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PRESENTER:
Dr. Sunil Suthar
MODERATOR:
Dr.Yogesh Satija
INTELLECTUAL DISABILITY (ID)
īƒ˜ Significant limitations both in intellectual functioning
and in adaptive behavior as expressed in conceptual,
social, and practical adaptive skills.
īƒ˜ Prevalence
īƒ˜ 1-3% of population
īƒ˜ Slightly more males than females
īƒ˜ More prevalent in lower SES and in minority groups
īƒ˜ especially for mild ID
īƒ˜ no differences for more severe levels
ICD-10 DIAGNOSTIC CRITERIA FOR
MENTAL RETARDATION
CLASS DEGREE IQ
F70 Mild mental retardation 50–69
F71 Moderate mental retardation 35–49
F72 Severe mental retardation 20–34
F73 Profound mental retardation <20
F78 Other mental retardation Sensory, physical, behavioral
impairments preclude standardized IQ testing.
F79 Unspecified mental retardation
DSM-IV-TR DIAGNOSTIC CRITERIA FOR
MENTAL RETARDATION
A) Significantly subaverage intellectual functioning: an IQ of approximately 70
or below on an individually administered IQ test (for infants, a clinical
judgment of significantly subaverage intellectual functioning).
B) Concurrent deficits or impairments in present adaptive functioning (i.e.,
the person's effectiveness in meeting the standards expected for his or her
age by his or her cultural group) in at least two of the following areas:
īƒ˜ communication, self-care, home living, social/interpersonal skills, use of
community resources, self-direction, functional academic skills, work, leisure,
health, and safety.
C) The onset is before age 18 years.
Features of mild, moderate, severe and
profound Intellectual Disability
mild moderate Severe/profound
IQ range 50-69 35-49 <35
% of cases 85% 10% 5%
Ability to self care Independent Need some help Limited
Language Reasonable Limited Basic or none
Reading and
writing
Reasonable Basic Minimal or none
Ability to work Semiskilled Unskilled,
supervised
Supervised basic
task
Social skill Normal Moderate Few
Physical problems Rare Sometimes Common
Aetiology
discovered
Sometimes Often Usually
Academic skill 6th grade or higher 2nd to 3rd grade -
Causes of Intellectual Disability :
ī‚— Prenatal 4-28%
ī‚— Natal 2-10%
ī‚— Postnatal 3-12%
ī‚— Unknown 30-50%
īƒ˜ Downs syndrome,
īƒ˜ Fragile X syndrome,
īƒ˜ Klinefelters syndrome
Chromosomal
disorders
Prenatal
īƒ˜ Inborn errors of metabolism:
galactosemia, phenylketonuria.
īƒ˜ Brain malformations :
genetic microcephaly,
hydrocephalus.
Single gene
disorders
īƒ˜ Deficiencies :
iodine deficiency
folic acid deficiency
īƒ˜ Using substances
alcohol
īƒ˜ Exposure to other harmful
chemicals
pollutants,
heavy metals,
harmful medications
īƒ˜ Infections e.g. TORCH HIV
Adverse material /
environmental
influences
īƒ˜ Complications of pregnancy,
īƒ˜ Diseases in mother such as heart and
kidney disease and diabetes,
īƒ˜ Placental dysfunction
Third trimester
( late pregnancy)
Natal
īƒ˜ Severe prematurity,
īƒ˜ Very low birth weight,
īƒ˜ Birth asphyxia
īƒ˜ Difficult and/or complicated delivery
īƒ˜ Birth trauma
Labour
(during delivery)
īƒ˜ Septicemia,
īƒ˜ Severe jaundice,
īƒ˜ Hypoglycemia
Neonatal
(first four weeks
of life)
īƒ˜ Brain infections such as
Japanese encephalitis,
Bacterial meningitis
īƒ˜ Head injury
īƒ˜ Chronic lead exposure
īƒ˜ Severe and prolonged malnutrition
īƒ˜ Gross understimulation
īƒ˜ Cretinism
Postnatal
(in infancy
and
childhood)
Representative Sample of Intellectual Disability
Syndromes and Behavioral Phenotypes
Syndrome Pathophysiology Clinical Features, Behavioral Phenotype
Down
syndrome
(m/c)
Trisomy 21,
95% non-
disjunction
īļHypotonia, upward-slanted palpebral fissures, midface
depression, flat, wide nasal bridge, simian crease, short stature,
increased incidence of thyroid abnormalities and congenital heart
disease
īļModerate intellectual disability, Passive, affable (2%–5%),
hyperactivity in childhood, stubborn, verbal > auditory processing,
increased risk of depression and dementia of the Alzheimer type in
adulthood
Fragile X
syndrome
Inactivation of
FMR1 gene at
Xq27.3 due to CGG
base repeats,
methylation;
īļLong face, large ears, midface hypoplasia, high, arched palate,
short stature, macro-orchidism, mitral valve prolapse, joint laxity,
strabismus
īļinattention, anxiety, stereotypies, speech and language delays, IQ
decline, gaze aversion, social avoidance, shyness, irritability,
learning disorder in some females, mild intellectual disability in
affected females, moderate to severe in males, verbal IQ >
performance IQ
DOWN SYNDROME
Journal of Human Genetics (2010)
Other syndromes associated with intellectual
disability:-
īƒŧ Phenylketonuria
īƒŧ Williams' syndrome
īƒŧ Tuberous sclerosis complex (TSC) 1 and 2
īƒŧ Angelman's syndrome
īƒŧ Cornelia de Lange syndrome
īƒŧ Cri-du-chat syndrome
īƒŧ Smith-Magenis syndrome
īƒŧ Rubinstein-Taybi syndrome
īƒŧ Neurofibromatosis type 1 (NF1)
īƒŧ Lesch-Nyhan syndrome
īƒŧ Galactosemia
īƒŧ Hurler's syndrome
īƒŧ Hunter's syndrome
īƒŧ Velocardiofacial syndrome, DiGeorge's syndrome (“CATCH
22”)
īƒŧ Prader-Willi syndrome
īƒŧ Cerebral palsyâ€Ļâ€Ļâ€Ļ.
Elements Of Clinical Evaluation Of Patient With
Intellectual Disability (Mental Retardation)
īƒŧClinical history
īƒŧFamily pedigree (three generation)
īƒŧPsychiatric Interview
īƒŧPhysical examination
īƒŧNeurological examination
īƒŧLaboratory examination
īƒŧNeuroimaging
īƒŧPsychological assessment
History Taking:
ī‚— Most often obtained from the parents or the caretaker.
ī‚— Family history of mental retardation; psychiayric illness;
consanguinity of the parents; and hereditary disorders.
ī‚— Assesses the overall level of functioning and intellectual capacity of
the parents and the emotional climate of the home.
ī‚— Prenatal, Natal, Postnatal :-parental age, recurrent abortion,
congenital anomalies, apgar scores at 5min, newborn weight, length
and head circumference, weight gain.
ī‚— Childhood history:- CNS symptoms such as cerebral palsy, epilepsy
or autism, growth chart, developmental milestones,
Psychiatric Interview
īƒ˜ The interviewer should not be guided by the patient's mental age, which
cannot fully characterize the person.
īƒ˜ When addressed as if they were children, some retarded persons become
justifiably insulted, angry, and uncooperative. Passive and dependent persons,
alternatively, may assume the child's role that they think is expected of them.
īƒ˜ Patient's verbal abilities, including receptive and expressive language, should
be assessed as soon as possible by observing the communication between the
caretakers and the patient and by taking the history.
īƒ˜ Communication has to be at the patient's level, being concrete and using
simple words, short sentences.
Psychiatric Interview
īƒ˜ If the patient uses sign language, the caretaker may have to stay during the
interview as an interpreter.
īƒ˜ Retarded persons often have the lifelong experience of failing in many areas,
and they may be anxious about seeing an interviewer.
īƒ˜ The interviewer and the caretaker should attempt to give such patients a
clear, supportive, concrete explanation of the diagnostic process, particularly
patients with sufficiently receptive language.
īƒ˜ Giving patients the impression that their bad behavior is the cause of the
referral should be avoided.
īƒ˜ Support and praise should be offered .
Psychiatric Interview
īƒ˜ Leading questions should be avoided, because retarded persons may be
suggestible and wish to please others.
īƒ˜ The patient's control over motility patterns should be ascertained, and
clinical evidence of distractibility and distortions in perception and memory
may be evaluated.
īƒ˜ The use of speech, reality testing, and the ability to generalize from
experiences should be noted.
īƒ˜ In general, the psychiatric examination of a retarded person should reveal
how the patient has coped with the stages of development..
Psychiatric disorders among people with ID.
ī‚— Prevalence higher than general population.
ī‚— Delusions, hallucinations and obsession may not be easily
reconized in people who have limited language development
and can not describe them.
ī‚— More emphasis is given to behaviour.
ī‚— Lower the IQ , greater the difficulty in diagnosing psychiatric
disorder.
Psychiatric disorders among people with ID.
1. Schizophrenia –
īƒ˜ Prevalence 3%
īƒ˜ If IQ <45 it is difficult to diagnose schizophrenia, In these cases the
diagnosis of psychosis NOS should be considered .
īƒ˜ Some individuals display presumptive evidence of response to
hallucinations (e.g., striking or shouting at empty space, throwing
imaginary peers from furniture) or adopt catatonic postures that can appear
to be psychotic in origin.
īƒ˜ Some of the symptoms of underlying brain damage such as stereotyped
movements ands social withdrawal may wrongly suggest schizophrenia.
īƒ˜ It is always necessary to compare behaviour before and after the onset of
suspected psychiaric disorder.
Psychiatric disorders among people with ID.
2. Mood disorder-
īą Depression- diagnosis has to be made mainly on appearance of sadness ,
changes in appatite and sleep, and behavioural changes of retardation or
agitation.
īą Mania- has to be diagnosed mainly from over activity and behavioural
signs of excitement, irritability, or nervousness.
īą d/d- thyroid dysfunction (especialy in down syndrome).
3. Anxiety disorder-
īą Frequent at the time of stress,
īą Post traumatic stress disorder have been reported in people with ID who
have suffered physical or sexual abuse.
īą Common symptoms of anxiety in the population with intellectual
disability include aggression, agitation, compulsive or repetitive
behaviors, self-injury, and insomnia. Panic may be expressed as agitation,
screaming, crying, or clinging.
Psychiatric disorders among people with ID.
4. Eating Disorders- Pica is perhaps the most common eating disorder
among persons with intellectual disability. Over eating and obesity are features of
Prader-Willi syndrome.
5. Dementia- adult with down syndrome are more likely to develop
dementia than general population.
6. Attention-Deficit/Hyperactivity Disorder-
īƒ˜ The rates of ADHD in individuals with intellectual disability are estimated to
be between 9 and 18 percent.
īƒ˜ Diagnosis of ADHD is based on developmental considerations, namely,
hyperactivity, impulsivity, and inattention significantly greater than that
expected for a given developmental age, and thus the threshold for diagnosis
in persons with severe to profound intellectual disability should be elevated.
Psychiatric disorders among people with ID.
7. Oppositional Defiant Disorder/Conduct Disorder-
īƒ˜ Affect about 30 per cent of young people with mild/borderline intellectual
disability, More in males.
īƒ˜ Not applicable in non-verbal children with a more severe level of ID.
8. Tic disorders-
īƒ˜ Can be difficult to differentiate from stereotypic movements and self-stimulating
behaviour, particularly when the patient is non-verbal and cannot explain that the
tic is in response to an irresistible urge which can, however, be delayed.
īƒ˜ Tics such as facial grimace or vocalization are usually less complex body movements
and do not appear to be intentional as in the case with stereotypic movements.
9. Stereotypic movement disorder-
īƒ˜ Persistent driven non-functional motor behaviour which interferes with daily
activities or causes injury , requiring treatment, occurs in 2 to 3 per cent of young
people with more severe levels of intellectual disability.
Psychiatric disorders among people with ID.
10. Pervasive developmental disorders-
īƒ˜ About 75 to 80 per cent of children with autism also have intellectual disability.
īƒ˜ Autism is associated with high levels of emotional and behavioural problems
and burden of care.
īƒ˜ The diagnosis of Asperger's disorder is excluded by the presence of mental
retardation and delayed and deviant language development.
īƒ˜ They (Children with intellectual disability) can be differentiated from those who
have autism because
īƒŧ They show an ability and interest to communicate,
īƒŧ Use gesture and imitation,
īƒŧ Show an interest in reciprocal and imitative play
īƒŧ Interact responsively and with emotion in a manner appropriate to their
developmental level
PHYSICAL EXAMINATION
īƒ˜ Size of the child and his or her trunk and extremities should also be explored.
īƒ˜ Examine for any skin pigment abnormalities -
īƒ˜ Tuberous sclerosis- patches of hypopigmentation
īƒ˜ Phenylketonuria- decreased skin pigment
īƒ˜ Neurofibromatosis- cafÊ au lait spots (localized area of hyperpigmentation)
īƒ˜ Sturge-Weber syndrome -A unilateral port wine colored lesion over one side of the
face.
Tuberous sclerosis
ADENOMA SEBACEUM
ASH LEAF MACULES
īƒ˜ The circumference of the head should be measured
as part of the clinical investigation.
īƒ˜ Size of the head offer clues to a variety of conditions,such as microcephaly,
hydrocephalus, and Down syndrome
ī‚— Facial signs of mental retardation :
Hypertelorism, a flat nasal bridge, prominent eyebrows, epicanthal folds,
corneal opacities, retinal changes, low-set and small or misshapen ears, a
protruding tongue, and a disturbance in dentition
PHYSICAL EXAMINATION
PHYSICAL EXAMINATION
ī‚— Dull appearance-can be misleading
ī‚— A high-arched palate, the size of the thyroid gland,
ī‚— Dermatoglyphics may offer another diagnostic tool, because
uncommon ridge patterns and flexion creases on the hand are often
found in persons who are retarded.
ī‚— Assessment o Abdominal examination feeling for an enlarged liver or
spleen which may suggest galactosemia, Hurler's disease, Gaucher's
disease
NEUROLOGICAL EXAMINATION
īƒ˜ Up to 10 percent are hearing impaired, ranging from cortical deafness
to mild hearing deficits.
īƒ˜ Visual disturbances can range from blindness to disturbances of spatial
concepts, design recognition, and concepts of body image.
īƒ˜ seizure disorders occur in about 10 percent of all mentally retarded
persons and in one third of those with severe retardation.
īƒ˜ When neurological abnormalities are present, their incidence and
severity generally rise in direct proportion to the degree of retardation.
NEUROLOGICAL EXAMINATION
īƒ˜ About 75 percent of all children with cerebral palsy have intellectual
disability.
īƒ˜ Disturbances in motor areas are manifested in abnormalities of muscle
tone (spasticity or hypotonia), reflexes (hyperreflexia), and involuntary
movements (choreoathetosis).
īƒ˜ The infants with the poorest prognosis are those who manifest a
combination of inactivity, general hypotonia, and exaggerated response
to stimuli
īƒ˜ In older children, hyperactivity, short attention span, distractibility,
and a low frustration tolerance are often signs of brain damage.
NEUROIMAGING
īą Skull X-rays –useful in diagnosis of:
ī‚§ craniosynostosis,
ī‚§ hydrocephalus,
ī‚§ and other disorders that result in intracranial calcifications (e.g.,
toxoplasmosis, tuberous sclerosis, cerebral angiomatosis, and
hypoparathyroidism).
īą MRI is regarded as superior to CT.
īą CT may be more useful when the intent is to look for possible:
ī‚§ Intracranial calcification, for example, in tuberous sclerosis complex,
ī‚§ Abnormalities of the skull, as in craniosynostosis syndromes.
NEUROIMAGING
Conditions in which neuroimaging is indicated:
īą Patients in which there is little doubt that structural brain
abnormalities will be present, an imaging study may still be useful to
serve as a baseline against which to measure progression.
īą Other conditions include
īą Patients with seizures,
īą Microcephaly or macrocephaly,
īą Loss of previously acquired skills,
īą Neurologic signs such as spasticity, dystonia, or altered reflexes.
īą EEG in seizure disorder.
LABORATORY EXAMINATION
ī‚— In the absence of specific indications other than mere intellectual
disability, metabolic testing is likely to be of little additional
diagnostic value.
ī‚— Testing in the presence of clinical suspicion may includeīƒ 
īƒŧ plasma and urine amino and organic acid studies,
īƒŧ acid/base balance,
īƒŧ thyroid function tests,
īƒŧ lysosomal enzyme analysis,
īƒŧ plasma and urine carnitine analyses,
īƒŧ plasma very long chain fatty acids, among others-phenylketonuria,
hypothyroidism, and galactosemia.
īƒŧ Cytogenetics for karyotyping - e.g. looking for genetic syndromes e.g. Down's
syndrome, Fragile-X syndrome, Klinefelter's syndrome.
Clinical Findings and Laboratory Abnormalities That
Increase Suspicion for Underlying Metabolic Disorder
īƒ˜ Abnormal sexual differentiation
īƒ˜ Arachnodactyly
īƒ˜ Cataracts, corneal clouding
īƒ˜ Disturbance of consciousness, lethargy
īƒ˜ Hypo or depigmented skin, hyperkeratotic plaques on soles and palms
īƒ˜ Dysmorphic features Failure to thrive, feeding abnormalities, vomiting
īƒ˜ Growth abnormality
īƒ˜ Hair abnormalities (fragility)
īƒ˜ Hypotonia, dystonia, ataxia Macrocephaly, microcephaly
Neurodegenerative symptoms (regression or loss of skills)
īƒ˜ Organomegaly , Retinal pigmentation
īƒ˜ Seizures, specific electroencephalogram abnormalities
īƒ˜ Self-injurious behavior – lesh nyhan syndrome
īƒ˜ Skeletal malformations
īƒ˜ Hyperammonemia Hyperuricemia Hypo/hyperglycemia
,Hypocholesterolemia
PSYCHOLOGICAL ASSESSMENT
The intellectual functioning and adaptive behaviour in a
mentally retarded are assessed using a combination of test
and schedules. The important are:
a) Measurement of the overall level of general intellectual
functioning.(IQ)
b) Assessment of adaptive behaviour
c) Developmental assessment(DQ)
PSYCHOLOGICAL ASSESSMENT
ī‚— Copying geometric figures, the Goodenough Draw-a-Person Test, the Kohs
Block Test, and geometric puzzles all may be used as quick screening tests of
visual-motor coordination.
ī‚— The Gesell and Bayley scales and the Cattell Infant Intelligence Scale are most
commonly used with infants.
ī‚— For children, the Stanford-Binet Intelligence Scale and the third edition of the
Wechsler Intelligence Scale for Children (WISC-III) are those most widely used
.
ī‚— The tests often found useful in detecting brain damage are the Bender Gestalt
Test and the Benton Visual Retention Test .
ī‚— In addition, a psychological evaluation should assess perceptual, motor,
linguistic, and cognitive abilities.
IQ Tests For Diagnosing Intellectual
Disability
īą Wechsler Preschool and Primary Scale of Intelligence-Revised (Wechsler, 1989)
īą Wechsler Intelligence Test for Children-III (Wechsler, 1991)
īą Wechsler Adult Intelligence Scale-Revised (Wechsler, 1981)
īą Stanford-Binet Intelligence Scale: Fourth Edition (Thorndike, Hagen, and Sattler, 1986)
In INDIA –
A) Verbal test-
Binet Kamat test
Binet Kulshresta test
Malin's intelligence test for Indian children.
B) Nonverbal and performances tests :
Developmental screening test
Raven's progressive matrices
Seguin form board test
Gassel's drawing test
Malin's intelligence scale for Indian Children-performance scale
Alexander pass-along test
Draw-A-Man test
Koh's block design test
Common Measures of Adaptive Behavior
īą Vineland Adaptive Behavior Scales (Sparrow, Balla, and Cicchetti, 1984)
Age range : Birth to 18 yrs
Domains Tested: Communication: Receptive, Expressive, Written .
Daily Living Skills: Personal, Domestic, Community
Socialization: Interpersonal Relations, Play and Leisure, Coping Skill.
īą AAMR Adaptive Behavior Scales (Lambert, Nihira, and Leland, 1993)
Age range : 3 yrs to 18 yrs
Domains Tested: Independent Functioning, Physical development, Economic
Activity, School . Motor Skills: Fine, Gross.
īą Scales of Independent Behavior- Revised (Bruininks, Woodcock,
Weatherman, and Hill 1996)
īąBinet Simon Scale Hindi Adaptation (BSSHA–K) (Age group 2ÂŊ to 18
years.)
DEVELOPMENTAL ASSESSMENT
ī‚— This is based on combination of clinical experience and standardized
methods of measuring intelligence, language, motor performance, and
social skill.
ī‚— expressed in terms of developmental quotients(DQ's)
ī‚— Although IQ is the best general index of intellectual development, it is
not reliable in very young or among people who have severe to profound
degree intellectual disability.
ī‚— Commonly used instruments for Developmental assessment are in
India :-
īƒ˜ Bayley infant scales
īƒ˜ Gassell's developmental schedules
īƒ˜ NIMH development assessment schedules.
DIFFERENTIAL DIAGNOSIS
īƒ˜ Early infantile autism
īƒ˜ Attention deficit hyperactivity
īƒ˜ Child with hearing impairment
īƒ˜ Cultural deprivation and lack of stimulation
īƒ˜ Specific learning disabilities
īƒ˜ Childhood psychosis
īƒ˜ Child with visual handicap
īƒ˜ Children under the age of 18 years who meet the diagnostic criteria for
dementia and who have an IQ below 70 are given the diagnoses of
DEMENTIA AND INTELLECTUAL DISABILITY.
īƒ˜ Those whose IQs drop below 70 after the age of 18 years and who have new
onsets of cognitive disorders are not given the diagnosis of intellectual
disability but only the diagnosis of DEMENTIA.
TREATMENT
ī‚— Biological, psychological, social, and developmental
dimensions should all be considered when designing a
treatment plan for an individual with intellectual disability.
ī‚— Treatment plan includes attention to
īƒŧ Psychoeducational,
īƒŧ Psychotherapeutic, and
īƒŧ Psychopharmacologic interventions.
Parents Counselling :
Following points are noteworthy in this contest (Madhavan,1990).
ī‚— 1. The child's actual condition should be explained in simple words to the
parents.
ī‚— 2. Enough time must be taken while counseling.
ī‚— 3.Misleading, giving false information or building false hopes in parents must
be avoided.
ī‚— 4. Information regarding professional help for treating associated conditions
like seizures, hyperactivity, psychosis etc. must be made available to the
parents.
ī‚— 5. Attitude such over protection ie., doing everything for the child and
shielding them from any challenging situation, should be corrected as it
hinders the development of whatever capacities the child may have.
PARENTS COUNSELLING :
ī‚— 6. The attitude of rejection, that is ignoring the child thinking that he is good for nothing
should be changed so that the child can be helped to learn by systematic training.
ī‚— 7. The parents should be made aware of what they may expect of their child
ī‚— 8. Some patents suffer from guilt feeling assuming that they are responsible for their child's
condition. It should be explained that the condition is due to causes over which parents
have no direct control.
ī‚— 9. The counselor should explain the effectiveness and role of the parents and other family
members in training a mentally retarded.
ī‚— 10. Some parents believe that training a mentally retarded child needs specialized skills and
they may not be able to train their child. Parents should be explained that training a
mentally retarded child dose not need complex skills and repeated training in simple steps,
they can be taught.
ī‚— 11. Parents should be helped to learn the skills in training through demonstrations and
observations.
Individualized Educational Programming
ī‚— IEP is very much essential in training the retarded children because no
two retarded children can be taught the same programme. Since each
child differs from the other with regard to his/ her needs, strength and
weakness of the programme developed should be tailored to suit needs of
each child.
ī‚— components of the IEP are :
īƒ˜ Current level of functioning of the child in specific skills.
īƒ˜ Annual goals
īƒ˜ Short term objectives
īƒ˜ Methods of training
īƒ˜ Materials required for training
īƒ˜ Manpower
īƒ˜ Duration
īƒ˜ Terminal behaviour
īƒ˜ Evaluation for further programme planning
BEHAVIOURAL TREATMENTS
ī‚— The backbone of any treatment programme is
educational with formal instruction in the skills which includes:
ī‚— Independence and self-help skills-
īƒ˜ These include the basic skills of everyday life, such as feeding, dressing, or
managing stairs or, at a higher level, the use of public transport, and how to care
for clothes, shop, and budget.
īƒ˜ Acquiring these skills gives a sense of achievement as well as of increased
independence.
ī‚— Communication skills-
īƒ˜ Frustration of living in an uncomprehending world frequently contributes
to disturbance as the person falls back on various forms of attention-seeking
or violent behaviour to get their message across.
īƒ˜ It is helped by an easier and more effective means of communication.
BEHAVIOURAL TREATMENTS
īƒ˜ This may range from simple gestures (such as pulling at the trousers to
indicate the need for toileting), through a system of pointing to symbols
or pictures, to complex signing which can convey abstract concepts such
as emotional states.
īƒ˜ Language may be verbal or non-verbal, with a sign language such as
Makaton (a simplified form of standard sign language) being taught
simultaneously in both modalities.
īƒ˜ These usually reinforce each other so that a course in signing may
improve speech.
BEHAVIOURAL TREATMENTS
īƒ˜ Behavioral therapies are demonstrably effective in managing many
maladaptive behaviors, particularly aggression and self-injury, in persons
with intellectual disability.
īƒ˜ Teaching appropriate habits and skills. These can include basic skills such as
dressing, continence, communication, and establishing a normal pattern of
sleep; or, at a more sophisticated level, training in social skills, copingskills,
dating skills, and assertiveness.
īƒ˜ The unlearning of other maladaptive forms of behaviour.
īƒ˜ Positive reinforcement for desired behaviors and benign punishment (e.g.,
loss of privileges) for objectionable behaviors have been helpful.
Task Analysis
ī‚— To train a person in any skill, there are certain common basic principles
to be followed. Initially the skill must be broken down in to small
sequential steps. This is called 'Task Analysis'.
ī‚— For example, a simple task 'Wearing slippers' - the major sub tasks
(a) identifying one's own slippers,
(b) identifying right and left slipper
(c) inserting the correct foot in one slipper and
(d) inserting the other foot in its slipper. Thus one has to perform all of
these subtasks to complete the act of wearing slipper based on the task
analysis the child should be trained.
COGNITIVE THERAPY
īąCognitive therapy is usually used with people in the mild or
borderline range of retardation.
īąDispelling false beliefs and relaxation exercises with self-
instruction, has also been recommended for mentally retarded
persons who can follow the instructions.
īąProblem-solving skills are formally taught.
Vocational Training and Job
Placement :
ī‚— Like any other system of education and training, the ultimate goal in training a mentally
retarded person is to make him independent and earn his livelihood. Each mentally
retarded person must be assessed for his readiness for work and opportunity for
employment after training should be considered before training in a job-
A)Self employment : Envelope making, agarbathi and candle making, running small pan
shop , Dairy farms, poultry and agriculture are also good examples in this regard.
B)Sheltered workshop : Here, mentally retarded are trained in specific tasks matched for
their ability and they work under supervision. This requires a workshop with qualified
staff to train them. The products are made here on contract basis, where raw material are
received and finished products returned.
C)Open employment: The retarded person can be employed on certain routine repetitive
jobs like any other normal person and get paid in a similar manner. This includes office
boys, lift operators, restaurant table cleaners, gardeners etc.
PHARMACOTHERAPY
ī‚— Antidepressants- Cardiac anomalies are common in some
intellectual disability syndromes, and the anticholinergic side effects
of some medications may be particularly significant in persons with
Down syndrome; Due to lowering seizure threshold, clomipramine is
generally not a first-line treatment for compulsive behaviour.
ī‚— Anticonvulsants- carbamazepine (Tegretol) and valproic acid
(Depakine), have been used clinically for aggressive behavior in
children and adolescents. Persons with PWS treated with the
anticonvulsant topiramate, whose skin-picking behavior improved.
ī‚— Mood stabilizer -lithium has shown to be effective in reducing
outbursts of aggression, particularly where there is irritability and
explosiveness.
PHARMACOTHERAPY
ī‚— Antipsychotics- greater risk of developing tardive dyskinesia than the general
population.
Considerable evidence of effectiveness of risperidone in treating severe
disruptive behavior in children and adolescents with cognitive disability.
Trials have found that at daily doses ranging from .02 to .06 mg/kg, children
and adolescents with various disruptive behavior disorders, the majority of whom
also had attention deficit hyperactivity disorder, improved significantly.
ī‚— Psychostimulants- Despite reports of paradoxical responses to stimulant
medications in persons with intellectual disability, with higher-than-expected rates
of emergent motor tics and emotional lability, a growing body of literature
supports the use of stimulant drugs for the treatment of ADHD in the context of
intellectual disability.
PHARMACOTHERAPY
ī‚— Opioid Antagonists- Naltrexone is the opioid antagonist most widely used for
SIB, but the literature is mixed. Typical daily doses range from .5 to 2.0 mg/kg in
children and up to 200 mg in adults.
ī‚— Nootropics- drugs that positively affect cognition. Lobaugh and colleagues
recently reported the first controlled trial of piracetam in children with down
syndrome-
Piracetam was associated with a number of side effects, including aggression,
agitation, sexual arousal, poor sleep, and diminished appetite. There were no
consistent benefits of piracetam over placebo identifiable in this study.
Legislation
ī‚— In 1995. the ‘person with disability act’ came ino India.
This act envisages mandatory support for prevention, early detection,
education, employement and other facilities for the welfare of person
with diabilities in general and mental retardation in particular.
ī‚— in 1999 the ‘National Trust Act’ came in force. This Act proposes to
involve the parents of mentally challenged persons and voluntary
organizations in setting up and running a variety of services and
facilities with government funding.
ī‚—
ī‚— The IDEA’s (Individuals with Disabilities Education Act)disability
terms and definitions guide how States in their own turn define
disability and who is eligible for a free appropriate public
education under special education law.
Thank youâ€Ļ

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Intellectual disability by dr sunil

  • 2. INTELLECTUAL DISABILITY (ID) īƒ˜ Significant limitations both in intellectual functioning and in adaptive behavior as expressed in conceptual, social, and practical adaptive skills. īƒ˜ Prevalence īƒ˜ 1-3% of population īƒ˜ Slightly more males than females īƒ˜ More prevalent in lower SES and in minority groups īƒ˜ especially for mild ID īƒ˜ no differences for more severe levels
  • 3.
  • 4. ICD-10 DIAGNOSTIC CRITERIA FOR MENTAL RETARDATION CLASS DEGREE IQ F70 Mild mental retardation 50–69 F71 Moderate mental retardation 35–49 F72 Severe mental retardation 20–34 F73 Profound mental retardation <20 F78 Other mental retardation Sensory, physical, behavioral impairments preclude standardized IQ testing. F79 Unspecified mental retardation
  • 5. DSM-IV-TR DIAGNOSTIC CRITERIA FOR MENTAL RETARDATION A) Significantly subaverage intellectual functioning: an IQ of approximately 70 or below on an individually administered IQ test (for infants, a clinical judgment of significantly subaverage intellectual functioning). B) Concurrent deficits or impairments in present adaptive functioning (i.e., the person's effectiveness in meeting the standards expected for his or her age by his or her cultural group) in at least two of the following areas: īƒ˜ communication, self-care, home living, social/interpersonal skills, use of community resources, self-direction, functional academic skills, work, leisure, health, and safety. C) The onset is before age 18 years.
  • 6. Features of mild, moderate, severe and profound Intellectual Disability mild moderate Severe/profound IQ range 50-69 35-49 <35 % of cases 85% 10% 5% Ability to self care Independent Need some help Limited Language Reasonable Limited Basic or none Reading and writing Reasonable Basic Minimal or none Ability to work Semiskilled Unskilled, supervised Supervised basic task Social skill Normal Moderate Few Physical problems Rare Sometimes Common Aetiology discovered Sometimes Often Usually Academic skill 6th grade or higher 2nd to 3rd grade -
  • 7. Causes of Intellectual Disability : ī‚— Prenatal 4-28% ī‚— Natal 2-10% ī‚— Postnatal 3-12% ī‚— Unknown 30-50%
  • 8. īƒ˜ Downs syndrome, īƒ˜ Fragile X syndrome, īƒ˜ Klinefelters syndrome Chromosomal disorders Prenatal īƒ˜ Inborn errors of metabolism: galactosemia, phenylketonuria. īƒ˜ Brain malformations : genetic microcephaly, hydrocephalus. Single gene disorders īƒ˜ Deficiencies : iodine deficiency folic acid deficiency īƒ˜ Using substances alcohol īƒ˜ Exposure to other harmful chemicals pollutants, heavy metals, harmful medications īƒ˜ Infections e.g. TORCH HIV Adverse material / environmental influences
  • 9. īƒ˜ Complications of pregnancy, īƒ˜ Diseases in mother such as heart and kidney disease and diabetes, īƒ˜ Placental dysfunction Third trimester ( late pregnancy) Natal īƒ˜ Severe prematurity, īƒ˜ Very low birth weight, īƒ˜ Birth asphyxia īƒ˜ Difficult and/or complicated delivery īƒ˜ Birth trauma Labour (during delivery) īƒ˜ Septicemia, īƒ˜ Severe jaundice, īƒ˜ Hypoglycemia Neonatal (first four weeks of life)
  • 10. īƒ˜ Brain infections such as Japanese encephalitis, Bacterial meningitis īƒ˜ Head injury īƒ˜ Chronic lead exposure īƒ˜ Severe and prolonged malnutrition īƒ˜ Gross understimulation īƒ˜ Cretinism Postnatal (in infancy and childhood)
  • 11. Representative Sample of Intellectual Disability Syndromes and Behavioral Phenotypes Syndrome Pathophysiology Clinical Features, Behavioral Phenotype Down syndrome (m/c) Trisomy 21, 95% non- disjunction īļHypotonia, upward-slanted palpebral fissures, midface depression, flat, wide nasal bridge, simian crease, short stature, increased incidence of thyroid abnormalities and congenital heart disease īļModerate intellectual disability, Passive, affable (2%–5%), hyperactivity in childhood, stubborn, verbal > auditory processing, increased risk of depression and dementia of the Alzheimer type in adulthood Fragile X syndrome Inactivation of FMR1 gene at Xq27.3 due to CGG base repeats, methylation; īļLong face, large ears, midface hypoplasia, high, arched palate, short stature, macro-orchidism, mitral valve prolapse, joint laxity, strabismus īļinattention, anxiety, stereotypies, speech and language delays, IQ decline, gaze aversion, social avoidance, shyness, irritability, learning disorder in some females, mild intellectual disability in affected females, moderate to severe in males, verbal IQ > performance IQ
  • 13.
  • 14. Journal of Human Genetics (2010)
  • 15. Other syndromes associated with intellectual disability:- īƒŧ Phenylketonuria īƒŧ Williams' syndrome īƒŧ Tuberous sclerosis complex (TSC) 1 and 2 īƒŧ Angelman's syndrome īƒŧ Cornelia de Lange syndrome īƒŧ Cri-du-chat syndrome īƒŧ Smith-Magenis syndrome īƒŧ Rubinstein-Taybi syndrome īƒŧ Neurofibromatosis type 1 (NF1) īƒŧ Lesch-Nyhan syndrome īƒŧ Galactosemia īƒŧ Hurler's syndrome īƒŧ Hunter's syndrome īƒŧ Velocardiofacial syndrome, DiGeorge's syndrome (“CATCH 22”) īƒŧ Prader-Willi syndrome īƒŧ Cerebral palsyâ€Ļâ€Ļâ€Ļ.
  • 16. Elements Of Clinical Evaluation Of Patient With Intellectual Disability (Mental Retardation) īƒŧClinical history īƒŧFamily pedigree (three generation) īƒŧPsychiatric Interview īƒŧPhysical examination īƒŧNeurological examination īƒŧLaboratory examination īƒŧNeuroimaging īƒŧPsychological assessment
  • 17. History Taking: ī‚— Most often obtained from the parents or the caretaker. ī‚— Family history of mental retardation; psychiayric illness; consanguinity of the parents; and hereditary disorders. ī‚— Assesses the overall level of functioning and intellectual capacity of the parents and the emotional climate of the home. ī‚— Prenatal, Natal, Postnatal :-parental age, recurrent abortion, congenital anomalies, apgar scores at 5min, newborn weight, length and head circumference, weight gain. ī‚— Childhood history:- CNS symptoms such as cerebral palsy, epilepsy or autism, growth chart, developmental milestones,
  • 18. Psychiatric Interview īƒ˜ The interviewer should not be guided by the patient's mental age, which cannot fully characterize the person. īƒ˜ When addressed as if they were children, some retarded persons become justifiably insulted, angry, and uncooperative. Passive and dependent persons, alternatively, may assume the child's role that they think is expected of them. īƒ˜ Patient's verbal abilities, including receptive and expressive language, should be assessed as soon as possible by observing the communication between the caretakers and the patient and by taking the history. īƒ˜ Communication has to be at the patient's level, being concrete and using simple words, short sentences.
  • 19. Psychiatric Interview īƒ˜ If the patient uses sign language, the caretaker may have to stay during the interview as an interpreter. īƒ˜ Retarded persons often have the lifelong experience of failing in many areas, and they may be anxious about seeing an interviewer. īƒ˜ The interviewer and the caretaker should attempt to give such patients a clear, supportive, concrete explanation of the diagnostic process, particularly patients with sufficiently receptive language. īƒ˜ Giving patients the impression that their bad behavior is the cause of the referral should be avoided. īƒ˜ Support and praise should be offered .
  • 20. Psychiatric Interview īƒ˜ Leading questions should be avoided, because retarded persons may be suggestible and wish to please others. īƒ˜ The patient's control over motility patterns should be ascertained, and clinical evidence of distractibility and distortions in perception and memory may be evaluated. īƒ˜ The use of speech, reality testing, and the ability to generalize from experiences should be noted. īƒ˜ In general, the psychiatric examination of a retarded person should reveal how the patient has coped with the stages of development..
  • 21. Psychiatric disorders among people with ID. ī‚— Prevalence higher than general population. ī‚— Delusions, hallucinations and obsession may not be easily reconized in people who have limited language development and can not describe them. ī‚— More emphasis is given to behaviour. ī‚— Lower the IQ , greater the difficulty in diagnosing psychiatric disorder.
  • 22. Psychiatric disorders among people with ID. 1. Schizophrenia – īƒ˜ Prevalence 3% īƒ˜ If IQ <45 it is difficult to diagnose schizophrenia, In these cases the diagnosis of psychosis NOS should be considered . īƒ˜ Some individuals display presumptive evidence of response to hallucinations (e.g., striking or shouting at empty space, throwing imaginary peers from furniture) or adopt catatonic postures that can appear to be psychotic in origin. īƒ˜ Some of the symptoms of underlying brain damage such as stereotyped movements ands social withdrawal may wrongly suggest schizophrenia. īƒ˜ It is always necessary to compare behaviour before and after the onset of suspected psychiaric disorder.
  • 23. Psychiatric disorders among people with ID. 2. Mood disorder- īą Depression- diagnosis has to be made mainly on appearance of sadness , changes in appatite and sleep, and behavioural changes of retardation or agitation. īą Mania- has to be diagnosed mainly from over activity and behavioural signs of excitement, irritability, or nervousness. īą d/d- thyroid dysfunction (especialy in down syndrome). 3. Anxiety disorder- īą Frequent at the time of stress, īą Post traumatic stress disorder have been reported in people with ID who have suffered physical or sexual abuse. īą Common symptoms of anxiety in the population with intellectual disability include aggression, agitation, compulsive or repetitive behaviors, self-injury, and insomnia. Panic may be expressed as agitation, screaming, crying, or clinging.
  • 24. Psychiatric disorders among people with ID. 4. Eating Disorders- Pica is perhaps the most common eating disorder among persons with intellectual disability. Over eating and obesity are features of Prader-Willi syndrome. 5. Dementia- adult with down syndrome are more likely to develop dementia than general population. 6. Attention-Deficit/Hyperactivity Disorder- īƒ˜ The rates of ADHD in individuals with intellectual disability are estimated to be between 9 and 18 percent. īƒ˜ Diagnosis of ADHD is based on developmental considerations, namely, hyperactivity, impulsivity, and inattention significantly greater than that expected for a given developmental age, and thus the threshold for diagnosis in persons with severe to profound intellectual disability should be elevated.
  • 25. Psychiatric disorders among people with ID. 7. Oppositional Defiant Disorder/Conduct Disorder- īƒ˜ Affect about 30 per cent of young people with mild/borderline intellectual disability, More in males. īƒ˜ Not applicable in non-verbal children with a more severe level of ID. 8. Tic disorders- īƒ˜ Can be difficult to differentiate from stereotypic movements and self-stimulating behaviour, particularly when the patient is non-verbal and cannot explain that the tic is in response to an irresistible urge which can, however, be delayed. īƒ˜ Tics such as facial grimace or vocalization are usually less complex body movements and do not appear to be intentional as in the case with stereotypic movements. 9. Stereotypic movement disorder- īƒ˜ Persistent driven non-functional motor behaviour which interferes with daily activities or causes injury , requiring treatment, occurs in 2 to 3 per cent of young people with more severe levels of intellectual disability.
  • 26. Psychiatric disorders among people with ID. 10. Pervasive developmental disorders- īƒ˜ About 75 to 80 per cent of children with autism also have intellectual disability. īƒ˜ Autism is associated with high levels of emotional and behavioural problems and burden of care. īƒ˜ The diagnosis of Asperger's disorder is excluded by the presence of mental retardation and delayed and deviant language development. īƒ˜ They (Children with intellectual disability) can be differentiated from those who have autism because īƒŧ They show an ability and interest to communicate, īƒŧ Use gesture and imitation, īƒŧ Show an interest in reciprocal and imitative play īƒŧ Interact responsively and with emotion in a manner appropriate to their developmental level
  • 27. PHYSICAL EXAMINATION īƒ˜ Size of the child and his or her trunk and extremities should also be explored. īƒ˜ Examine for any skin pigment abnormalities - īƒ˜ Tuberous sclerosis- patches of hypopigmentation īƒ˜ Phenylketonuria- decreased skin pigment īƒ˜ Neurofibromatosis- cafÊ au lait spots (localized area of hyperpigmentation) īƒ˜ Sturge-Weber syndrome -A unilateral port wine colored lesion over one side of the face.
  • 29. īƒ˜ The circumference of the head should be measured as part of the clinical investigation. īƒ˜ Size of the head offer clues to a variety of conditions,such as microcephaly, hydrocephalus, and Down syndrome ī‚— Facial signs of mental retardation : Hypertelorism, a flat nasal bridge, prominent eyebrows, epicanthal folds, corneal opacities, retinal changes, low-set and small or misshapen ears, a protruding tongue, and a disturbance in dentition PHYSICAL EXAMINATION
  • 30. PHYSICAL EXAMINATION ī‚— Dull appearance-can be misleading ī‚— A high-arched palate, the size of the thyroid gland, ī‚— Dermatoglyphics may offer another diagnostic tool, because uncommon ridge patterns and flexion creases on the hand are often found in persons who are retarded. ī‚— Assessment o Abdominal examination feeling for an enlarged liver or spleen which may suggest galactosemia, Hurler's disease, Gaucher's disease
  • 31. NEUROLOGICAL EXAMINATION īƒ˜ Up to 10 percent are hearing impaired, ranging from cortical deafness to mild hearing deficits. īƒ˜ Visual disturbances can range from blindness to disturbances of spatial concepts, design recognition, and concepts of body image. īƒ˜ seizure disorders occur in about 10 percent of all mentally retarded persons and in one third of those with severe retardation. īƒ˜ When neurological abnormalities are present, their incidence and severity generally rise in direct proportion to the degree of retardation.
  • 32. NEUROLOGICAL EXAMINATION īƒ˜ About 75 percent of all children with cerebral palsy have intellectual disability. īƒ˜ Disturbances in motor areas are manifested in abnormalities of muscle tone (spasticity or hypotonia), reflexes (hyperreflexia), and involuntary movements (choreoathetosis). īƒ˜ The infants with the poorest prognosis are those who manifest a combination of inactivity, general hypotonia, and exaggerated response to stimuli īƒ˜ In older children, hyperactivity, short attention span, distractibility, and a low frustration tolerance are often signs of brain damage.
  • 33. NEUROIMAGING īą Skull X-rays –useful in diagnosis of: ī‚§ craniosynostosis, ī‚§ hydrocephalus, ī‚§ and other disorders that result in intracranial calcifications (e.g., toxoplasmosis, tuberous sclerosis, cerebral angiomatosis, and hypoparathyroidism). īą MRI is regarded as superior to CT. īą CT may be more useful when the intent is to look for possible: ī‚§ Intracranial calcification, for example, in tuberous sclerosis complex, ī‚§ Abnormalities of the skull, as in craniosynostosis syndromes.
  • 34. NEUROIMAGING Conditions in which neuroimaging is indicated: īą Patients in which there is little doubt that structural brain abnormalities will be present, an imaging study may still be useful to serve as a baseline against which to measure progression. īą Other conditions include īą Patients with seizures, īą Microcephaly or macrocephaly, īą Loss of previously acquired skills, īą Neurologic signs such as spasticity, dystonia, or altered reflexes. īą EEG in seizure disorder.
  • 35.
  • 36. LABORATORY EXAMINATION ī‚— In the absence of specific indications other than mere intellectual disability, metabolic testing is likely to be of little additional diagnostic value. ī‚— Testing in the presence of clinical suspicion may includeīƒ  īƒŧ plasma and urine amino and organic acid studies, īƒŧ acid/base balance, īƒŧ thyroid function tests, īƒŧ lysosomal enzyme analysis, īƒŧ plasma and urine carnitine analyses, īƒŧ plasma very long chain fatty acids, among others-phenylketonuria, hypothyroidism, and galactosemia. īƒŧ Cytogenetics for karyotyping - e.g. looking for genetic syndromes e.g. Down's syndrome, Fragile-X syndrome, Klinefelter's syndrome.
  • 37. Clinical Findings and Laboratory Abnormalities That Increase Suspicion for Underlying Metabolic Disorder īƒ˜ Abnormal sexual differentiation īƒ˜ Arachnodactyly īƒ˜ Cataracts, corneal clouding īƒ˜ Disturbance of consciousness, lethargy īƒ˜ Hypo or depigmented skin, hyperkeratotic plaques on soles and palms īƒ˜ Dysmorphic features Failure to thrive, feeding abnormalities, vomiting īƒ˜ Growth abnormality īƒ˜ Hair abnormalities (fragility) īƒ˜ Hypotonia, dystonia, ataxia Macrocephaly, microcephaly Neurodegenerative symptoms (regression or loss of skills) īƒ˜ Organomegaly , Retinal pigmentation īƒ˜ Seizures, specific electroencephalogram abnormalities īƒ˜ Self-injurious behavior – lesh nyhan syndrome īƒ˜ Skeletal malformations īƒ˜ Hyperammonemia Hyperuricemia Hypo/hyperglycemia ,Hypocholesterolemia
  • 38. PSYCHOLOGICAL ASSESSMENT The intellectual functioning and adaptive behaviour in a mentally retarded are assessed using a combination of test and schedules. The important are: a) Measurement of the overall level of general intellectual functioning.(IQ) b) Assessment of adaptive behaviour c) Developmental assessment(DQ)
  • 39. PSYCHOLOGICAL ASSESSMENT ī‚— Copying geometric figures, the Goodenough Draw-a-Person Test, the Kohs Block Test, and geometric puzzles all may be used as quick screening tests of visual-motor coordination. ī‚— The Gesell and Bayley scales and the Cattell Infant Intelligence Scale are most commonly used with infants. ī‚— For children, the Stanford-Binet Intelligence Scale and the third edition of the Wechsler Intelligence Scale for Children (WISC-III) are those most widely used . ī‚— The tests often found useful in detecting brain damage are the Bender Gestalt Test and the Benton Visual Retention Test . ī‚— In addition, a psychological evaluation should assess perceptual, motor, linguistic, and cognitive abilities.
  • 40. IQ Tests For Diagnosing Intellectual Disability īą Wechsler Preschool and Primary Scale of Intelligence-Revised (Wechsler, 1989) īą Wechsler Intelligence Test for Children-III (Wechsler, 1991) īą Wechsler Adult Intelligence Scale-Revised (Wechsler, 1981) īą Stanford-Binet Intelligence Scale: Fourth Edition (Thorndike, Hagen, and Sattler, 1986) In INDIA – A) Verbal test- Binet Kamat test Binet Kulshresta test Malin's intelligence test for Indian children. B) Nonverbal and performances tests : Developmental screening test Raven's progressive matrices Seguin form board test Gassel's drawing test Malin's intelligence scale for Indian Children-performance scale Alexander pass-along test Draw-A-Man test Koh's block design test
  • 41. Common Measures of Adaptive Behavior īą Vineland Adaptive Behavior Scales (Sparrow, Balla, and Cicchetti, 1984) Age range : Birth to 18 yrs Domains Tested: Communication: Receptive, Expressive, Written . Daily Living Skills: Personal, Domestic, Community Socialization: Interpersonal Relations, Play and Leisure, Coping Skill. īą AAMR Adaptive Behavior Scales (Lambert, Nihira, and Leland, 1993) Age range : 3 yrs to 18 yrs Domains Tested: Independent Functioning, Physical development, Economic Activity, School . Motor Skills: Fine, Gross. īą Scales of Independent Behavior- Revised (Bruininks, Woodcock, Weatherman, and Hill 1996) īąBinet Simon Scale Hindi Adaptation (BSSHA–K) (Age group 2ÂŊ to 18 years.)
  • 42. DEVELOPMENTAL ASSESSMENT ī‚— This is based on combination of clinical experience and standardized methods of measuring intelligence, language, motor performance, and social skill. ī‚— expressed in terms of developmental quotients(DQ's) ī‚— Although IQ is the best general index of intellectual development, it is not reliable in very young or among people who have severe to profound degree intellectual disability. ī‚— Commonly used instruments for Developmental assessment are in India :- īƒ˜ Bayley infant scales īƒ˜ Gassell's developmental schedules īƒ˜ NIMH development assessment schedules.
  • 43. DIFFERENTIAL DIAGNOSIS īƒ˜ Early infantile autism īƒ˜ Attention deficit hyperactivity īƒ˜ Child with hearing impairment īƒ˜ Cultural deprivation and lack of stimulation īƒ˜ Specific learning disabilities īƒ˜ Childhood psychosis īƒ˜ Child with visual handicap īƒ˜ Children under the age of 18 years who meet the diagnostic criteria for dementia and who have an IQ below 70 are given the diagnoses of DEMENTIA AND INTELLECTUAL DISABILITY. īƒ˜ Those whose IQs drop below 70 after the age of 18 years and who have new onsets of cognitive disorders are not given the diagnosis of intellectual disability but only the diagnosis of DEMENTIA.
  • 44. TREATMENT ī‚— Biological, psychological, social, and developmental dimensions should all be considered when designing a treatment plan for an individual with intellectual disability. ī‚— Treatment plan includes attention to īƒŧ Psychoeducational, īƒŧ Psychotherapeutic, and īƒŧ Psychopharmacologic interventions.
  • 45. Parents Counselling : Following points are noteworthy in this contest (Madhavan,1990). ī‚— 1. The child's actual condition should be explained in simple words to the parents. ī‚— 2. Enough time must be taken while counseling. ī‚— 3.Misleading, giving false information or building false hopes in parents must be avoided. ī‚— 4. Information regarding professional help for treating associated conditions like seizures, hyperactivity, psychosis etc. must be made available to the parents. ī‚— 5. Attitude such over protection ie., doing everything for the child and shielding them from any challenging situation, should be corrected as it hinders the development of whatever capacities the child may have.
  • 46. PARENTS COUNSELLING : ī‚— 6. The attitude of rejection, that is ignoring the child thinking that he is good for nothing should be changed so that the child can be helped to learn by systematic training. ī‚— 7. The parents should be made aware of what they may expect of their child ī‚— 8. Some patents suffer from guilt feeling assuming that they are responsible for their child's condition. It should be explained that the condition is due to causes over which parents have no direct control. ī‚— 9. The counselor should explain the effectiveness and role of the parents and other family members in training a mentally retarded. ī‚— 10. Some parents believe that training a mentally retarded child needs specialized skills and they may not be able to train their child. Parents should be explained that training a mentally retarded child dose not need complex skills and repeated training in simple steps, they can be taught. ī‚— 11. Parents should be helped to learn the skills in training through demonstrations and observations.
  • 47. Individualized Educational Programming ī‚— IEP is very much essential in training the retarded children because no two retarded children can be taught the same programme. Since each child differs from the other with regard to his/ her needs, strength and weakness of the programme developed should be tailored to suit needs of each child. ī‚— components of the IEP are : īƒ˜ Current level of functioning of the child in specific skills. īƒ˜ Annual goals īƒ˜ Short term objectives īƒ˜ Methods of training īƒ˜ Materials required for training īƒ˜ Manpower īƒ˜ Duration īƒ˜ Terminal behaviour īƒ˜ Evaluation for further programme planning
  • 48. BEHAVIOURAL TREATMENTS ī‚— The backbone of any treatment programme is educational with formal instruction in the skills which includes: ī‚— Independence and self-help skills- īƒ˜ These include the basic skills of everyday life, such as feeding, dressing, or managing stairs or, at a higher level, the use of public transport, and how to care for clothes, shop, and budget. īƒ˜ Acquiring these skills gives a sense of achievement as well as of increased independence. ī‚— Communication skills- īƒ˜ Frustration of living in an uncomprehending world frequently contributes to disturbance as the person falls back on various forms of attention-seeking or violent behaviour to get their message across. īƒ˜ It is helped by an easier and more effective means of communication.
  • 49. BEHAVIOURAL TREATMENTS īƒ˜ This may range from simple gestures (such as pulling at the trousers to indicate the need for toileting), through a system of pointing to symbols or pictures, to complex signing which can convey abstract concepts such as emotional states. īƒ˜ Language may be verbal or non-verbal, with a sign language such as Makaton (a simplified form of standard sign language) being taught simultaneously in both modalities. īƒ˜ These usually reinforce each other so that a course in signing may improve speech.
  • 50. BEHAVIOURAL TREATMENTS īƒ˜ Behavioral therapies are demonstrably effective in managing many maladaptive behaviors, particularly aggression and self-injury, in persons with intellectual disability. īƒ˜ Teaching appropriate habits and skills. These can include basic skills such as dressing, continence, communication, and establishing a normal pattern of sleep; or, at a more sophisticated level, training in social skills, copingskills, dating skills, and assertiveness. īƒ˜ The unlearning of other maladaptive forms of behaviour. īƒ˜ Positive reinforcement for desired behaviors and benign punishment (e.g., loss of privileges) for objectionable behaviors have been helpful.
  • 51. Task Analysis ī‚— To train a person in any skill, there are certain common basic principles to be followed. Initially the skill must be broken down in to small sequential steps. This is called 'Task Analysis'. ī‚— For example, a simple task 'Wearing slippers' - the major sub tasks (a) identifying one's own slippers, (b) identifying right and left slipper (c) inserting the correct foot in one slipper and (d) inserting the other foot in its slipper. Thus one has to perform all of these subtasks to complete the act of wearing slipper based on the task analysis the child should be trained.
  • 52. COGNITIVE THERAPY īąCognitive therapy is usually used with people in the mild or borderline range of retardation. īąDispelling false beliefs and relaxation exercises with self- instruction, has also been recommended for mentally retarded persons who can follow the instructions. īąProblem-solving skills are formally taught.
  • 53. Vocational Training and Job Placement : ī‚— Like any other system of education and training, the ultimate goal in training a mentally retarded person is to make him independent and earn his livelihood. Each mentally retarded person must be assessed for his readiness for work and opportunity for employment after training should be considered before training in a job- A)Self employment : Envelope making, agarbathi and candle making, running small pan shop , Dairy farms, poultry and agriculture are also good examples in this regard. B)Sheltered workshop : Here, mentally retarded are trained in specific tasks matched for their ability and they work under supervision. This requires a workshop with qualified staff to train them. The products are made here on contract basis, where raw material are received and finished products returned. C)Open employment: The retarded person can be employed on certain routine repetitive jobs like any other normal person and get paid in a similar manner. This includes office boys, lift operators, restaurant table cleaners, gardeners etc.
  • 54. PHARMACOTHERAPY ī‚— Antidepressants- Cardiac anomalies are common in some intellectual disability syndromes, and the anticholinergic side effects of some medications may be particularly significant in persons with Down syndrome; Due to lowering seizure threshold, clomipramine is generally not a first-line treatment for compulsive behaviour. ī‚— Anticonvulsants- carbamazepine (Tegretol) and valproic acid (Depakine), have been used clinically for aggressive behavior in children and adolescents. Persons with PWS treated with the anticonvulsant topiramate, whose skin-picking behavior improved. ī‚— Mood stabilizer -lithium has shown to be effective in reducing outbursts of aggression, particularly where there is irritability and explosiveness.
  • 55. PHARMACOTHERAPY ī‚— Antipsychotics- greater risk of developing tardive dyskinesia than the general population. Considerable evidence of effectiveness of risperidone in treating severe disruptive behavior in children and adolescents with cognitive disability. Trials have found that at daily doses ranging from .02 to .06 mg/kg, children and adolescents with various disruptive behavior disorders, the majority of whom also had attention deficit hyperactivity disorder, improved significantly. ī‚— Psychostimulants- Despite reports of paradoxical responses to stimulant medications in persons with intellectual disability, with higher-than-expected rates of emergent motor tics and emotional lability, a growing body of literature supports the use of stimulant drugs for the treatment of ADHD in the context of intellectual disability.
  • 56. PHARMACOTHERAPY ī‚— Opioid Antagonists- Naltrexone is the opioid antagonist most widely used for SIB, but the literature is mixed. Typical daily doses range from .5 to 2.0 mg/kg in children and up to 200 mg in adults. ī‚— Nootropics- drugs that positively affect cognition. Lobaugh and colleagues recently reported the first controlled trial of piracetam in children with down syndrome- Piracetam was associated with a number of side effects, including aggression, agitation, sexual arousal, poor sleep, and diminished appetite. There were no consistent benefits of piracetam over placebo identifiable in this study.
  • 57. Legislation ī‚— In 1995. the ‘person with disability act’ came ino India. This act envisages mandatory support for prevention, early detection, education, employement and other facilities for the welfare of person with diabilities in general and mental retardation in particular. ī‚— in 1999 the ‘National Trust Act’ came in force. This Act proposes to involve the parents of mentally challenged persons and voluntary organizations in setting up and running a variety of services and facilities with government funding. ī‚— ī‚— The IDEA’s (Individuals with Disabilities Education Act)disability terms and definitions guide how States in their own turn define disability and who is eligible for a free appropriate public education under special education law.