General guidelines for intellectual and developmental disabilities

A developmental disability, according to the Developmental Disabilities Assistance and Bill of Rights Act, 2000; is defined as a severe, chronic disability which:*originated at birth or during childhood,*is expected to continue indefinitely, and*substantially restricts the individual's functioning in several major life activities.

*and are likely to be lifelong

More specifically,*mental or physical impairment or a combination of both*is manifested before the person attains age 22*results in substantial functional limitations in three or

more of the following areas of major life activity:*self-care*receptive and expressive language*learning*mobility*self-direction*capacity for independent living, and*economic self-sufficiency;

*except that such term when applied to infants and young children means individuals from birth to age five, inclusive, who have substantial developmental delay or specific congenital or acquired conditions with a high probability of resulting in developmental disabilities if services are not provided.

*Causes

can occur before, during or after birth. *before birth : genetic problems, poor prenatal care, or exposure of the fetus to toxins including drugs and alcohol.

*During birth :Difficulties during birth, restricted oxygen supply to the infant or

*after birth :accidents after birth can also cause traumatic brain injury resulting in developmental disabilities.

*Longer-term postnatal causes include malnutrition and social deprivation.

*Types of disabilities

Examples of developmental disabilities include:* Behavior disorders* Brain injury* Cerebral palsy* Down syndrome* Fetal alcohol syndrome* Intellectual disability* Spina Bifida

* Physical defects /dysfunction or motor problems: poliomyelitis, cerebral palsy, loss of limbs, motor neuron diseases, neuro muscular disorders, muscular dystrophy

* Sensory deficits : partial or complete blindness, deafness* Intelligence / cognitive deficits : mental retardation, slow learners* Emotional disturbances* Learning problems : dyslexia, dysgraphia, dyscalculia* Speech and language disorders: articulation, voice pitch, loudness, rhythm

stuttering, expressing* Pervasive developmental disorders :Autism, Asperger syndrome

*Intellectual and Developmental Disabilities (IDD)

Developmental Disabilities is an umbrella term that includes intellectual disability but also includes other disabilities that are apparent during childhood. 

Intellectual disability encompasses the “cognitive” part of this definition, that is, a disability that is broadly related to thought processes.

starts any time before a child turns 18 and is characterized by problems with both:>Intellectual functioning or intelligence: include the ability to learn, reason, problem solve, and other skills; and>Adaptive behavior: which includes everyday social and life skills.

"IDD" is the term often used to describe situations in which intellectual disability and other disabilities are present.3

Because intellectual and other developmental disabilities often co-occur, intellectual disability professionals often work with people who have both types of disabilities.

*“An intellectual disability, formerly referred to as “mental retardation”

*term used when a person has certain limitations in mental functioning and skills such as communicating, self care and social skills.

*These limitations cause a child to learn and develop more slowly than a typical child.

*Its severity is determined by the discrepancy between the individual's capabilities in learning and in and the expectations of the social environment.

(Project IDEAL, 2008)

The Nature of Intellectual Disability

Severity of ID based on the levels of intellectual functioning

• An intelligence quotient (IQ) is a total score derived from one of several standardized tests designed to assess human intelligence.

• Lewis M. Terman revised the Simon-Binet IQ Scale in 1916 and published the Stanford Revision of the Binet-Simon Scale of Intelligence (also known as the Stanford-Binet). The following scale resulted for classifying IQ scores:

IQ ScaleOver 140 - Genius or almost genius 120 - 140 - Very superior intelligence 110 - 119 - Superior intelligence 90 - 109 - Average or normal intelligence 80 - 89 - Dullness 70 - 79 - Borderline deficiency in intelligenceUnder 70 - Feeble-mindedness

*Low IQ & Mental RetardationAn IQ under 70 is considered as "mental retardation" or limited mental ability. The severity of the mental retardation is commonly broken into 4 levels:

50-70 - Mild mental retardation (85%)35-50 - Moderate mental retardation (10%)20-35 - Severe mental retardation (4%)IQ < 20 - Profound mental retardation (1%)

*High IQ & Genius IQGenius or near-genius IQ is considered to start around 140 to 145. Less than 1/4 of 1 percent fall into this category. Here are some common designations on the IQ scale:

115-124 - Above average125-134 - Gifted135-144 - Very gifted145-164 - Genius165-179 - High genius180-200 - Highest genius

*“Significantly sub-average general intellectual functioning, existing concurrently with deficits in adaptive behavior and manifested during the developmental period, that adversely affects a child’s educational performance.”

IDEA (Individuals with Disabilities Education Act)

Definitions for Intellectual Disability

*“Characterized by significant limitations both in intellectual functioning and adaptive behavior, which covers many everyday social and practical skills. The disability originates before the age of 18.”

AAIDD (American Association of Intellectual and Developmental Disabilities, 2006)

Mental Retardation/ Intellectual Disability in DSM – IV – TR is an Axis II Disorder criteria that includes:* Intelligence Test Scores* Adaptive Functioning* Age of Onset (DSM-IV-TR, 2000)

The Nature of Intellectual Disability

Diagnostic and Statistical Manual of Mental Disorders (DSM)

*published by American Psychiatric Association (APA)

*offers a common language and standard criteria for the classification of mental disorders

*relied upon, by clinicians, researchers, psychiatric drug regulation agencies, health insurance companies etc..

*The DSM is now in its fifth edition, DSM-5, published on May 18, 2013.

*The International Statistical Classification of Diseases and Related Health Problems(ICD), produced by the (WHO) is the other commonly used manual for mental disorders but distinguished from the DSM in that it covers health as a whole

*While the DSM is the official diagnostic system for mental disorders in the US, ICD is used more widely in Europe and other parts of the world.

*All categories used in DSM -IV -TR are found in ICD -10, but not all ICD-10 categories are in DSM-IV-TR

*Multiaxial system of DSM -IV -TR

* Axis I Clinical disorders and other disorders that may be a focus of clinical attention

*Axis II Personality disorders and mental retardation

* Axis III General medical conditions* Axis IV Psychosocial and environmental problems

* Axis V Global Assessment of Functioning (GAF) Scale

15

*Multiaxial ClassificationAxis I

16

*Multiaxial ClassificationNote Global Assessment for Axis V

Develop. disDSM IV: Disorders Usually First Diagnosed in

Infancy, Childhood or Adolescence

1. Mental Retardation (Coded on Axis II)2. Learning Disorders3. Motor Skills Disorder4. Communication Disorders5. Pervasive Developmental Disorders6. Attention-Deficit and Disruptive Behavior

Disorders7. Feeding and Eating Disorders of Infancy or Early

Childhood8. Tic Disorders9. Elimination Disorders

5. DSM IV Diagnostic Criteria for Pervasive Developmental Disorders

childhood disintegrative disorder: • Children with this rare condition begin their

development normally in all areas, physical and mental.

• At some point, usually between ages 2 and 10, a child with this illness loses many of the skills he or she has developed.

• In addition to the loss of social and language skills, a child with disintegrative disorder may lose control of other functions, including bowel and bladder control.

Rett syndrome : • Children with this very rare disorder have the

symptoms associated with a PDD and also suffer problems with physical development.

• generally suffer the loss of many motor or movement skills -- such as walking and use of their hands -- and develop poor coordination.

• This condition has been linked to a defect on the X chromosome, so it almost always affects girls.

Pervasive development disorder, not otherwise specified (PDD-NOS): • referred to as a milder form of autism• This category is used to refer to children who have

significant problems with communication and play, and some difficulty interacting with others, but are too social to be considered autistic.

7. DSM IV Diagnostic Criteria for Feeding and Eating Disorders of Infancy or Early Childhood

A. Feeding disturbance as manifested by persistent failure to eat adequately with significant failure to gain weight or significant loss of weight over at least 1 month

B. The disturbance is not due to an associated gastrointestinal or other general medical condition (e.g., esophageal reflux)

C. The disturbance is not better accounted for by another mental disorder (e.g., Rumination Disorder) or by lack of available food

D. The onset is before age 6 years• Pica• Rumination Disorder• Feeding Disorder of Infancy or Early

childhood

8. Tic Disorders

*Tics are sudden twitches, movements, or sounds that people do repeatedly.  

*Tics that involve movements are called motor tics and those that are sounds are called vocal tics.

*People who have tics cannot stop their body from doing these things. For example, a person with a motor tic might keep blinking over and over again. Or, a person with a vocal tic might make a grunting sound unwillingly.

*common in children and may last for less than one year

*most notable tic disorder is Tourette’s syndrome, in which both physical (eyes blink) and verbal tics (grunting, coughing, throat clearing, swallowing, blowing, or sucking sounds.)occur in the same individual, often at the same time. 

*Transient tic disorder also involves both types of tics, but they often occur individually.

* In 2013, the Diagnostic and Statistical Manual, 5th Edition (DSM-5) renamed this disorder as provisional tic disorder

DSM IV Diagnostic Criteria for Tic Disorders

Tourette's Disorder

A. Both multiple motor and one or more vocal tics have been present at some time during the illness, although not necessarily concurrently. (A tic is a sudden, rapid, recurrent, nonrhythmic, stereotyped motor movement or vocalization.) 

B. The tics occur many times a day (usually in bouts) nearly every day or intermittently throughout a period of more than 1 year, and during this period there was never a tic-free period of more than 3 consecutive months. 

C. The onset is before age 18 years. 

D. The disturbance is not due to the direct physiological effects of a substance (e.g., stimulants) or a general medical condition (e.g., Huntington's disease or postviral encephalitis).

There are two types :• Encopresis is the repeated

passing of feces into places other than the toilet, such as in underwear or on the floor. This behavior may or may not be done on purpose.

• Enuresis is the repeated passing of urine in places other than the toilet.

9. Elimination Disorders

DSM IV Diagnostic Criteria for Other Disorders of Infancy, Childhood or Adolescence

Other Disorders of Infancy, Childhood, or Adolescence: •Selective Mutism• Separation Anxiety Disorder• Reactive Attachment Disorder of Infancy or Early Childhood • Stereotypic Movement Disorder• Disorder of Infancy, Childhood, or Adolescence NOS

•WHO Definition“a condition of arrested or incomplete development of the mind, which is especially characterized by impairment of skills manifested during the developmental period, which contribute to the overall level of intelligence, i.e., cognitive, language, motor, and social abilities”

•Core symptoms– Low intellectual functioning IQ <70 (i.e., 2 SD below mean)

AND– Impaired adaptive behavior

•Types: Mild ID (IQ 50-69), Moderate (IQ 35-49) Severe (IQ 20-34), Profound (IQ 0-20)

*Borderline Intellectual Functioning

Intellectual DisabilityThe Basics

*Speech*Perception*Cognition*Concentration*Memory

*Emotion*Movement*Behavior

Intellectual DisabilityClinical Symptoms

•Heterogeneous•Mild ID: no specific cause in 40% of cases

–Genetic causes, injury, infections, poor nutrition

•Marked ID: specific cause found more often–Genetic: Trisomy 21, Fragile X, single gene disorders

–Prenatal: fetal alcohol syndrome, maternal infection like HIV

–Perinatal: placental dysfunction, birth trauma, septicemia, jaundice

–Postnatal: brain infection, head injury

Intellectual DisabilityEtiology

PRENATAL CAUSES

PERINATAL CAUSES

POSTNATAL CAUSES

 1. Chromosomal Disorders

2. Inborn Errors of Metabolism

3. Developmental Disorders of Brain Formation

4. Environmental Influences

1. Anoxia (complete deprivation of oxygen)

2. Low birth weight (LBW)

3. Syphilis and herpes simplex

1. Biological

2. Psychosocial

3. Child Abuse and Neglect

Classifications of Intellectual Disability

PRENATAL CAUSES

1. Chromosomal

Disorders

*affects many parts of the body and range from relatively mild to severe.

*slow growth before and after birth leading to:• short stature• intellectual disability

(moderate to severe)• abnormalities of bones in

the arms, hands, and fingers.

• excessive body hair (hypertrichosis)

• problems with the digestive tract Cornelia de

Lange Syndrome

*distinctive facial features, including arched eyebrows that often meet in the middle (synophrys), long eyelashes, low-set ears, small and widely spaced teeth, and a small and upturned nose

*Some people with this condition are born with an opening in the roof of the mouth called a cleft palate.

*small head (microcephaly)*behavior problems*Involves heart defects*hearing loss*Manifest self-injurious behavior

*A piece of chromosome 5 is missing.

* Infants with this condition often have a high-pitched cry that sounds like that of a cat.

*delayed development, microcephaly, low birth weight, and weak muscle tone (hypotonia) in infancy.

*distinctive facial features, including widely set eyes (hypertelorism), low-set ears, a small jaw, and a rounded face.

*Some are born with a heart defect.*Difficulty swallowing and sucking*Hyperactive, aggressive, and repetitive movements Cri-du-Chat

Syndrome

*Also referred to as Trisomy 21*Usually not an inherited condition

*most common type of chromosomal disorder

*Developmental: short stature, delayed development, learning disability, or speech delay in a child

*Cognitive: intellectual disability or difficulty thinking and understanding

*Mouth: abnormally large tongue or displacement of the tongue, small mouth

Down’s Syndrome

• Eyes: lazy eye or spots

• Distinctive facial features, such as a flat face, small ears

• A short neck and short arms and legs.

• Low muscle tone and loose joints. Muscle tone usually improves by late childhood

Intellectual DisabilityConditions Associated with ID: Down

Syndrome

*A genetic condition in which a male is born with an extra copy of the X chromosome :XXY

* result of a random genetic error after conception.

*Sterility in men*Decreased IQ *Poor coordination*Skeletal abnormalities*Poor coordinationa

Klinefelter’s Syndrome

• have low testosterone and reduced muscle mass, facial hair and body hair.

• attention deficit hyperactivity disorder predominantly inattentive

• breast enlargement, delayed puberty, flaccid muscles, low-set ears, osteoporosis, reduced sex drive, or small penis

*caused especially by the absence of certain genes normally present on the copy of chromosome 15 inherited from the father

*learning difficulties*growth abnormalities*obsessive eating*weak muscle tone (hypotonia) Prader-Willi

Syndrome

*Beginning in childhood, affected individuals develop an insatiable appetite, which leads to chronic overeating (hyperphagia) and obesity.

*Sometimes obese also develop type 2 diabetes mellitus

*The leading genetic cause of obesity.

*At risk of developing a variety of other health problems: heart defects, kidney problems, scoliosis, etc.

*Both affected males and females have underdeveloped genitals.

* Puberty is delayed or incomplete, and most affected individuals are unable to have children (infertile).

* narrow forehead*almond-shaped eyes, *triangular mouth; *short stature; *small hands and feet. *Some people with Prader-Willi syndrome have unusually fair skin and light-colored hair.

*Significant externalizing behaviors (tantrums/ aggression/ passive aggression, etc

• genetic disorder associated with 3 major clinical elements: overproduction of uric acid, neurologic disability, and behavioral problems.

• occurs almost exclusively in males

• overproduction of uric acid :gouty arthritis (arthritis caused by an accumulation of uric acid in the joints), kidney and bladder stones.

• The neurologic disability include abnormal involuntary muscle movements, such as tensing of various muscles (dystonia), jerking movements (chorea), and flailing of the limbs (ballismus). Lesch-Nyhan Disease

•  behavioral problems include intellectual disability (mental retardation) and aggressive and impulsive behaviors.

• usually cannot walk, require assistance sitting, and generally use a wheelchair.

• Self-injury (including biting and head banging) is the most common and distinctive behavioral problem in individuals with Lesch-Nyhan syndrome.

*Fragile X Syndrome

*an inherited condition Associated with X chromosome in the 23rd pair of chromosomes that is abnormally susceptible to damage, especially by folic acid deficiency.

*Usually, males are more severely affected*causes a range of developmental problems including learning disabilities and cognitive impairment.

*Children affected have anxiety and hyperactive behavior such as fidgeting or impulsive actions, attention deficit disorder (ADD)

*About one-third of individuals with fragile X syndrome have features of autism spectrum disorders that affect communication and social interaction

*prominent jaws*macro-orchidism (large testes)*long and thin faces*long and soft ears and hands*enlarged heads

*Results from the absence of one of the X chromosomes (XO) in females

*Usually results in learning disability – not mental retardation

*Common problems are in spatial relations, mathematics, memory, attention, and social competence

*Physical development includes lack of secondary sex characteristics, sterility, and short stature

*webbing of the neck, puffiness or swelling of the hands and feet

*Associated with heart defects and kidney problems Turner’s Syndrome

*Caused by the absence of material on the seventh pair of chromosome.

*“elfin” facial features.*Their unusual sensitivity to sound makes them competent in music and language despite of their low IQ level.

*Identified medical concerns including cardiac, digestive, and feeding difficulties

William’s Syndrome

*Wide range of IQ, but most have Mild Mental Retardation to Borderline Intellectual Functioning

*Overly friendly in their interactions *Highly anxious *Well-developed vocabulary but difficulty with reciprocal

conversation

*An ability to learn to read over time and with strength in phonics-based spelling

*Limited visual-spatial development and poor visual-motor skills (e.g., handwriting problems)

*Limited mathematical and numerical knowledge

PRENATAL CAUSES

2. Inborn Errors of

Metabolism

*Galactosemia - inability of the body to use simple sugar galactose

*Hunter Syndrome – defective breakdown of chemical mucopolysaccharide.

*Phenylketonuria (PKU) – inability of the body to convert phenylalanine to tyrosine

*Tay-Sachs Disease – absence of Hex-A enzyme.

Can be prevented through an early detection (e.g. newborn screening) and can be treated by providing a special diet program.

PRENATAL CAUSES

3. Developmental Disorders of

Brain Formation

*microcephaly usually is the result of the brain developing abnormally in the womb or not growing as it should after birth.

*The intellectual disability usually ranges from severe to profound.

*There is no specific treatment and life expectancy is low.

Microcephalus

*causes

*Craniosynostosis. The premature fusing of the joints (sutures) between the bony plates that form an infant's skull keeps the brain from growing..

*Chromosomal abnormalities. Down syndrome*Decreased oxygen to the fetal brain (cerebral anoxia).

* Infections of the fetus during pregnancy.  German measles (rubella), chickenpox (varicella) etc

*Exposure to drugs, alcohol or certain toxic chemicals in the womb. 

*Severe malnutrition*Uncontrolled phenylketonuria (fen-ul-kee-toe-NU-ree-uh), also known as PKU, in the mother. PKU is a birth defect that hampers the body's ability to break down the amino acid phenylalanine.

*Results from an accumulation of cerebrospinal fluid inside or outside the brain.

*water on the brain.*The degree of intellectual disability depends on how early the condition is diagnosed and treated. Hydrocephalus

(Hallahan & Kauffman,2003)

Causes:• blockage develops that prevents CSF

from flowing normally

• there is a decrease in the ability of blood vessels to absorb it

• brain produces an excess amount of it

• Too much of this fluid puts your brain under too much pressure causing brain swelling, which can damage your brain tis

Underlying Causes

starts before a baby is born. This can result from:• a birth defect in which the spinal column doesn’t

closea genetic abnormality• certain infections that occur during pregnancy,

such as rubella

This condition can also occur in infants, toddlers, and older children due to:• central nervous system infections such as

meningitis, especially in babies• bleeding in the brain during or shortly after

delivery, especially in babies born prematurely• injuries that occur before, during, or after delivery• head trauma• central nervous system tumors

PRENATAL CAUSES

4.Environmental Influences

*Maternal Malnutrition and Infection

*Fetal Alcohol Syndrome (FAS)

*Lead exposure*Illicit drug exposure*Exposure to Radiation

*Rubella (German measles)

(Hallahan & Kauffman,2003) (Piearangelo & Giuliani, 2007)

PERINATAL CAUSES

*Anoxia (deprivation of oxygen)

*Low birth weight (LBW)

*Syphilis and herpes simplex

PERINATAL CAUSES

(Hallahan & Kauffman,2003)

POSTNATAL CAUSES

1.Environmental and

Psychosocial Problems

*Nutritional Problems*Adverse living conditions

*Inadequate health care

*Lack of early cognitive stimulation

*Child abuse and neglect

*Traumatic Brain Injury

*Meningitis or Encephalitis

*Lead Poisoning

* Developmentally Disabled Assistanceand Bill of Rights Act

(1975)

I. Persons with developmental disabilities have a right to appropriate treatment, services and habilitation for such disabilities.II. The Federal Government and the States both have an obligation to assure that public funds are not provided to any institutional or other residential programs for persons with developmental disabilities that—

A. Does not provide treatment services, and habilitation which is appropriate to the needs to such persons or

B. Does not meet the following minimum standards:1. Provision of a nourishing, well-balanced daily diet to the persons with developmental disabilities served by the program2. Provision to such persons of appropriate and sufficient medical and dental services3. Prohibition of the use of physical restraint on such persons unless absolutely necessary and prohibition of the use of such restraint a punishment or a s a substitute for a habilitation program4. Prohibition on the excessive use of chemical restraints on such persons and the use of such restraints as punishment or as a substitute for a habilitation program or in quantities that interfere with services, treatment, or habilitation for such persons.5.Permission for close relatives of such persons to visit them at reasonable hours without prior notice6. Compliance with adequate fire and safety standards as may be promulgated by the Secretary of Health, Education, & Welfare.

* Americans with Disabilities Act1990 (Public Law 101-336)

Purposes:

 

I.  to  provide  a  clear  and  comprehensive  national  mandate  for  the  elimination  of  discrimination against individuals with disabilities; II to provide clear, strong, consistent enforceable standards addressing discrimination against individuals with disabilities; III. to ensure that the Federal Government plays a central role in enforcing the standards established in this Act on behalf of individuals with disabilities; IV. to invoke the sweep of congressional authority, including the power to enforce the Fourteenth Amendment and to regulate commerce, in order to address the major areas of discrimination faced day to day by people with disabilities

* Reasons fro the ebb and flow of mental health ideologyCaplan (1969)

Acceptance of ideas has little to do with their intrinsic value.  Community-oriented reforms did not succeed in the past for the following reasons:

 1. A shortage of manpower to implement programs, which in partreflects inadequate training facilities.

2. A shortage of resources associated with the low priority accordedthe mentally ill by the public and their legislators.

3. Theories concerning the etiology and treatment of mental disorder favored exciting new ideas and fads. At the turn of the century, the intellectual and scientific community was buzzing with new ideas in neuropathology and psychoanalysis. Optimistic treatment mo dalities linking patients back to their home communities were considered unrealistic and "old-fashioned.“

4. The naivete and lack of understanding of psychiatrists about prob lems of community dynamics that complicated the implementa tion of their programs. Theories of psychic functioning cannot be formulated in a vacuum. Factors in the social, political, economic, and scientific milieu affect the manner in which theories are developed as well as the success of their implementation.

* Reasons fro the ebb and flow of mental health ideologyCaplan (1969)

Acceptance of ideas has little to do with their intrinsic value.  Community-oriented reforms did not succeed in the past for the following reasons:

5. The tendency of psychiatrists to embrace panaceas and to hail each change as the solution to all difficulties. The system was oversold to the public and to fellow professionals. When shortcomings were revealed, there was a pendulum swing in the opposite direction with the entire system being jettisoned.

6. When public complaints about the failure of optimistic promisesstarted to surface, psychiatrists tended to deal with such outside pressure by evasion rather than by direct confrontation. Psychia trists retreated into their professional guild and lost touch with the realities of community life.

7. Faddish theories were entirely untested. By the time the shortcomings of expensive plans had been realized, so much propaganda and money had been spent that it was difficult to abandon or replace them.