Intellectual Disability Flashcards
Define developmental disability
Severe chronic disabilities (cognitive or physical or both). Appear before the age of 22 - likely to be lifelong.
Developmental delay
present before tage 5. May lead to permanent developmental disability or may become resolved over time and/or with treatment
Intellectual Disability (ID)
Significant limitations in intellectual functioning and adaptive behavior. originates before 18.
What is intellectual functioning?
General mental capacity, (learning, reasoning, problem solving). IQ of 70-75 or below required for diagnosis of ID
What is adaptive behavior?
conceptual, social and practical skills that people have learned to be able to function in their everyday lives.
What are significant limitations in Adaptive behavior?
performance that is at least 2 SD below the mean on a standardized test
What are some conceptual skills?
Receptive and expressive language, reading and writing, money concepts, time, self-directions
What are some social skills?
Interpersonal, responsibility, self esteem, gullibility, naivete, follows rules/obeys laws
What are some practical skills?
Eating, dressing, mobility.
IADL - preparing meals, managing money, housekeeping
maintaing safe environment
occupational skills
What are the 5 assumptions that the definition of ID must be applied in?
Appropriate context Consider cultural/linguistic diversity Limitations coexist with strength develop profile of needed supports With supports life with ID will generally improve
Intermittent Level of Support
Support is not always needed. It is provided on an “as needed” basis and is most likely to be required at life transitions (e.g., moving from school to work)
Limited Level of Support
consistent support is required, though not on a daily basis (non-intensive)
Extensive Level of Support
regular, daily support is required in at least some environments (e.g., daily home-living support)
Pervasive Level of Support
daily extensive support, perhaps of a life-sustaining nature, is required in multiple environments
What age range does help me grow serve?
prenatal to age three (early intervention, home visitation)
Etiology of ID
40-50% have no identifiable cause.
Causes may be prenatal Chromosomal/errors of metabolism), perinatal (birth asphyxia), postnatal (seizure disorders, malnutrition)
Associated motor/sensory problems
May have delays in motor development (motor learning/motor control)
Difficulty monitoring sensory input
How to children with ID compare to those who develop typically?
> # reps to learn Difficulty generalizing skills Difficulty maintaining skills that arent practiced Slower response time More limited repertoire of responses
Causes of Down Syndrome
Most not inherited Trisomy 21 (nondisjunction - 95%, translocation (4%), mosaic - 1%)
When are pre-natal screenings recommended
First trimester (11-14 weeks) 2nd trimester (15-20 weeks)
What do they look for at the first trimester screen?
nucal translucency Blood test (PAPP-A, hCG)
What do they look for at the 2nd trimester screen?
hCG, estriol, AFP, Inhibin
Common characteristics of Downs Syndrome
Flat occiput/face, upward slating eyes, flat nasal bridge, small ears, protruding tongue
Sensory/visual deficits, hearing loss, heart defects, immune deficiency, thyroid disease, osteoporosis, sleep apnea, Alzheimers
Musculoskeletal characteristics of Downs Syndrome
Hypotonia, ligamentous laxity (atlantoaxial instability), decreased strength and cardiovascular fitness
Gross motor development with Down Syndrome
Occurs in same order as typically developing peers. Physical and medical characteristics of down syndrome can interfere with development (linked to mental age, gross more quickly than fine motor)
Motor development improves with practice (more practice, clumsiness and decreased refinement), difficulty with balance (slower reaction times), decreased strength, strong visual learners.
When do gross motor skills improve the fastest?
Birth to age 6. fastest at younger ages then levels off.
How do DS kids compare to typical children with regard to GMS?
With DS didnt achieve all skills on GMFM by age 6, DS need more time to learn movements as complexity increases.
What is fragile X syndrome?
Mutation of the FMR1 geneMost commonly inherited form of ID (more males than females), females are usually the carrier
S/S fragile X syndrome
Facial features (some not all); large head, long face, prominent ears, chin, and forehead
developmental delays
social/behavioral problems
seizures
males usually mild-severe ID, females can have normal intelligence or some degree of ID.
Behavioral problems of Fragile X syndrome
Anxiety ADHD Sensory Defensiveness Hyperarousal Aggression Sleep disturbances Self Abuse
MSK S/S of Fragile X syndrome
hypotonia joint laxity joint dislocation scoliosis balance problems pes planus pectus excavatum
What are fetal alcohol spectrum disorders? (FASD)
An umbrella term for a group of conditions that can occur in a person whose mother drank alcohol during pregnancy.
How does FASD work?
Alcohol crosses placenta to baby, immature liver metabolizes it slowly so longer exposure to alch for baby, it’s a teratogen for neurodevelopmental disorders
What is ARBD?
Alcohol Related Birth Defects - malformations and dysplasias of cardiac, skeletal, renal, ocular, auditory and other systems.
S/S Fetal Alcohol Syndrome
Abnormal facial features
Growth problems
CNS abnormalities
Deficits in motor devel, posture, balance, coordination, gait
Problems with learning, memory, attention span, communication, vision or hearing
Hard time in school/trouble getting along with others.
Alcohol related neurodevelpomental disorder
Complex range of disabilities in neurodevelopment and behavior, adaptive skills, self regulation
How is ARND different from FAS
ARND includes FAS in which there are distinct facial features and growth retardation.
Will people with ARND have ID and problems with behavior/learning?
They might. Might to poorly inschool, have difficuties with math, memory, attention, judgement, and poor impulse control
What organ systems might be affected by ARBD?
heart, kidneys, bones, hearing or mix.
What is Prader-Willi Syndrome?
Microdeletion of long arm of paternal chromosome 15 - rare
S/S Prader WIlli syndrome (around birth)
Hypotonic at birth, difficulty with feeding and sucking, FTT, muscle weakness, strabismus, speech and motor delays (sit 12 mo, walking 24 mo)
Characteristic facial features of prader willi syndrome
almond shaped eyes, narrowing of the head at the temples, durned down mouth, thin upper lip
Later S/S of Prader WIlli Syndrome
Develops insatiable appetite around 2-3 y.o.
Decreased growth hormone (lack of adol. growth spurt)
Short stature (small hands/feet)
Delayed/incomplete puberty
behavior problems
inadequate temperature control
sleep disturbances
skin picking
mild ID
risk for pathologic Fx 2ndary to OP d/t dec sex hormones
High pain threshold
MSK S/S Prader WIlli
scoliosis, kyphosis, foot pronation, osteoporosis
Difficulties with balance/coordination and motor planning, decreased strength
Obesity related complications (type 2 diabetes, heart disease, stroke)
Medications for prader willi
Growth hormone, sex steroids, none effective for hyperphagia.
Clinical S/suggesting lesion in the hypothalmis and ANS
Altered respiration response, temp regulation, dc metabolic rate, altered BP response, increase pain tol, poor satiety rec, diminished salivation, excessive daytime sleepiness
What is Angelman Syndrome?
Microdeletion of maternal chromosome 15 UBE3A protein (important for synaptic connections in the brain) Freq misdiagnosed as CP or autism
S/S Angelman’s syndrome
Developmental delay, ataxic gait/tremulous movement of limbs (quick & jerky)
Hypermotoric (frequent laughing, smiling, hand flapping, waving), non-verbal or minimal use of words, short attention span.
Seizures
microcephaly
Hypopigmentation