of part of 15th
chromosome (15q11-13) that derives from
father
most common cause

Characteristics of
Prader Willi Syndrome
Distinct faces
down turned mouth
almond shaped eyes
Hypopigmentation in 50%
Hypotonia (_____ muscle tone)
delayed motor development
failure to thrive and feeding problems in infancy
morbid obesity after infancy
obesity related health problems

More Characteristics of Prader Willi

Syndrome
short stature relative to family
adult malesavg. 5 ft. 1 inch
adult femalesavg. 4 ft. 10 ins.
small hands and feet

delayed secondary sex characteristics;

infertility
day time sleepiness and high threshold of
pain
abnormal temperature regulation

Cognitive Functioning in
Prader Willi Syndrome
Average IQ is 70
Strengths in visual processing
Social behavioral issues (_______ _________)
Hoarding or stealing food

Picking at skin, especially front of hands and legs

Temper tantrums
Stubbornness
Compulsive
Upset by changes in order
Anxiety

Prader Willi Syndrome Treatment

Growth hormone to increase height
Weight management
low calorie diet

less than 600 kcals per day in children

less than 1000 kcals per day in adults

limited food intake

regular exercise

Question
Jose has intellectual disabilities. As an
infant, he was lethargic and had difficulty
eating; however, at about one year of age
he became obsessed with food. This is
characteristic of
a) Down syndrome
b) Williams syndrome
c) Fragile X syndrome
d) Prader-Willi syndrome

Phenylketonuria (PKU)
recessive gene on 12th
chromosome
______ error of ________
Causes body to not be able to
metabolize phenylalanine (found
in ________ and nutrasweet)
Builds up in brain and causes
brain damage
Treatment involves diet with no
protein or nutrasweet
Diet must be followed until ______

Phenylketonuria (PKU)
the longer we wait to begin diet, the lower the IQ will be

Age of treament and IQ in PKU (Hanley et al.)

100

93.5
71.6

80
IQ

60

54.5

55.5

40.8

40
20
0
0-2

2-6

6-12

12-24

over 24

months

months

months

months

months

age

Screening Tests

Maternal serum screening

Nuchal translucency sonogram
Amniocentesis
Chorionic villus sampling
Newborn screening

Amniocentesis
Take sample of
amniotic fluid and
analyze it
Done at 13-16 weeks
1/250 risk of
miscarriage

Chorionic Villus Sampling (CVS)

Take sample of
chorion, placental
tissue
Done at 7-11 weeks
1% chance of
miscarriage

Hydrocephalus
Can result in intellectual
impairment

Untreated hydrocephalus

Treated hydrocephalus

Hydocephalus
treatment
Treat with shunt
Amount of intellectual
impairment depends on
shunts ability to drain fluid
Can do fetal surgery and
implant shunt in fetus
Monitor child for signs that
shunt is malfunctioning,
blocked or infected
increased lethargy, fever,
enlarged head size

Microcephalus
Micro= ___________
Cephalus or cephalo = _____________

Newborn with microcephaly

Microcephaly
Causes of microcephaly
Maternal protein deficiency
Various genetic disorders
School age children with
microcephaly

Although text says

life expectancy is short,
life expectancy is usually
related to size of head

Fetal Alcohol Spectrum Disorder

Fetal alcohol syndrome
Have all 4 characteristics

Fetal Alcohol Spectrum Disorder

Fetal alcohol effects syndrome

Have 3 of 4 characteristics
Usually lack the distinctive facial appearance

Characteristics of Fetal
Alcohol Spectrum Disorder
History of prenatal alcohol exposure
Growth deficiency for height and/or weight
Distinctive facial appearance including:

short eye slits

flat mid face
thin upper lip
short, upturned nose
smooth or long
philtrum
misaligned or
misshapen secondary
teeth

Characteristics of Fetal Alcohol

Spectrum Disorder
Epicanthal folds

Flat midface

Smooth philtrum

Characteristics of Fetal
Alcohol Spectrum Disorder

History of prenatal alcohol exposure

Growth deficiency for height and/or weight
Distinctive facial appearance
Brain or CNS damage as evidenced by:

Microcephaly
Reduced size of caudate nucleus in basal ganglia
(responsible for memory, attention, and cognition)
Intellectual impairment
tremors and or seizures
hyperactivity
ocular problems
attention deficits
poor coordination
poor impulse and anger control
difficulties with judgment & abstract reasoning
memory impairments

Fetal Alcohol Effects Syndrome

(FAES)
Aka alcohol-related
neurodevelopmental
disorder (ARND)
Fewer physical signs
Occurs 3 times more
frequently than FAS
(Streissguth, 1997)

Fetal Alcohol Spectrum Disorder

Secondary characteristics

90% develop mental health problems

60% quit school
60% trouble with law
50% incarcerated at some time
49% inappropriate sexual behavior
35% alcohol or drug problems

Congenital Rubella
aka German measles
Problematic if mom gets rubella
during early part of pregnancy
or right before pregnancy

Rubella:

Pregnancy outcomes in mothers who had congenital

rubella (Sever, Hardy et al.,)

Age of
infection
Preconception
First trimester
Second
trimester
Third trimester

0-28 days
before
conception
0-12 weeks
after
conception
13-26 weeks
after
conception
27-40 weeks
after
conception

Percentage of
Affected
infants
43%
51%
23%
0%

Most
severe
outcomes

Effects of Congenital Rubella

congenital cataracts
blind
intellectual impairment
deaf
deaf-blind
hydrocephaly
microcephaly
heart defects
Congenital cataracts

Causes (contd)
_______ causes (at the time of birth)
Anoxia
Low birth weight
Infections passed from mother
Syphilis
Herpes

Anoxia (lack of or too little ______)

Dont know exactly how
much anoxia is needed
to produce brain damage
Causes of anoxia:

Breech birth
Prolonged labor
Precipitous birth
Placenta previa
Cephalo-pelvic
insufficiency
Umbilical cord prolapse or
cord wrapped around
babys neck
Drugs use to speed labor
or sedate mother

Low Birth Weight

Full term babies
born after ______ weeks

weigh less than 2500 grams (_______ lbs)

Aka small for gestational age

Low birth weight vs. Prematurity

Time of
gestation

Weight

Premature

Less than Less than

36 weeks 5.5 lbs.

Low birth
weight

More than Less than

36 weeks 5.5 lbs.

Low Birth Weight

Causes of Low Birth
Weight

Maternal smoking
Maternal malnutrition
Maternal cocaine use
Multiple and close
pregnancies
Teen or young mother

Effects of Low Birth

Weight
Death
5 times increased risk

Low IQ
Microcephaly
Failure to thrive
syndrome

Congenital Syphilis Syndrome

Sexually transmitted disease in mother
Caused by a spirochete bacteria
Bacteria crosses the placenta and damages the fetus
Crossover cannot occur until 16th-18th week of pregnancy

Can prevent by:

Treating infected pregnant women with penicillin prior to the 16th
week of pregnancy
Give untreated babies penicillin after birth

Effects of Congenital Syphilis

Syndrome
Syphilis in baby

Fetal death
30% are stillborn

(Mavrov et al., 2001)

Distension of brain ventricle system

Intellectual impairment in 6% (Mavrov et al., 2001)

Congenital Herpes
Viral infection spread through exposure to
Herpes lesions
Prevent exposure to lesions through
Caesarian delivery

Effects of Congenital Herpes

Seizures
Death
50% die within 6 or 7 days

Intellectual Impairment and/or Cerebral

Palsy
Herpes lesions on baby

Causes (contd)
Postnatal causes (_____ birth)
Biological postnatal causes

Traumatic brain injury**

Encephalitis
Meningitis
Lead poisoning

Psychosocial postnatal causes

**Discussed in depth in Chapter 13

Meningitis & Encephalitis

Encephalitis-inflammation
of head or brain

Meningitis-inflammation
of meninges

Can occur at any time;

Can result in death, intellectual impairment, hearing loss,
deaf-blindness, and/or finger/toe loss

Lead Poisoning
Lead accumulates in brain and bodily
organs
Can cause:

Learning disabilities
Lower IQ
Borderline or mild intellectual impairment
Behavior problems
Neurological problems
Kidney disease

Sources of Lead
Paint
chips

Toys, jewelry, blinds,

and
ceramic
items from China

Lead pipes &

solder
In old plumbing

Dirt from
areas
near freeways

Psychosocial
Retardation prevalence per 1,000 School-age children by SES and IQ level
Socioeconomic status (SES)
High

Middle

Low

0-20 IQ

20-50 IQ

50-75/80 IQ

10

25

50

75/80-90 IQ

50

170

300

Psychosocial
Children who live
in poverty for first
5 years of life
average 9 IQ
points lower than
those who have
never lived in
poverty (Duncan et al., 1994)

Common correlates of low income

Poor language and verbal skills of parents

Low maternal education
Few books and toys
Limited educational opportunities and expectations
Single and/or teenage mothers

reduced supervision
reduced time spent with parent
emotional support may be lacking
lack of knowledge of child care

Inadequate prenatal care

Multiple pregnancies in short time period
Limited routines, rituals and consistency
sometimes, constant, unpredictable changes (Bernheimer, 2003)

From Greenwood, Hart, Walker, Risley (1994)

Talk addressed to Child by Parent

6000
4000

Upper
SES
Middle
SES
Low SES

2000
0
12

18

24

30

36

age in months

Talk addressed by Child to Parent

3000
2500
vocabulary

vocabulary

Psychosocial

2000
Upper SES

1500

Middle SES

1000

Lower SES

500
0
12

18

24
age in months

30

36

Vocabulary size of the child is

the single most important
predictor of reading success
(Anderson & Nagy, 2002)
Low vocabulary development in child

Low achievement in
school

Low maternal education

Low Mental Age in child

Early school drop out

Parenthood

Identification
Intelligence tests
IQ tests for children
(WISC-IV)
Cautions in using and
interpreting IQ tests
IQs can _______ from one
administration to next
All tests are culturally
biased
Younger the child, the less
valid the test
IQ does not guarantee
outcomes

Adaptive behavior skills

Parent or teacher answer
questions concerning the
ability of student to
perform adaptive skills
Vineland

Need both

Psychological and Behavioral

Characteristics
Genetic syndromes and behavioral
phenotypes
Specific learning problems

Poor working memory

Poor language comprehension and production
Poor self-regulation (metacognition)
Need external motivation
learned helplessness

Poor social development

Increased gullibility

Educational Considerations
Functional academics
NCLB access to the general curriculum in:
_________, __________, and _____

Instruction in real-life settings with real

materials

Grocery shopping in grocery store

Educational Considerations
Systematic instruction
Involves systematic
________, consequences
for performance, transfer of
stimulus control, and data
based decisions

Constant time delay

Progressive time delay

Prompt types
Ambiguous verbal
Specific verbal
Modeling
Gestures
Priming
Enough physical help to
start performing

Physical assistance
Faded through
graduated guidance

Question
Karen is trying to transfer control away
from prompts to a more naturally occurring
stimulus. She tells her daughter Pick up
your toys. Put them in that box (points to
box). Over the next few days, she will tell
her daughter to pick up her toys, but will
wait 10 seconds before telling her to put
them in the box. This is an example of
a) Progressive time delay
b) Constant time delay
c) Intermittent time delay

Functional behavioral assessment

(FBA)
Finding out the consequences, antecedents, and
setting events that maintain the behavior

Used to identify the purpose or function of the behavior

5 most common functions of

behavior

1. ______ attention
2. escape from a ________
3. access to ______
________
4. self stimulation / sensory stimulation
5. lack of knowledge or skill

Positive behavioral intervention and support (PBIS)

Support ______ behavior rather than ______ undesirable

behavior

Service delivery models

Less restrictive

Regular class with support (inclusion)

Class wide peer tutoring
Co-teaching

Resource room
Pennsylvania law limits 20 students to a resource room

Special class
in PA, usually 12-15 life skills or learning support
students
in PA, usually 8 students with multiple disabilities

Special school
day school
residential school

Hospital or homebound
More restrictive

Assessment
Progress monitoring
Academic skills
Curriculum-based
measurement (CBM)

Adaptive behavior skills

Need
both types

Outcome measures
Academic skills
PSSA-testing
accommodations
PASAalternative
assessment

Adaptive behavior
skills
Quality of life

Early Intervention
Programs designed for:
Prevention
_______ Preschool
Project
At age 27,
Higher graduation
rates
Fewer arrests
Less teen pregnancies
Less welfare
Higher income

or

Further development
Language
Conceptual skills
Behavior
Motor skills

Transition to Adulthood
Self-determination
Ability to make
personal choices, plan
and regulate ones life,
and be a self _______
Cultural differences
Can it be carried too
far?

Transition to Adulthood
Person-centered planning
Focus on the person
Supports a team approach
Vision, purposes, and
goals
Understand focus persons
past, present and future life
Develop actions for
change, mutual support,
personal and team
development, and learning
Usually creates the best
contextual fit

Goals of Person Centered Planning

(Iovannone, 2004)
1.

2.

3.

4.

5.

Being present and participating in community

life
Gaining and maintaining satisfying
relationships
Expressing preferences and making choices in
everyday life
Having opportunities to fulfill respected roles
and live in dignity
Continuing to develop personal competencies

Transition to Adulthood:
Community Adjustment
Community residential
facilities
aka ______ ______
3-10 folks
Supervision

Supported living
Own home or
apartment
Support

Transition to Adulthood:
Employment

Sheltered Workshop
no integration
less than minimum
wage
"non-meaningful"
work
no opportunities for
advancement
support, often
continuous

Transition to Adulthood:
Employment
Supported Competitive
integrated
prevailing wage and
benefits
real, meaningful work
opportunities for
advancement
ongoing, unlimited support
support in form of job
coach, natural supports or
technological supports