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DR. SUBRATA NASKAR
INTELLECTUAL DISABILITY
PART - I
DEFINITION
• THE AMERICAN ASSOCIATION OF INTELLECTUAL &
DEVELOPMENTAL DISABILITIES (AAIDD) HAS DEFINED
INTELLECTUAL DISABILITY AS A DISABILITY CHARACTERIZED BY
SIGNIFICANT LIMITATIONS IN BOTH INTELLECTUAL
FUNCTIONING AND IN ADAPTIVE BEHAVIOUR, WHICH COVERS
MANY EVERYDAY SOCIAL AND PRACTICAL SKILLS WHICH
ORIGINATES BEFORE THE AGE OF 18.
• THE DIAGNOSTIC AND STATISTICAL MANUAL OF MENTAL
DISORDERS, FIFTH EDITION (DSM-V) 7 DEFINES INTELLECTUAL
DISABILITY AS FOLLOWS:
• “INTELLECTUAL DISABILITY (INTELLECTUAL DEVELOPMENTAL
DISORDER) IS A DISORDER WITH ONSET DURING THE DEVELOPMENTAL
PERIOD THAT INCLUDES BOTH INTELLECTUAL AND ADAPTIVE
FUNCTIONING DEFICITS IN CONCEPTUAL, SOCIAL, AND PRACTICAL
DOMAINS.”
2
HISTORY
• THE EARLIEST REFERENCE TO
INTELLECTUAL DISABILITY
DATES TO THE EGYPTIAN
PAPYRUS OF THEBES IN 1552
B.C. (HARRIS 2006)
• GREEK AND ROMAN
PHILOSOPHERS, WHO VALUED
REASONING ABILITIES,
DISPARAGED PEOPLE WITH
INTELLECTUAL DISABILITIES AS
BARELY HUMAN.
THE EBERS PAPYRUS (1550 BC) FROM ANCIENT EGYPT
3
• THE OLDEST PHYSIOLOGICAL VIEW OF INTELLECTUAL DISABILITY IS IN
THE WRITINGS OF HIPPOCRATES IN THE LATE FIFTH CENTURY B.C.
WHO BELIEVED THAT IT WAS CAUSED BY AN IMBALANCE IN THE FOUR
HUMORS IN THE BRAIN.
4
• UNTIL THE ENLIGHTENMENT IN EUROPE, CARE AND ASYLUM WAS PROVIDED BY
FAMILIES AND THE CHURCH (IN MONASTERIES AND OTHER RELIGIOUS
COMMUNITIES), FOCUSING ON THE PROVISION OF BASIC PHYSICAL NEEDS SUCH
AS FOOD, SHELTER AND CLOTHING. NEGATIVE STEREOTYPES WERE PROMINENT IN
SOCIAL ATTITUDES OF THE TIME.
• IN THE 13TH CENTURY, ENGLAND DECLARED PEOPLE WITH INTELLECTUAL
DISABILITIES TO BE INCAPABLE OF MAKING DECISIONS OR MANAGING THEIR
AFFAIRS. GUARDIANSHIPS WERE CREATED TO TAKE OVER THEIR FINANCIAL
AFFAIRS. 5
• IN THE 17TH CENTURY, THOMAS WILLIS PROVIDED THE FIRST DESCRIPTION OF
INTELLECTUAL DISABILITIES AS A DISEASE. HE BELIEVED THAT IT WAS CAUSED BY
STRUCTURAL PROBLEMS IN THE BRAIN. ACCORDING TO WILLIS, THE ANATOMICAL
PROBLEMS COULD BE EITHER AN INBORN CONDITION OR ACQUIRED LATER IN LIFE.
• IN THE 18TH AND 19TH CENTURIES, HOUSING AND CARE MOVED AWAY FROM
FAMILIES AND TOWARDS AN ASYLUM MODEL. CHILDREN WITH INTELLECTUAL
DISABILITIES AND BEHAVIOURAL ABNORMALITIES WERE REMOVED FROM THEIR
FAMILIES AND PLACED IN SPECIALIZED INSTITUTIONS WHICH PROVIDED VERY MINIMAL
BASIC EDUCATION AND SURVIVED ON THE SERVICES PROVIDED THE RESIDENTS.
6
• JEAN-MARC ITARD IS CREDITED WITH CREATING THE FIRST SYSTEMATIC
INTERVENTION PROGRAM FOR A PERSON WITH INTELLECTUAL DISABILITY IN LATE
18TH CENTURY FRANCE, AND THE FIRST RESIDENTIAL FACILITY WAS FOUNDED IN
THE MID-19TH CENTURY IN SWITZERLAND.
• IN THE LATE 19TH CENTURY, IN RESPONSE TO CHARLES DARWIN'S ON THE ORIGIN
OF SPECIES, FRANCIS GALTON PROPOSED SELECTIVE BREEDING OF HUMANS TO
REDUCE INTELLECTUAL DISABILITIES.
• PSYCHOLOGICAL TESTS TO ASSESS INTELLIGENCE WERE DEVELOPED IN THE 20TH
CENTURY, WHICH INCREASED CASE IDENTIFICATION.
7
• EARLY IN THE 20TH CENTURY THE EUGENICS MOVEMENT BECAME POPULAR
THROUGHOUT THE WORLD WHICH LED TO FORCED STERILIZATION AND
PROHIBITION OF MARRIAGE IN MOST OF THE DEVELOPED WORLD AND WAS
LATER USED BY ADOLF HITLER AS A RATIONALE FOR THE MASS MURDER OF
INTELLECTUALLY CHALLENGED INDIVIDUALS DURING THE HOLOCAUST.
8
‘FEEBLEMINDEDNESS’
‘IDIOCY’
‘MENTAL SUB NORMALITY’
9
• THE TERM 'MENTAL RETARDATION' WAS INTRODUCED BY THE AMERICAN
ASSOCIATION ON MENTAL RETARDATION IN 1961, AND SOON AFTERWARDS WAS
ADOPTED BY THE AMERICAN PSYCHIATRIC ASSOCIATION (APA) IN ITS DIAGNOSTIC
AND STATISTICAL MANUAL FOR MENTAL DISORDERS (DSM).
• MENTAL RETARDATION REPLACED OLDER TERMS SUCH AS ‘FEEBLEMINDEDNESS’,
‘IDIOCY’, AND ‘MENTAL SUB NORMALITY’ THAT HAD BECOME PEJORATIVE.
10
THE JOURNEY FROM MENTAL RETARDATION TO
INTELLECTUAL DISABILITY
11
• ROSA MARCELLINO, AN 8-YEAR-OLD GIRL WITH DOWN
SYNDROME FROM MARYLAND, WAS TAUNTED
FREQUENTLY AND PEJORATIVELY CALLED 'RETARD' IN A
DEMEANING MANNER.
• WITH SUPPORT FROM HER STATE REPRESENTATIVE AND
U.S SENATOR BARBARA MIKULSKI, LEGISLATION WAS
INITIATED LEADING TO THE CHANGE IN THE LAW.
• THE TERM 'MENTAL RETARDATION' HAS BEEN
ELIMINATED IN THE UNITED STATES, A FEDERAL STATUTE
(PUBLIC LAW 111-256, ROSA'S LAW) REPLACES THE
TERM 'MENTAL RETARDATION' WITH 'INTELLECTUAL
DISABILITY' AND REQUIRES THAT PERSON FIRST
LANGUAGE BE USED WHEN REFERRING TO THOSE
AFFECTED IN ALL FEDERAL LAWS.
12
• THE NEW TERM PROPOSED IN DSM-5 IS INTELLECTUAL
DISABILITY (ID).
• THE NEW DSM-5 CATEGORY IS SYNONYMOUS WITH
THE PROPOSED ICD-11 DIAGNOSIS OF IDDS, IN THAT IT
REFERS TO A HEALTH CONDITION OR DISORDER.
• THE ICD-11 WORKING GROUP HAS PROPOSED
REPLACING MENTAL RETARDATION WITH
INTELLECTUAL DEVELOPMENTAL DISORDERS (IDDS), A
TERM IT DEFINES AS 'A GROUP OF DEVELOPMENTAL
CONDITIONS CHARACTERIZED BY SIGNIFICANT
IMPAIRMENT OF COGNITIVE FUNCTIONS, WHICH ARE
ASSOCIATED WITH LIMITATIONS OF LEARNING,
ADAPTIVE BEHAVIOUR AND SKILLS'.
13
WHO ARE THE
INTELLECTUALLY DISABLED ?
14
DSM V CRITERIA
• THE FOLLOWING THREE CRITERIA MUST BE MET:
• DEFICITS IN INTELLECTUAL FUNCTIONS, SUCH AS
• REASONING
• PROBLEM SOLVING
• PLANNING
• ABSTRACT THINKING
• JUDGMENT
• ACADEMIC LEARNING
• LEARNING FROM EXPERIENCE
• CONFIRMED BY BOTH CLINICAL ASSESSMENT AND INDIVIDUALIZED,
STANDARDIZED INTELLIGENCE TESTING. 15
DSM V CRITERIA
• DEFICITS IN ADAPTIVE FUNCTIONING THAT RESULT IN FAILURE TO MEET
DEVELOPMENTAL AND SOCIOCULTURAL STANDARDS FOR PERSONAL
INDEPENDENCE AND SOCIAL RESPONSIBILITY.
• WITHOUT ONGOING SUPPORT, THE ADAPTIVE DEFICITS LIMIT
FUNCTIONING IN ONE OR MORE ACTIVITIES OF DAILY LIFE, SUCH AS
COMMUNICATION, SOCIAL PARTICIPATION, AND INDEPENDENT LIVING,
ACROSS MULTIPLE ENVIRONMENTS, SUCH AS HOME, SCHOOL, WORK, AND
COMMUNITY.
16
DSM V CRITERIA
• ONSET OF INTELLECTUAL AND ADAPTIVE DEFICITS DURING THE
DEVELOPMENTAL PERIOD
17
GENERAL INTELLECTUAL FUNCTIONING
• IT IS DEFIENED AS THE RESULTS OBTAINED BY THE ADMINISTRATION
OF A STANDARDIZED GENERAL INTELLIGENCE TESTS DEVELOPED FOR
THE PURPOSE AND ADAPTED TO THE CONDITIONS OF THE REGION/
COUNTRY.
18
SUBNORMAL INTELLECTUAL FUNCTIONING
• SUB-AVERAGE INTELLECTUAL FUNCTIONING IS DEFINED AS “AN IQ OF
ABOUT 70 OR BELOW (APPROXIMATELY 2 STANDARD DEVIATIONS
BELOW THE MEAN).
• STANDARD ERROR OF MEASUREMENT IS 3 OR 4 POINTS OVER OR
UNDER THE SCORE OBTAINED ON A PARTICULAR TEST.
• ZONE OF UNCERTAINTY – I.Q = 66-74
19
ADAPTIVE BEHAVIOUR
• IT IS DEFIENED AS THE DEGREE WITH WHICH THE INDIVIDUAL MEETS
THE STANDARDS OF PERSONAL INDEPENDENCE & SOCIAL
RESPONSIBILITY EXPECTED OF HIS AGE & CULTURAL GROUP. THE
EXPECTATION OF ADAPTIVE BEHAVIOUR VARY WITH THE
CHRONOLOGICAL AGE.
20
IMPAIRMENT IN ADAPTIVE
BEHAVIOUR
• DURING INFANCY & EARLY
CHILDHOOD:
1. SENSORY & MOTOR SKILL
DEVELOPMENT.
2. COMMUNICATION SKILLS
[ SPEECH & LANGUAGE)
3. SELF HELP SKILLS.
4. SOCIALIZATION.
21
DURING CHILDHOOD & ADOLESCENCE
1. APPLICATION OF BASIC ACADEMIC SKILLS TO
DAILY LIFE ACTIVITIES.
2. APPLICATION OF APPROPRIATE REASONING &
JUDGEMENT IN THE MASTERY OF THE
ENVIORNMENT.
3. SOCIAL SKILLS
22
DURING LATE ADOLESCENCE & ADULT LIFE
VOCATIONAL TRAINING & SOCIAL RESPONSIBILITIES.
23
24
NORMAL
DEVELOPMENTAL MILESTONES
1ST TWO YEARS
25
26
27
28
29
CLASSIFICATION
30
DSM 5
31
32
ICD 10
33
PROPOSED ICD 11 CRITERIA FOR
INTELLECTUAL DEVELOPMENTAL DISORDER
THERE ARE THREE REQUIREMENTS FOR A
DIAGNOSIS OF DISORDERS OF ID
• THE PRESENCE OF SIGNIFICANT LIMITATIONS IN INTELLECTUAL
FUNCTIONING
• THE PRESENCE OF SIGNIFICANT LIMITATIONS IN ADAPTIVE
BEHAVIOUR
• ONSET DURING THE DEVELOPMENTAL PERIOD.
34
Marc J. Tassé, Ruth Luckasson, and Margaret Nygren (2013) AAIDD Proposed Recommendations for ICD–11 and the Condition
Previously Known as Mental Retardation. Intellectual and Developmental Disabilities: April 2013, Vol. 51, No. 2, pp. 127-131.
doi: http://dx.doi.org/10.1352/1934-9556-51.2.127
ICD 11 PROPOSED CLASSIFICATION OF IDD
• DISORDER OF INTELLECTUAL DISABILITY, MARKED
• DISORDER OF INTELLECTUAL DISABILITY, EXTENSIVE
• DISORDER OF INTELLECTUAL DISABILITY, PERVASIVE
• DISORDER OF INTELLECTUAL DISABILITY, OTHER
35
Marc J. Tassé, Ruth Luckasson, and Margaret Nygren (2013) AAIDD Proposed Recommendations for ICD–11 and the
Condition Previously Known as Mental Retardation. Intellectual and Developmental Disabilities: April 2013, Vol. 51, No.
2, pp. 127-131. doi: http://dx.doi.org/10.1352/1934-9556-51.2.127
RATIONALE
• FIRST, THEY RECOMMENDED COMBINING THE PROPOSED “SEVERE” AND
“PROFOUND” CLASSIFICATIONS INTO ONE CATEGORY.
• THERE IS NO SCIENTIFIC RATIONALE OR PSYCHOMETRIC VALIDITY TO
MAINTAINING A 4-LEVEL CLASSIFICATION SYSTEM.
• COLLAPSING ALL INDIVIDUALS WITH IQ SCORES BELOW 40 INTO ONE
CATEGORY IS MORE SCIENTIFICALLY AND PSYCHOMETRICALLY SUPPORTED
THAN ATTEMPTING TO IMPOSE A CLASSIFICATION CUT-OFF THAT IS FIVE
STANDARD DEVIATIONS BELOW THE POPULATION MEAN (I.E., IQ SCORE  = 
25).
• EXISTING STANDARDIZED TESTS OF INTELLIGENCE CANNOT RELIABLY OR
VALIDLY DISTINGUISH AMONG INDIVIDUALS WITH IQ SCORES BELOW 40.
HENCE, WE PROPOSE A CLASSIFICATION OF DISORDERS OF ID WITH THREE
LEVELS OF SEVERITY.
36
EPIDEMIOLOGY
• INTELLECTUAL DISABILITY AFFECTS APPROXIMATELY 1-4% OF THE
POPULATION IN DEVELOPED COUNTRIES.
• IN GENERAL IT IS CONSIDERED THAT 2% OF THE INDIAN POPULATION
CONSTITUTES PERSONS WITH THIS DISABILITY.
• MENTAL RETARDATION IS ABOUT 1.5 TIMES MORE COMMON AMONG
MEN THAN AMONG WOMEN.
37
• ACCORDING TO CENSUS 2011 DATA, THERE ARE CURRENTLY 1,505,624
‘MENTALLY RETARDED’ PEOPLE IN INDIA.
• IN RURAL AREAS, THE INCIDENCE OF MENTAL RETARDATION IS 3.1% AND IN
URBAN, IT IS 0.9%.
• THE NATIONAL INSTITUTE FOR THE MENTALLY HANDICAPPED (NIMH),
SECUNDERABAD MENTIONS THAT 2% OF THE GENERAL POPULATION IS
INTELLECTUALLY DISABLED.
• THREE QUARTERS OF THEM ARE WITH MILD RETARDATION AND ONE-
FOURTH ARE WITH SEVERE RETARDATION (PANDA, 1999).
38
THE ASSAM SCENARIO
• AS PER THE DATA OBTAINED FROM CENSUS 2011, THE TOTAL
POPULATION OF ASSAM IS 31,205,576 AMONG WHICH TOTAL
4,80,065 PEOPLE ARE SUFFERING FROM SOME SORT OF DISABILITY.
39
TOTAL
POPULATION
TOTAL
DISABLED
TOTAL
INTELLECTUALLY
DISABLED
(previously called
mental retardation)
ASSAM 31,205,576 4,80,065 26,374
CACHAR 1,736,617 27,457 1,586
KARIMGANJ 1,228,686 18,832 1,138
HAILAKANDI 659,296 9,723 617
TOTAL OF
3 DISTRICTS
3,624,599 56012 3,341
CENSUS OF INDIA 2011 40
• THIS DATA SHOWS THAT THERE CURRENTLY 26,374 PEOPLE WITH
INTELLECTUAL DISABILITY IN ASSAM, WHICH IS 0.08% OF THE TOTAL
POPULATION.
• IN THE DISTRICTS OF CACHAR, HAILAKANDI AND KARIMGANJ, THE TOTAL
NUMBER OF INTELLECTUALLY DISABLED PEOPLE ARE 3,341 WHICH IS 0.09%
OF THE TOTAL POPULATION.
• THIS FIGURE IS MUCH LESS THAN THE REPORTED PREVALENCE OF
INTELLECTUAL DISABILITY REPORTED AT NATIONAL LEVEL, WHICH IS 2% OF
THE TOTAL POPULATION ACCORDING TO NIMH REPORTS.
• THIS INDICATES TWO POSSIBILITIES, EITHER THERE IS A LESS INCIDENCE AND
PREVALENCE OF INTELLECTUAL DISABILITY IN THIS PART OF THE COUNTRY
OR A SIGNIFICANT PORTION OF INTELLECTUALLY DISABLED PEOPLE REMAIN
UNDERDIAGNOSED OR NON-REPORTED. 41
42
ETIOLOGY
PRENATAL CAUSES
• MATERNAL INFECTIONS OF RUBELLA, TOXOPLASMA ETC.
• PRENATAL USE OF TOXIC SUBSTANCES LIKE ETHANOL, CARBON
MONOXIDE EXPOSURE.
• RH INCOMPATIBILITY.
• GENETIC DISORDERS:
• CHROMOSOMAL ABERRATIONS: E.G. DOWN’S SYNDROME, PRADER WILLI
SYNDROME, CRI-DU-CHAT SYNDROME
• DISORDERS WITH AUTOSOMAL DOMINANCE: E.G. TUBEROUS SCLEROSIS
• DISORDERS WITH AUTOSOMAL RECESSIVE INHERITANCE: E.G.
PHENYLKETONURIA
• X-LINKED DISORDERS: E.G. FRAGILE X
• PRENATAL INJURY
43
PERINATAL CAUSES
• INFECTIONS E.G. HERPES SIMPLEX VIRUS 2
• MECHANICAL INJURY DURING BIRTH
• PERINATAL HYPOXIA
• OTHER PERINATAL CAUSES LIKE RETINOPATHY
OF PREMATURITY, NEONATAL
HYPERBILIRUBINEMIA
44
POSTNATAL CAUSES
• INFECTIONS: BACTERIAL AND VIRAL
INFECTIONS OF THE BRAIN DURING
CHILDHOOD LIKE MENINGITIS OR
ENCEPHALITIS.
• TOXIC SUBSTANCES: E.G. EXPOSURE TO
LEAD.
• OTHER CAUSES: HYPOTHYROIDISM,
DIETARY DEFICIENCIES, BRAIN
TUMOURS.
45
DISCUSSION ON SOME PROMINENT
ETIOLOGY OF INTELLECTUAL DISABILITY
GENETIC DISORDERS
46
DOWN SYNDROME
• THE SINGLE MOST IMPORTANT PRENATAL GENETIC DISORDER
RESPONSIBLE FOR INTELLECTUAL DISABILITY IS DOWN SYNDROME.
THERE ARE THREE WIDELY ACCEPTED CAUSES THAT EXPLAINS THE
GENETIC BASIS OF DOWN SYNDROME:
• COMPLETE TRISOMY 21
• MOSAIC TRISOMY 21
• TRANSLOCATION TRISOMY 21
47
IN GENETICS, A MOSAIC OR MOSAICISM
DENOTES THE PRESENCE OF TWO OR MORE
POPULATIONS OF CELLS WITH DIFFERENT
GENOTYPES IN ONE INDIVIDUAL WHO HAS
DEVELOPED FROM A SINGLE FERTILIZED EGG.
48
CLINICAL FEATURES
• UPWARD-SLANTED PALPEBRAL
FISSURES
• A LOW NASAL BRIDGE WITH
EPICANTHAL FOLD
• SMALL MOUTH AND EARS
• SINGLE PALMER CREASE (SIMIAN
CREASE)
• A FLAT NASAL BRIDGE
• SHORT AND WIDE PALMS
• CHARACTERISTIC DERMATOGLYPHIC
PATTERN.
49
FRAGILE X SYNDROME
• FRAGILE X SYNDROME IS THE SECOND MOST
COMMON SINGLE CAUSE OF ID.
• THE SYNDROME RESULTS FROM A MUTATION ON
THE X CHROMOSOME AT WHAT IS KNOWN AS THE
FRAGILE SITE (XQ27.3).
• THE TYPICAL PHENOTYPE INCLUDES A LARGE, LONG
HEAD AND EARS, SHORT STATURE, HYPER
EXTENSIBLE JOINTS, AND POST PUBERTAL
MACROORCHIDISM.
• THE MENTAL RETARDATION RANGES FROM MILD TO
SEVERE.
50
FRAGILE X SYNDROME IS BELIEVED TO OCCUR IN ABOUT 1 OF
EVERY 1,000 MALES AND 1 OF EVERY 2,000 FEMALES. 51
PRADER-WILLI SYNDROME
• PRADER-WILLI SYNDROME IS POSTULATED TO RESULT FROM A SMALL
DELETION INVOLVING CHROMOSOME 15, USUALLY OCCURRING
SPORADICALLY.
• ITS PREVALENCE IS LESS THAN 1 OF 10,000.
• PERSONS WITH THE SYNDROME EXHIBIT COMPULSIVE EATING
BEHAVIOR AND OFTEN OBESITY, MENTAL RETARDATION,
HYPOGONADISM, SMALL STATURE, HYPOTONIA, AND SMALL HANDS
AND FEET.
• CHILDREN WITH THE SYNDROME OFTEN HAVE OPPOSITIONAL AND
DEFIANT BEHAVIOR.
52
PRADER-WILLI SYNDROME
53
CRI-DU-CHAT SYNDROME
• CHILDREN WITH CAT'S CRY
SYNDROME LACK PART OF
CHROMOSOME 5.
54
• SEVERE RETARDATION
• MICROCEPHALY
• LOW-SET EARS
• OBLIQUE PALPEBRAL FISSURES
• HYPERTELORISM
• MICROGNATHIA.
• THE CHARACTERISTIC CAT-LIKE CRY CAUSED BY
LARYNGEAL ABNORMALITIES THAT GAVE THE
SYNDROME ITS NAME GRADUALLY CHANGES AND
DISAPPEARS WITH INCREASING AGE.
• DIFFICULTY SWALLOWING AND SUCKING
• LOW BIRTH WEIGHT AND POOR GROWTH
• HYPERACTIVE, AGGRESSIVE, AND REPETITIVE
MOVEMENTS
55
KLINEFELTER’S SYNDROME & TURNER’S
SYNDROME
• TURNER SYNDROME OCCURS IN 1/2500 LIVE BIRTHS. IT IS CAUSED BY
A PARTIAL OR COMPLETE ABSENCE OF ONE OF THE X
CHROMOSOMES IN A FEMALE.
• IN THE CASE OF KLINEFELTER SYNDROME, THE MALE HAS 2 X
CHROMOSOMES AND 1 Y CHROMOSOME
56
KLINEFELTER’S SYNDROME & TURNER’S
SYNDROME
57
KLINEFELTER’S
SYNDROME &
TURNER’S
SYNDROME
58
• CAUSED BY THE ABSENCE OF MATERIAL
ON THE SEVENTH PAIR OF
CHROMOSOME.
• THEIR UNUSUAL SENSITIVITY TO SOUND
MAKES THEM COMPETENT IN MUSIC AND
LANGUAGE DESPITE OF THEIR LOW IQ
LEVEL.
WILLIAM’S SYNDROME
WILLIAM’S SYNDROME
PHYSICAL FEATURES
• SHORT STATURE
• ELFINLIKE FACIES
• BROAD FOREHEAD
• DEPRESSED NASAL BRIDGE
• STELLATE PATTERN OF THE IRIS
• WIDELY SPACED TEETH
• FULL LIPS
• RENAL AND CARDIOVASCULAR
ABNORMALITIES
• THYROID ABNORMALITIES
• HYPERCALCEMIA
BEHAVIOURAL FEATURES
• ANXIETY
• HYPERACTIVITY
• OUTGOING
• SOCIABLE
• VERBAL SKILLS > VISUAL SPATIAL SKILLS
60
CORNELIA DE LANGE SYNDROME
• MULTIPLE GENES HAVE BEEN ASSOCIATED WITH THE CONDITION
• SLOW GROWTH BEFORE AND AFTER BIRTH
• INTELLECTUAL DISABILITY THAT IS USUALLY SEVERE TO PROFOUND
• SKELETAL ABNORMALITIES INVOLVING THE ARMS AND HANDS
• DISTINCTIVE FACIAL FEATURES
• ARCHED EYEBROWS THAT OFTEN GROW TOGETHER IN THE MIDDLE (SYNOPHRYS)
• LONG EYELASHES
• LOW-SET EARS
• SMALL, WIDELY SPACED TEETH
• SMALL, UPTURNED NOSE.
• MANIFEST SELF-INJURIOUS BEHAVIOR
61
62
RETT'S DISORDER
• RETT'S DISORDER IS HYPOTHESIZED
TO BE AN X-LINKED DOMINANT
SYNDROME THAT IS DEGENERATIVE
AND AFFECTS MAINLY FEMALES.
63
• DECELERATION OF HEAD GROWTH BETWEEN AGES 5 AND 48 MONTHS
• LOSS OF PREVIOUSLY ACQUIRED PURPOSEFUL HAND SKILLS BETWEEN 5
AND 30 MONTHS WITH THE SUBSEQUENT DEVELOPMENT OF STEREOTYPED
HAND MOVEMENTS (E.G., HAND-WRINGING OR HAND WASHING)
• LOSS OF SOCIAL ENGAGEMENT EARLY IN THE COURSE ( ALTHOUGH OFTEN
SOCIAL INTERACTION DEVELOPS LATER)
• APPEARANCE OF POORLY COORDINATED GAIT OR TRUNK MOVEMENTS
• SEVERELY IMPAIRED EXPRESSIVE AND RECEPTIVE LANGUAGE DEVELOPMENT
WITH SEVERE PSYCHOMOTOR RETARDATION 64
NEUROFIBROMATOSIS
• ALSO CALLED VON RECKLINGHAUSEN'S DISEASE
• NEUROFIBROMATOSIS IS THE MOST COMMON OF THE NEUROCUTANEOUS
SYNDROMES CAUSED BY A SINGLE DOMINANT GENE, WHICH MAY BE
INHERITED OR BE A NEW MUTATION.
• THE DISORDER OCCURS IN ABOUT 1 OF 5,000 BIRTHS.
• CHARACTERIZED BY
• CAFE AU LAIT SPOTS ON THE SKIN AND BY NEUROFIBROMAS
• OPTIC GLIOMAS
• ACOUSTIC NEUROMAS, CAUSED BY ABNORMAL CELL MIGRATION.
• MILD MENTAL RETARDATION OCCURS IN UP TO ONE THIRD OF THOSE
WITH THE DISEASE. 65
NEUROFIBROMATOSIS
66
TUBEROUS SCLEROSIS
• TUBEROUS SCLEROSIS IS THE SECOND MOST COMMON OF THE
NEUROCUTANEOUS SYNDROMES; A PROGRESSIVE MENTAL
RETARDATION OCCURS IN UP TO TWO THIRDS OF ALL AFFECTED
PERSONS.
• IT OCCURS IN ABOUT 1 OF 15,000 PERSONS AND IS INHERITED BY
AUTOSOMAL DOMINANT TRANSMISSION.
67
VOGT’S TRIAD
• MENTAL RETARDATION
• EPILEPSY
• ADENOMA SEBACEUM
68
CLINICAL FEATURES
69
ADENOMA SEBACEUM
SHAGREEN PATCH
ASH LEAF PATCH
ADRENOLEUKODYSTROPHY
• THE MOST COMMON OF SEVERAL DISORDERS OF SUDANOPHILIC
CEREBRAL SCLEROSIS, ADRENOLEUKODYSTROPHY IS CHARACTERIZED BY
• DIFFUSE DEMYELINATION OF THE CEREBRAL WHITE MATTER RESULTING IN VISUAL
AND INTELLECTUAL IMPAIRMENT
• SEIZURES
• SPASTICITY
• PROGRESSION TO DEATH.
• THE DISORDER IS TRANSMITTED BY A SEX-LINKED GENE LOCATED ON THE
DISTAL END OF THE LONG ARM OF THE X CHROMOSOME.
70
71
INBORN ERRORS OF METABOLISM
72
PHENYLKETONURIA
PKU IS TRANSMITTED AS A SIMPLE RECESSIVE AUTOSOMAL
MENDELIAN TRAIT AND OCCURS IN ABOUT 1 OF EVERY 10,000
TO 15,000 LIVE BIRTHS.
73
CLINICAL
FEATURES
• SEVERELY RETARDED, BUT SOME ARE REPORTED TO
HAVE BORDERLINE OR NORMAL INTELLIGENCE.
• ECZEMA
• CONVULSIONS OCCUR IN ABOUT A THIRD OF ALL
PATIENTS.
• THEY FREQUENTLY HAVE TEMPER TANTRUMS
• OFTEN DISPLAY BIZARRE MOVEMENTS OF THEIR
BODIES AND UPPER EXTREMITIES, INCLUDING
TWISTING HAND MANNERISMS
• A MUSTY ODOR IN THE CHILD'S BREATH, SKIN OR
URINE, CAUSED BY TOO MUCH PHENYLALANINE IN THE
BODY
• FAIR SKIN AND BLUE EYES, BECAUSE PHENYLALANINE
CANNOT TRANSFORM INTO MELANIN — THE PIGMENT
RESPONSIBLE FOR HAIR AND SKIN TONE
• ABNORMALLY SMALL HEAD (MICROCEPHALY)
74
MAPLE SYRUP URINE DISEASE
• AN INBORN ERROR OF METABOLISM CAUSED BY DEFECTIVE OXIDATIVE
DECARBOXYLATION OF KETO ACIDS OF LEUCINE, ISOLEUCINE, AND
VALINE.
• THESE BRANCHED-CHAIN AMINO ACIDS ARE PRESENT IN THE BLOOD AND
URINE IN HIGH CONCENTRATIONS.
• MANIFESTATIONS OF DISEASE INCLUDE FEEDING DIFFICULTIES, PHYSICAL
AND MENTAL RETARDATION, AND A URINE ODOR SIMILAR TO THAT OF
MAPLE SYRUP; NEONATAL DEATH IS COMMON.
75
LESCH-NYHAN SYNDROME
• LESCH-NYHAN SYNDROME IS A RARE DISORDER CAUSED BY A
DEFICIENCY OF AN ENZYME INVOLVED IN PURINE METABOLISM.
• THE DISORDER IS X-LINKED; PATIENTS HAVE MENTAL RETARDATION,
MICROCEPHALY, SEIZURES, CHOREOATHETOSIS, AND SPASTICITY.
76
LESCH-NYHAN SYNDROME
77
• GALACTOSEMIA - INABILITY OF THE BODY TO USE SIMPLE SUGAR GALACTOSE
• HUNTER SYNDROME – DEFECTIVE BREAKDOWN OF CHEMICAL
MUCOPOLYSACCHARIDE.
• TAY-SACHS DISEASE – ABSENCE OF HEX-A ENZYME.
78
MATERNAL INFECTIONS
79
RUBELLA (GERMAN MEASLES)
• THE MAJOR CAUSE OF MENTAL RETARDATION CAUSED BY MATERNAL
INFECTION.
• THE CHILDREN OF AFFECTED MOTHERS MAY SHOW SEVERAL
ABNORMALITIES, INCLUDING CONGENITAL HEART DISEASE, MENTAL
RETARDATION, CATARACTS, DEAFNESS, MICROCEPHALY, AND
MICROPHTHALMIA.
• THE INCIDENCE RISES TO ALMOST 50 PERCENT WHEN THE INFECTION
OCCURS IN THE FIRST MONTH OF PREGNANCY.
80
CYTOMEGALIC INCLUSION DISEASE
• SOME CHILDREN ARE STILLBORN, AND OTHERS HAVE JAUNDICE,
MICROCEPHALY, HEPATOSPLENOMEGALY, AND RADIOGRAPHIC
FINDINGS OF INTRACEREBRAL CALCIFICATION.
• THE DIAGNOSIS IS CONFIRMED BY POSITIVE FINDINGS OF THE VIRUS
IN THROAT AND URINE CULTURES.
81
SYPHILIS
• SYPHILIS IN PREGNANT WOMEN WAS ONCE THE MAIN CAUSE OF
VARIOUS NEUROPATHOLOGICAL CHANGES IN THEIR OFFSPRING,
INCLUDING MENTAL RETARDATION.
• TODAY, THE INCIDENCE OF SYPHILITIC COMPLICATIONS OF
PREGNANCY FLUCTUATES WITH THE INCIDENCE OF SYPHILIS IN THE
GENERAL POPULATION.
82
• TOXOPLASMOSIS
• IT CAUSES MILD OR SEVERE MENTAL RETARDATION AND, IN SEVERE CASES,
HYDROCEPHALUS, SEIZURES, MICROCEPHALY, AND CHORIORETINITIS.
• HERPES SIMPLEX
• THE HERPES SIMPLEX VIRUS CAN BE TRANSMITTED TRANSPLACENTALLY, IT IS ONE OF
THE MOST COMMON MODE OF INFECTION DURING BIRTH.
• MICROCEPHALY, MENTAL RETARDATION, INTRACRANIAL CALCIFICATION, AND
OCULAR ABNORMALITIES MAY RESULT.
83
ACQUIRED IMMUNE DEFICIENCY SYNDROME (AIDS)
• MANY FETUSES OF MOTHERS WITH AIDS NEVER COME TO TERM BECAUSE
OF STILLBIRTH OR SPONTANEOUS ABORTION.
• OF INFANTS BORN INFECTED WITH THE HUMAN IMMUNODEFICIENCY VIRUS
(HIV), UP TO HALF HAVE PROGRESSIVE ENCEPHALOPATHY, MENTAL
RETARDATION, AND SEIZURES WITHIN THE FIRST YEAR OF LIFE.
84
PRENATAL USE OF TOXIC SUBSTANCES
85
FETAL
ALCOHOL
SYNDROME
• A TYPICAL PHENOTYPIC PICTURE OF FACIAL DYSMORPHISM THAT INCLUDES
HYPERTELORISM, MICROCEPHALY, SHORT PALPEBRAL FISSURES, INNER
EPICANTHAL FOLDS, AND A SHORT, TURNED-UP NOSE.
• THE ENTIRE SYNDROME OCCURS IN UP TO 15 PERCENT OF BABIES BORN TO
WOMEN WHO REGULARLY INGEST LARGE AMOUNTS OF ALCOHOL.
86
PRENATAL DRUG EXPOSURE
• COCAINE
• HEROIN
• DIAZEPAM
• PHENOBARBITAL
• CHLORPROMAZINE
87
• THE INTELLECTUAL DISABILITY USUALLY
RANGES FROM SEVERE TO PROFOUND.
• THERE IS NO SPECIFIC TREATMENT AND
LIFE EXPECTANCY IS LOW.
MICROCEPHALUS
HOLOPROSENCEPHALY (HPE, ONCE KNOWN AS
ARHINENCEPHALY) IS A CEPHALIC DISORDER IN
WHICH THE PROSENCEPHALON (THE
FOREBRAIN OF THE EMBRYO) FAILS TO
DEVELOP INTO TWO HEMISPHERES.
• RESULTS FROM AN ACCUMULATION
OF CEREBROSPINAL FLUID INSIDE
OR OUTSIDE THE BRAIN.
• THE DEGREE OF INTELLECTUAL
DISABILITY DEPENDS ON HOW
EARLY THE CONDITION IS
DIAGNOSED AND TREATED.
HYDROCEPHALUS
91
COMPLICATIONS OF PREGNANCY
COMPLICATIONS OF PREGNANCY
• TOXEMIA OF PREGNANCY AND
UNCONTROLLED MATERNAL DIABETES
RESULT IN MENTAL RETARDATION.
• MATERNAL MALNUTRITION DURING
PREGNANCY OFTEN RESULTS IN
PREMATURITY AND OTHER OBSTETRICAL
COMPLICATIONS.
• VAGINAL HEMORRHAGE, PLACENTA
PREVIA, PREMATURE SEPARATION OF THE
PLACENTA, AND PROLAPSE OF THE CORD
CAN DAMAGE THE FETAL BRAIN BY
CAUSING ANOXIA
93
PERINATAL CAUSES
• PREMATURE INFANTS AND INFANTS WITH LOW
BIRTHWEIGHT ARE AT HIGH RISK FOR
NEUROLOGICAL AND INTELLECTUAL IMPAIRMENTS
THAT APPEAR DURING THEIR SCHOOL YEARS.
• INFANTS WHO SUSTAIN INTRACRANIAL
HEMORRHAGES OR SHOW EVIDENCE OF CEREBRAL
ISCHEMIA ARE ESPECIALLY VULNERABLE TO
COGNITIVE ABNORMALITIES.
• AMONG CHILDREN WITH VERY LOW BIRTHWEIGHT,
20 PERCENT HAS SIGNIFICANT DISABILITIES,
INCLUDING CEREBRAL PALSY, MENTAL
RETARDATION, AUTISM, AND LOW INTELLIGENCE
WITH SEVERE LEARNING PROBLEMS.
94
95
POSTNATAL CAUSES
• NUTRITIONAL PROBLEMS
• ADVERSE LIVING
CONDITIONS
• INADEQUATE HEALTH CARE
• LACK OF EARLY COGNITIVE
STIMULATION
ENVIRONMENTAL AND PSYCHOSOCIAL PROBLEMS
• CHILD ABUSE AND NEGLECT
• TRAUMATIC BRAIN INJURY
• VIRAL INFECTIONS LIKE
MENINGITIS OR
ENCEPHALITIS
• HEAVY METAL POISONING
ESP. LEAD POISONING
ENVIRONMENTAL AND PSYCHOSOCIAL PROBLEMS
98
COMMON COMORBID
PSYCHIATRIC DISORDERS
COMMON COMORBID PSYCHIATRIC DISORDERS
• INTELLECTUAL DISABILITY IS A SIGNIFICANT RISK FACTOR FOR
PSYCHOPATHOLOGY IN GENERAL.
• PSYCHIATRIC DISORDERS ARE THREE TO FOUR TIMES HIGHER IN
PEOPLE WITH AN ID DIAGNOSIS THAN IN THE GENERAL POPULATION.
• THE PREVALENCE OF PSYCHIATRIC DISORDERS IN INDIVIDUALS WITH
ID IS ESTIMATED TO BE BETWEEN 30% AND 60%
99
• i. INTERNATIONAL STUDIES
• ERIC EMERSON ET AL (2007) IN A STUDY CONDUCTED IN BRITAIN FOUND THE
PREVALENCE OF PSYCHIATRIC DISORDERS WAS 36% AMONG CHILDREN WITH
INTELLECTUAL DISABILITY AND 8% AMONG CHILDREN WITHOUT
• II. INDIAN STUDIES
• A STUDY WAS CONDUCTED BY C.R.J. KHESS ET AL (1998), AT THE CHILD PSYCHIATRIC
UNIT (CPU.) OF THE CENTRAL INSTITUTE OF PSYCHIATRY (C.I.P.) OVER A ONE YEAR
PERIOD THE STUDY SAMPLE CONSTITUTED OF 60 PATIENTS, FOUND PSYCHIATRIC
COMORBIDITY WAS PRESENT IN 34 (56.67%) PATIENTS.
100
• WHEN ASSESSING FOR PSYCHIATRIC DISORDERS IN PEOPLE WITH AN ID,
ASSESSMENT PROCEDURES MUST BE MODIFIED TO TAKE INTO ACCOUNT
ASSOCIATED DISORDERS, SUCH AS COMMUNICATION DISORDERS, AUTISM
SPECTRUM DISORDER, AND MOTOR, SENSORY, OR OTHER CO-OCCURRING
CONDITIONS.
• KNOWLEDGEABLE INFORMANTS ARE ESSENTIAL DURING ASSESSMENT TO
IDENTIFY CHANGES IN IRRITABILITY, MOOD REGULATION, INCREASED
AGGRESSION, EATING PROBLEMS, SLEEP PROBLEMS, AND CHANGES IN
ADAPTIVE BEHAVIOUR AT WORK, AT HOME, AND IN THE COMMUNITY
SETTINGS.
101
THE MOST COMMON PSYCHIATRIC COMORBIDITIES
ASSOCIATED WITH THE INTELLECTUALLY DISABLED ARE :
• ATTENTION-DEFICIT/HYPERACTIVITY DISORDER
• AUTISM SPECTRUM DISORDER
• ANXIETY DISORDERS
• BIPOLAR AND RELATED, DISORDER
• DEPRESSIVE DISORDER
• SCHIZOPHRENIA SPECTRUM AND OTHER PSYCHOTIC DISORDER
• FEEDING AND EATING DISORDER
• DISRUPTIVE, IMPULSE CONTROL AND CONDUCT DISORDERS
• SOMATIC SYMPTOM & RELATED DISORDER
102
ATTENTION-DEFICIT/HYPERACTIVE
DISORDERS
• THE PREVALENCE OF ADHD IN CHILDREN WITH ID IS ESTIMATED TO
BE BETWEEN 8.7% AND 16%, COMPARED WITH 5% IN THE GENERAL
POPULATION.
• FOR PERSONS WITH ID, THE DIAGNOSIS OF ADHD DEPENDS UPON
THE PRESENCE OF SYMPTOMS EXCESSIVE FOR AN INDIVIDUAL’S
MENTAL AGE OR REMARKABLE COMPARED WITH PEERS WHO HAVE
SIMILAR LEVELS OF RETARDATION.
103
• A STUDY CONDUCTED IN THE CHILD PSYCHIATRY UNIT OF A TERTIARY
PSYCHIATRIC HOSPITAL BY KHESS R.J. ET AL.78 EVALUATED THAT
(14%) OF THE STUDY SUBJECTS HAD COMORBID HYPERKINETIC
DISORDER.
• A STUDY CONDUCTED IN THE DEPARTMENT OF PSYCHIATRY, SMCH,
INCLUDING 100 ID PATIENTS, SHOWED THE PREVALENCE OF ADHD TO
BE 11%
104
AUTISM SPECTRUM DISORDER
• IT HAS BEEN FOUND THAT INCREASED SEVERITY OF ID IS POSITIVELY CORRELATED WITH
THE INCIDENCE OF AUTISM.
• IN FACT, IT CAN SOMETIMES BE DIFFICULT TO DISTINGUISH AUTISM FROM SEVERE ID.
• HISTORICALLY, THE PREVALENCE OF INTELLECTUAL DISABILITY IN AUTISM IS ESTIMATED
AT 70%, BUT RECENT STUDIES, SUGGEST THAT THE PREVALENCE OF INTELLECTUAL
DISABILITY IN AUTISM-SPECTRUM CONDITIONS MAY BE CONSIDERABLY LOWER.
• IT HAS BEEN SUGGESTED THAT THE ASSOCIATION BETWEEN AUTISM AND
INTELLECTUAL DISABILITY MAY BE INFLATED BECAUSE OF CLINICAL ASCERTAINMENT
BIAS.
105
• Cooper and colleagues FOUND THE PREVALENCE OF AUTISM SPECTRUM
DISORDER TO BE 4.4% IN PERSONS WITH ID.
• A STUDY CONDUCTED IN THE CHILD PSYCHIATRY UNIT OF A TERTIARY
PSYCHIATRIC HOSPITAL BY Khess r.J. Et al. EVALUATED THAT (11%) OF THE
STUDY SUBJECTS HAD AUTISM.
• IN OUR STUDY WE FOUND THE PREVALENCE TO BE 3%
106
ANXIETY DISORDERS
• FOR PEOPLE WITH INTELLECTUAL DISABILITY, THE PREVALENCE OF ANXIETY DISORDERS HAS BEEN
SHOWN TO BE MUCH HIGHER.
• THE VARIABILITY OF PREVALENCE IS FROM 1% TO 25%. THIS MAY BE DUE TO REDUCED
COGNITIVE ABILITIES AND INCREASED VULNERABILITY TO ENVIRONMENTAL DEMANDS.
• IN OUR STUDY WE FOUND THE PREVALENCE TO BE 3%
• AN INDIVIDUAL WITH INTELLECTUAL DISABILITY MAY NOT BE ABLE TO IDENTIFY SUBJECTIVE
ANXIETY AS AN UNDERLYING CAUSE OF DISTRESS AND THE RESULTING AGITATION AND
AGGRESSION MAY BE SUGGESTIVE FOR A DIAGNOSIS OF IMPULSE CONTROL DISORDER RATHER
THAN REFLECTING THE UNDERLYING ANXIETY.
107
• FREQUENT SYMPTOMS OF ANXIETY IN INTELLECTUALLY DISABLED
PERSONS INCLUDE AGGRESSION, AGITATION, SELF-INJURY, AND
INSOMNIA. THESE SYMPTOMS ARE OFTEN DIFFICULT TO DISTINGUISH
FROM IMPULSE CONTROL DISORDERS.
• ANXIETY DISORDERS OR POSTTRAUMATIC STRESS DISORDER MAY
PRESENT IN RESPONSE TO FRUSTRATION OR THREATENING SITUATIONS;
FEARFULNESS AND AVOIDANT BEHAVIOURS.
• PANIC ATTACKS MAY RESEMBLE RAGE OUTBURSTS, AND RESULT IN
AGGRESSION OR SELF-INJURY.
108
BIPOLAR AND RELATED DISORDER
• AFFECTIVE DISORDERS IN ALL FORMS DO OCCUR IN PERSONS WITH
MENTAL RETARDATION.
• THE PRESENCE AND DEGREE OF MENTAL RETARDATION MODIFY
MANIFESTATIONS OF THESE DISORDERS.
• DIAGNOSIS IS DIFFICULT BECAUSE OF THE ABSENCE OF CLASSICAL
MANIFESTATIONS AND FREQUENT OCCURRENCE OF INTER-EPISODE
BEHAVIOUR DISTURBANCES
109
• A STUDY CONDUCTED IN THE CHILD PSYCHIATRY UNIT OF A TERTIARY
PSYCHIATRIC HOSPITAL BY KHESS R.J. ET AL. EVALUATED THAT (8%) OF
THE STUDY SUBJECTS HAD COMORBID MOOD DISORDER.
• IN OUR STUDY WE FOUND THE PREVALENCE TO BE 6%
110
DEPRESSIVE DISORDER
• OWING TO INTELLECTUAL AND COMMUNICATION LIMITATIONS, IT IS
DIFFICULT TO USE THE PSYCHIATRIC DIAGNOSTIC INTERVIEW AND
APPLY STANDARD DIAGNOSTIC CRITERIA TO THIS POPULATION. THUS,
DEPRESSION MAY BE MISDIAGNOSED OR UNDERDIAGNOSED.
• THE POINT PREVALENCE OF DEPRESSION AMONG ADULTS WITH
INTELLECTUAL DISABILITY IS ESTIMATED TO BE 4% (COOPER & BAILEY,
2001).
• IN OUR STUDY WE FOUND THE PREVALENCE TO BE 4%
111
SCHIZOPHRENIA SPECTRUM AND OTHER
PSYCHOTIC DISORDER
• INDIVIDUALS WITH INTELLECTUAL DISABILITY EXHIBIT THE SAME TYPES OF
PSYCHOTIC DISORDERS AS ADULTS OF NORMAL INTELLIGENCE, ALTHOUGH
AN ACCURATE DIAGNOSIS IS OFTEN DIFFICULT TO MAKE.
• DIAGNOSTIC OVERSHADOWING, FOR EXAMPLE, IN WHICH ABNORMAL
BEHAVIOURS ARE ASSUMED TO BE THE RESULT OF ID RATHER THAN
POTENTIAL COMORBID PSYCHOPATHOLOGY, MAY OBSCURE
IDENTIFICATION OF PSYCHIATRIC CONDITIONS.
• A STUDY BY PENROSE REPORTED THAT 3.8% OF 1280 INDIVIDUALS WITH
INTELLECTUAL DISABILITY HAD SCHIZOPHRENIA AND 1.9% HAD AFFECTIVE
PSYCHOSES.
112
• C.R.J. KHESS ET AL (1998) IN HIS STUDY REPORTED THAT 11.76% OF
THE PATIENTS WERE FOUND TO HAVE PSYCHOSIS, BUT NONE
FULFILLED THE CRITERIA FOR SCHIZOPHRENIA
• IN OUR STUDY WE FOUND THE PREVALENCE TO BE 14%
113
FEEDING AND EATING DISORDER
• PICA AND RUMINATION SEEMS TO BE THE MOST COMMON EATING
DISORDER IN THE INTELLECTUALLY DISABLED.
• THE INGESTION OF NON-NUTRITIVE SUBSTANCES, REGURGITATION
AND RUMINATION OCCURS WITH GREATER FREQUENCY AS THE
SEVERITY OF COGNITIVE DISABILITY INCREASES.
• ANOREXIA NERVOSA AND BULIMIA NERVOSA ARE RELATIVELY
UNCOMMON IN THE INTELLECTUALLY DISABLED
114
• DESPITE DIAGNOSTIC AND METHODOLOGICAL DIFFICULTIES,
PSYCHOPATHOLOGY AND EATING DISORDER RESEARCH STUDIES
SUGGEST THAT 3-42% OF INSTITUTIONALIZED ADULTS WITH ID AND
1-19% OF ADULTS WITH ID IN THE COMMUNITY HAVE DIAGNOSABLE
EATING DISORDERS (Gravestock S)
• IN OUR STUDY WE FOUND THE PREVALENCE TO BE 6%
115
DISRUPTIVE, IMPULSE CONTROL AND
CONDUCT DISORDERS
• IMPULSE CONTROL DIFFICULTIES REPRESENT A MAJOR FEATURE OF A
NUMBER OF BEHAVIOURAL AND MENTAL DISORDERS IN CHILDREN,
YOUTH, AND ADULTS WITH INTELLECTUAL DISABILITIES (ID).
• Khess R.J. et al.IN THEIR STUDY EVALUATED THAT (2%) OF THE STUDY
SUBJECTS HAD COMORBID CONDUCT DISORDER.
• IN OUR STUDY WE FOUND THE PREVALENCE RATE TO BE 3%
116
SOMATIC SYMPTOM & RELATED DISORDER
• SOMATIC SYMPTOM & RELATED DISORDER IS LARGELY COMMON IN
PATIENTS WITH ID.
• MOSTLY, IT IS SEEN IN PATIENTS WITH MILD AND MODERATE ID.
• HOWEVER, THE PREVALENCE RATE IS NOT SO WELL RESEARCHED.
• IN OUR STUDY, WE FOUND THE PREVALENCE TO BE 5%
117
118
INTELLECTUAL DISABILITY
&
SUBSTANCE ABUSE
• SLAYTER (2010) REVIEWED HEALTHCARE BILLING CLAIMS AND
CONCLUDED THAT 2.6% OF ALL PEOPLE WITH ID HAD A DIAGNOSABLE
SUBSTANCE ABUSE DISORDER.
• OTHER ESTIMATES USING DIFFERENT METHODOLOGIES VARY WIDELY,
RANGING AS HIGH AS 26% (STRAIN, BUCCINO, BROONER, SCHMIDT, &
BIGELOW, 1993).
• WHEN A PERSON WITH ID ALSO HAS A MENTAL ILLNESS OR A DUAL
DIAGNOSIS, THE ESTIMATES OF CO-OCCURRING SUBSTANCE ABUSE
RANGE FROM 7% TO 20% (SINCLAIR, 2004).
• THE RESEARCH IN THIS AREA IS SPARSE, BUT THE NUMBERS ARE
ALARMING.
119
• PEOPLE WITH ID WHO ARE SUBSTANCE ABUSERS SHARE SOME UNIQUE
TRAITS.
• AS A GROUP, THEY TEND TO BEGIN DRINKING ALCOHOL A COUPLE YEARS
LATER THAN THEIR PEERS WITHOUT ID (SLAYTER).
• THEY ARE LESS LIKELY TO SEEK HELP FOR THEIR PROBLEM AND EVEN WHEN
THEY DO, THE RESOURCES THAT ARE HELPFUL FOR THE GENERAL PUBLIC
FALL SHORT OF MEETING THEIR NEEDS.
• ADDITIONALLY, THIS GROUP IS AT GREATER RISK OF COMPLICATIONS FROM
DRINKING BECAUSE THEY TEND TO BE PRESCRIBED MEDICATIONS FOR
OTHER CONDITIONS, SUCH AS SEIZURES, METABOLIC DISORDERS, AND CO-
OCCURRING MENTAL ILLNESS THAT MIGHT NEGATIVELY INTERACT WITH
ALCOHOL AND DRUGS.
120
121
SEIZURE DISORDER IN INTELLECTUAL DISABILITY
SEIZURE DISORDER IN INTELLECTUAL DISABILITY
• WHEN CONSIDERED ACROSS THE SPECTRUM OF INTELLECTUAL DISABILITY,
THE FREQUENCY AND SEVERITY OF EPILEPSY VARIES INVERSELY WITH THE
LEVEL OF MENTAL RETARDATION (ROBERTS, 1986).
• AMONG INDIVIDUALS WITH MILD RETARDATION, THE DISTRIBUTION OF
VARIOUS SUBTYPES OF SEIZURES APPROXIMATES THE GENERAL
POPULATION.
• AMONG INDIVIDUALS WITH SEVERE/PROFOUND RETARDATION,
INCREASED RATES OF UNDERLYING NEUROLOGICAL ABNORMALITIES ARE
MANIFESTED BY A HIGHER FREQUENCY OF MIXED SEIZURE DISORDERS,
AND AN ONSET OF SEIZURES IN INFANCY OR EARLY CHILDHOOD (RATEY &
DYNEK, 1996; ROBERTS, 1986).
122
• COMMUNITY-BASED STUDIES OF EPILEPSY IN ADULTS WITH ID SHOW
A PREVALENCE OF 16–26%. BOWLEY, C. ET AL (2000).
• IN OUR STUDY WE FOUND THE PREVALENCE RATE TO BE 22%
123
TO BE CONTINUED…..
IN PART - II
• MANAGEMENT OF INTELLECTUAL DISABILITY
• SERVICES AND SUPPORT
• LEGAL ASPECTS
124
THANK YOU

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INTELLECTUAL DISABILITY PART- I

  • 1. DR. SUBRATA NASKAR INTELLECTUAL DISABILITY PART - I
  • 2. DEFINITION • THE AMERICAN ASSOCIATION OF INTELLECTUAL & DEVELOPMENTAL DISABILITIES (AAIDD) HAS DEFINED INTELLECTUAL DISABILITY AS A DISABILITY CHARACTERIZED BY SIGNIFICANT LIMITATIONS IN BOTH INTELLECTUAL FUNCTIONING AND IN ADAPTIVE BEHAVIOUR, WHICH COVERS MANY EVERYDAY SOCIAL AND PRACTICAL SKILLS WHICH ORIGINATES BEFORE THE AGE OF 18. • THE DIAGNOSTIC AND STATISTICAL MANUAL OF MENTAL DISORDERS, FIFTH EDITION (DSM-V) 7 DEFINES INTELLECTUAL DISABILITY AS FOLLOWS: • “INTELLECTUAL DISABILITY (INTELLECTUAL DEVELOPMENTAL DISORDER) IS A DISORDER WITH ONSET DURING THE DEVELOPMENTAL PERIOD THAT INCLUDES BOTH INTELLECTUAL AND ADAPTIVE FUNCTIONING DEFICITS IN CONCEPTUAL, SOCIAL, AND PRACTICAL DOMAINS.” 2
  • 3. HISTORY • THE EARLIEST REFERENCE TO INTELLECTUAL DISABILITY DATES TO THE EGYPTIAN PAPYRUS OF THEBES IN 1552 B.C. (HARRIS 2006) • GREEK AND ROMAN PHILOSOPHERS, WHO VALUED REASONING ABILITIES, DISPARAGED PEOPLE WITH INTELLECTUAL DISABILITIES AS BARELY HUMAN. THE EBERS PAPYRUS (1550 BC) FROM ANCIENT EGYPT 3
  • 4. • THE OLDEST PHYSIOLOGICAL VIEW OF INTELLECTUAL DISABILITY IS IN THE WRITINGS OF HIPPOCRATES IN THE LATE FIFTH CENTURY B.C. WHO BELIEVED THAT IT WAS CAUSED BY AN IMBALANCE IN THE FOUR HUMORS IN THE BRAIN. 4
  • 5. • UNTIL THE ENLIGHTENMENT IN EUROPE, CARE AND ASYLUM WAS PROVIDED BY FAMILIES AND THE CHURCH (IN MONASTERIES AND OTHER RELIGIOUS COMMUNITIES), FOCUSING ON THE PROVISION OF BASIC PHYSICAL NEEDS SUCH AS FOOD, SHELTER AND CLOTHING. NEGATIVE STEREOTYPES WERE PROMINENT IN SOCIAL ATTITUDES OF THE TIME. • IN THE 13TH CENTURY, ENGLAND DECLARED PEOPLE WITH INTELLECTUAL DISABILITIES TO BE INCAPABLE OF MAKING DECISIONS OR MANAGING THEIR AFFAIRS. GUARDIANSHIPS WERE CREATED TO TAKE OVER THEIR FINANCIAL AFFAIRS. 5
  • 6. • IN THE 17TH CENTURY, THOMAS WILLIS PROVIDED THE FIRST DESCRIPTION OF INTELLECTUAL DISABILITIES AS A DISEASE. HE BELIEVED THAT IT WAS CAUSED BY STRUCTURAL PROBLEMS IN THE BRAIN. ACCORDING TO WILLIS, THE ANATOMICAL PROBLEMS COULD BE EITHER AN INBORN CONDITION OR ACQUIRED LATER IN LIFE. • IN THE 18TH AND 19TH CENTURIES, HOUSING AND CARE MOVED AWAY FROM FAMILIES AND TOWARDS AN ASYLUM MODEL. CHILDREN WITH INTELLECTUAL DISABILITIES AND BEHAVIOURAL ABNORMALITIES WERE REMOVED FROM THEIR FAMILIES AND PLACED IN SPECIALIZED INSTITUTIONS WHICH PROVIDED VERY MINIMAL BASIC EDUCATION AND SURVIVED ON THE SERVICES PROVIDED THE RESIDENTS. 6
  • 7. • JEAN-MARC ITARD IS CREDITED WITH CREATING THE FIRST SYSTEMATIC INTERVENTION PROGRAM FOR A PERSON WITH INTELLECTUAL DISABILITY IN LATE 18TH CENTURY FRANCE, AND THE FIRST RESIDENTIAL FACILITY WAS FOUNDED IN THE MID-19TH CENTURY IN SWITZERLAND. • IN THE LATE 19TH CENTURY, IN RESPONSE TO CHARLES DARWIN'S ON THE ORIGIN OF SPECIES, FRANCIS GALTON PROPOSED SELECTIVE BREEDING OF HUMANS TO REDUCE INTELLECTUAL DISABILITIES. • PSYCHOLOGICAL TESTS TO ASSESS INTELLIGENCE WERE DEVELOPED IN THE 20TH CENTURY, WHICH INCREASED CASE IDENTIFICATION. 7
  • 8. • EARLY IN THE 20TH CENTURY THE EUGENICS MOVEMENT BECAME POPULAR THROUGHOUT THE WORLD WHICH LED TO FORCED STERILIZATION AND PROHIBITION OF MARRIAGE IN MOST OF THE DEVELOPED WORLD AND WAS LATER USED BY ADOLF HITLER AS A RATIONALE FOR THE MASS MURDER OF INTELLECTUALLY CHALLENGED INDIVIDUALS DURING THE HOLOCAUST. 8
  • 10. • THE TERM 'MENTAL RETARDATION' WAS INTRODUCED BY THE AMERICAN ASSOCIATION ON MENTAL RETARDATION IN 1961, AND SOON AFTERWARDS WAS ADOPTED BY THE AMERICAN PSYCHIATRIC ASSOCIATION (APA) IN ITS DIAGNOSTIC AND STATISTICAL MANUAL FOR MENTAL DISORDERS (DSM). • MENTAL RETARDATION REPLACED OLDER TERMS SUCH AS ‘FEEBLEMINDEDNESS’, ‘IDIOCY’, AND ‘MENTAL SUB NORMALITY’ THAT HAD BECOME PEJORATIVE. 10
  • 11. THE JOURNEY FROM MENTAL RETARDATION TO INTELLECTUAL DISABILITY 11
  • 12. • ROSA MARCELLINO, AN 8-YEAR-OLD GIRL WITH DOWN SYNDROME FROM MARYLAND, WAS TAUNTED FREQUENTLY AND PEJORATIVELY CALLED 'RETARD' IN A DEMEANING MANNER. • WITH SUPPORT FROM HER STATE REPRESENTATIVE AND U.S SENATOR BARBARA MIKULSKI, LEGISLATION WAS INITIATED LEADING TO THE CHANGE IN THE LAW. • THE TERM 'MENTAL RETARDATION' HAS BEEN ELIMINATED IN THE UNITED STATES, A FEDERAL STATUTE (PUBLIC LAW 111-256, ROSA'S LAW) REPLACES THE TERM 'MENTAL RETARDATION' WITH 'INTELLECTUAL DISABILITY' AND REQUIRES THAT PERSON FIRST LANGUAGE BE USED WHEN REFERRING TO THOSE AFFECTED IN ALL FEDERAL LAWS. 12
  • 13. • THE NEW TERM PROPOSED IN DSM-5 IS INTELLECTUAL DISABILITY (ID). • THE NEW DSM-5 CATEGORY IS SYNONYMOUS WITH THE PROPOSED ICD-11 DIAGNOSIS OF IDDS, IN THAT IT REFERS TO A HEALTH CONDITION OR DISORDER. • THE ICD-11 WORKING GROUP HAS PROPOSED REPLACING MENTAL RETARDATION WITH INTELLECTUAL DEVELOPMENTAL DISORDERS (IDDS), A TERM IT DEFINES AS 'A GROUP OF DEVELOPMENTAL CONDITIONS CHARACTERIZED BY SIGNIFICANT IMPAIRMENT OF COGNITIVE FUNCTIONS, WHICH ARE ASSOCIATED WITH LIMITATIONS OF LEARNING, ADAPTIVE BEHAVIOUR AND SKILLS'. 13
  • 14. WHO ARE THE INTELLECTUALLY DISABLED ? 14
  • 15. DSM V CRITERIA • THE FOLLOWING THREE CRITERIA MUST BE MET: • DEFICITS IN INTELLECTUAL FUNCTIONS, SUCH AS • REASONING • PROBLEM SOLVING • PLANNING • ABSTRACT THINKING • JUDGMENT • ACADEMIC LEARNING • LEARNING FROM EXPERIENCE • CONFIRMED BY BOTH CLINICAL ASSESSMENT AND INDIVIDUALIZED, STANDARDIZED INTELLIGENCE TESTING. 15
  • 16. DSM V CRITERIA • DEFICITS IN ADAPTIVE FUNCTIONING THAT RESULT IN FAILURE TO MEET DEVELOPMENTAL AND SOCIOCULTURAL STANDARDS FOR PERSONAL INDEPENDENCE AND SOCIAL RESPONSIBILITY. • WITHOUT ONGOING SUPPORT, THE ADAPTIVE DEFICITS LIMIT FUNCTIONING IN ONE OR MORE ACTIVITIES OF DAILY LIFE, SUCH AS COMMUNICATION, SOCIAL PARTICIPATION, AND INDEPENDENT LIVING, ACROSS MULTIPLE ENVIRONMENTS, SUCH AS HOME, SCHOOL, WORK, AND COMMUNITY. 16
  • 17. DSM V CRITERIA • ONSET OF INTELLECTUAL AND ADAPTIVE DEFICITS DURING THE DEVELOPMENTAL PERIOD 17
  • 18. GENERAL INTELLECTUAL FUNCTIONING • IT IS DEFIENED AS THE RESULTS OBTAINED BY THE ADMINISTRATION OF A STANDARDIZED GENERAL INTELLIGENCE TESTS DEVELOPED FOR THE PURPOSE AND ADAPTED TO THE CONDITIONS OF THE REGION/ COUNTRY. 18
  • 19. SUBNORMAL INTELLECTUAL FUNCTIONING • SUB-AVERAGE INTELLECTUAL FUNCTIONING IS DEFINED AS “AN IQ OF ABOUT 70 OR BELOW (APPROXIMATELY 2 STANDARD DEVIATIONS BELOW THE MEAN). • STANDARD ERROR OF MEASUREMENT IS 3 OR 4 POINTS OVER OR UNDER THE SCORE OBTAINED ON A PARTICULAR TEST. • ZONE OF UNCERTAINTY – I.Q = 66-74 19
  • 20. ADAPTIVE BEHAVIOUR • IT IS DEFIENED AS THE DEGREE WITH WHICH THE INDIVIDUAL MEETS THE STANDARDS OF PERSONAL INDEPENDENCE & SOCIAL RESPONSIBILITY EXPECTED OF HIS AGE & CULTURAL GROUP. THE EXPECTATION OF ADAPTIVE BEHAVIOUR VARY WITH THE CHRONOLOGICAL AGE. 20
  • 21. IMPAIRMENT IN ADAPTIVE BEHAVIOUR • DURING INFANCY & EARLY CHILDHOOD: 1. SENSORY & MOTOR SKILL DEVELOPMENT. 2. COMMUNICATION SKILLS [ SPEECH & LANGUAGE) 3. SELF HELP SKILLS. 4. SOCIALIZATION. 21
  • 22. DURING CHILDHOOD & ADOLESCENCE 1. APPLICATION OF BASIC ACADEMIC SKILLS TO DAILY LIFE ACTIVITIES. 2. APPLICATION OF APPROPRIATE REASONING & JUDGEMENT IN THE MASTERY OF THE ENVIORNMENT. 3. SOCIAL SKILLS 22
  • 23. DURING LATE ADOLESCENCE & ADULT LIFE VOCATIONAL TRAINING & SOCIAL RESPONSIBILITIES. 23
  • 25. 25
  • 26. 26
  • 27. 27
  • 28. 28
  • 31. 31
  • 33. 33 PROPOSED ICD 11 CRITERIA FOR INTELLECTUAL DEVELOPMENTAL DISORDER
  • 34. THERE ARE THREE REQUIREMENTS FOR A DIAGNOSIS OF DISORDERS OF ID • THE PRESENCE OF SIGNIFICANT LIMITATIONS IN INTELLECTUAL FUNCTIONING • THE PRESENCE OF SIGNIFICANT LIMITATIONS IN ADAPTIVE BEHAVIOUR • ONSET DURING THE DEVELOPMENTAL PERIOD. 34 Marc J. Tassé, Ruth Luckasson, and Margaret Nygren (2013) AAIDD Proposed Recommendations for ICD–11 and the Condition Previously Known as Mental Retardation. Intellectual and Developmental Disabilities: April 2013, Vol. 51, No. 2, pp. 127-131. doi: http://dx.doi.org/10.1352/1934-9556-51.2.127
  • 35. ICD 11 PROPOSED CLASSIFICATION OF IDD • DISORDER OF INTELLECTUAL DISABILITY, MARKED • DISORDER OF INTELLECTUAL DISABILITY, EXTENSIVE • DISORDER OF INTELLECTUAL DISABILITY, PERVASIVE • DISORDER OF INTELLECTUAL DISABILITY, OTHER 35 Marc J. Tassé, Ruth Luckasson, and Margaret Nygren (2013) AAIDD Proposed Recommendations for ICD–11 and the Condition Previously Known as Mental Retardation. Intellectual and Developmental Disabilities: April 2013, Vol. 51, No. 2, pp. 127-131. doi: http://dx.doi.org/10.1352/1934-9556-51.2.127
  • 36. RATIONALE • FIRST, THEY RECOMMENDED COMBINING THE PROPOSED “SEVERE” AND “PROFOUND” CLASSIFICATIONS INTO ONE CATEGORY. • THERE IS NO SCIENTIFIC RATIONALE OR PSYCHOMETRIC VALIDITY TO MAINTAINING A 4-LEVEL CLASSIFICATION SYSTEM. • COLLAPSING ALL INDIVIDUALS WITH IQ SCORES BELOW 40 INTO ONE CATEGORY IS MORE SCIENTIFICALLY AND PSYCHOMETRICALLY SUPPORTED THAN ATTEMPTING TO IMPOSE A CLASSIFICATION CUT-OFF THAT IS FIVE STANDARD DEVIATIONS BELOW THE POPULATION MEAN (I.E., IQ SCORE  =  25). • EXISTING STANDARDIZED TESTS OF INTELLIGENCE CANNOT RELIABLY OR VALIDLY DISTINGUISH AMONG INDIVIDUALS WITH IQ SCORES BELOW 40. HENCE, WE PROPOSE A CLASSIFICATION OF DISORDERS OF ID WITH THREE LEVELS OF SEVERITY. 36
  • 37. EPIDEMIOLOGY • INTELLECTUAL DISABILITY AFFECTS APPROXIMATELY 1-4% OF THE POPULATION IN DEVELOPED COUNTRIES. • IN GENERAL IT IS CONSIDERED THAT 2% OF THE INDIAN POPULATION CONSTITUTES PERSONS WITH THIS DISABILITY. • MENTAL RETARDATION IS ABOUT 1.5 TIMES MORE COMMON AMONG MEN THAN AMONG WOMEN. 37
  • 38. • ACCORDING TO CENSUS 2011 DATA, THERE ARE CURRENTLY 1,505,624 ‘MENTALLY RETARDED’ PEOPLE IN INDIA. • IN RURAL AREAS, THE INCIDENCE OF MENTAL RETARDATION IS 3.1% AND IN URBAN, IT IS 0.9%. • THE NATIONAL INSTITUTE FOR THE MENTALLY HANDICAPPED (NIMH), SECUNDERABAD MENTIONS THAT 2% OF THE GENERAL POPULATION IS INTELLECTUALLY DISABLED. • THREE QUARTERS OF THEM ARE WITH MILD RETARDATION AND ONE- FOURTH ARE WITH SEVERE RETARDATION (PANDA, 1999). 38
  • 39. THE ASSAM SCENARIO • AS PER THE DATA OBTAINED FROM CENSUS 2011, THE TOTAL POPULATION OF ASSAM IS 31,205,576 AMONG WHICH TOTAL 4,80,065 PEOPLE ARE SUFFERING FROM SOME SORT OF DISABILITY. 39
  • 40. TOTAL POPULATION TOTAL DISABLED TOTAL INTELLECTUALLY DISABLED (previously called mental retardation) ASSAM 31,205,576 4,80,065 26,374 CACHAR 1,736,617 27,457 1,586 KARIMGANJ 1,228,686 18,832 1,138 HAILAKANDI 659,296 9,723 617 TOTAL OF 3 DISTRICTS 3,624,599 56012 3,341 CENSUS OF INDIA 2011 40
  • 41. • THIS DATA SHOWS THAT THERE CURRENTLY 26,374 PEOPLE WITH INTELLECTUAL DISABILITY IN ASSAM, WHICH IS 0.08% OF THE TOTAL POPULATION. • IN THE DISTRICTS OF CACHAR, HAILAKANDI AND KARIMGANJ, THE TOTAL NUMBER OF INTELLECTUALLY DISABLED PEOPLE ARE 3,341 WHICH IS 0.09% OF THE TOTAL POPULATION. • THIS FIGURE IS MUCH LESS THAN THE REPORTED PREVALENCE OF INTELLECTUAL DISABILITY REPORTED AT NATIONAL LEVEL, WHICH IS 2% OF THE TOTAL POPULATION ACCORDING TO NIMH REPORTS. • THIS INDICATES TWO POSSIBILITIES, EITHER THERE IS A LESS INCIDENCE AND PREVALENCE OF INTELLECTUAL DISABILITY IN THIS PART OF THE COUNTRY OR A SIGNIFICANT PORTION OF INTELLECTUALLY DISABLED PEOPLE REMAIN UNDERDIAGNOSED OR NON-REPORTED. 41
  • 43. PRENATAL CAUSES • MATERNAL INFECTIONS OF RUBELLA, TOXOPLASMA ETC. • PRENATAL USE OF TOXIC SUBSTANCES LIKE ETHANOL, CARBON MONOXIDE EXPOSURE. • RH INCOMPATIBILITY. • GENETIC DISORDERS: • CHROMOSOMAL ABERRATIONS: E.G. DOWN’S SYNDROME, PRADER WILLI SYNDROME, CRI-DU-CHAT SYNDROME • DISORDERS WITH AUTOSOMAL DOMINANCE: E.G. TUBEROUS SCLEROSIS • DISORDERS WITH AUTOSOMAL RECESSIVE INHERITANCE: E.G. PHENYLKETONURIA • X-LINKED DISORDERS: E.G. FRAGILE X • PRENATAL INJURY 43
  • 44. PERINATAL CAUSES • INFECTIONS E.G. HERPES SIMPLEX VIRUS 2 • MECHANICAL INJURY DURING BIRTH • PERINATAL HYPOXIA • OTHER PERINATAL CAUSES LIKE RETINOPATHY OF PREMATURITY, NEONATAL HYPERBILIRUBINEMIA 44
  • 45. POSTNATAL CAUSES • INFECTIONS: BACTERIAL AND VIRAL INFECTIONS OF THE BRAIN DURING CHILDHOOD LIKE MENINGITIS OR ENCEPHALITIS. • TOXIC SUBSTANCES: E.G. EXPOSURE TO LEAD. • OTHER CAUSES: HYPOTHYROIDISM, DIETARY DEFICIENCIES, BRAIN TUMOURS. 45
  • 46. DISCUSSION ON SOME PROMINENT ETIOLOGY OF INTELLECTUAL DISABILITY GENETIC DISORDERS 46
  • 47. DOWN SYNDROME • THE SINGLE MOST IMPORTANT PRENATAL GENETIC DISORDER RESPONSIBLE FOR INTELLECTUAL DISABILITY IS DOWN SYNDROME. THERE ARE THREE WIDELY ACCEPTED CAUSES THAT EXPLAINS THE GENETIC BASIS OF DOWN SYNDROME: • COMPLETE TRISOMY 21 • MOSAIC TRISOMY 21 • TRANSLOCATION TRISOMY 21 47
  • 48. IN GENETICS, A MOSAIC OR MOSAICISM DENOTES THE PRESENCE OF TWO OR MORE POPULATIONS OF CELLS WITH DIFFERENT GENOTYPES IN ONE INDIVIDUAL WHO HAS DEVELOPED FROM A SINGLE FERTILIZED EGG. 48
  • 49. CLINICAL FEATURES • UPWARD-SLANTED PALPEBRAL FISSURES • A LOW NASAL BRIDGE WITH EPICANTHAL FOLD • SMALL MOUTH AND EARS • SINGLE PALMER CREASE (SIMIAN CREASE) • A FLAT NASAL BRIDGE • SHORT AND WIDE PALMS • CHARACTERISTIC DERMATOGLYPHIC PATTERN. 49
  • 50. FRAGILE X SYNDROME • FRAGILE X SYNDROME IS THE SECOND MOST COMMON SINGLE CAUSE OF ID. • THE SYNDROME RESULTS FROM A MUTATION ON THE X CHROMOSOME AT WHAT IS KNOWN AS THE FRAGILE SITE (XQ27.3). • THE TYPICAL PHENOTYPE INCLUDES A LARGE, LONG HEAD AND EARS, SHORT STATURE, HYPER EXTENSIBLE JOINTS, AND POST PUBERTAL MACROORCHIDISM. • THE MENTAL RETARDATION RANGES FROM MILD TO SEVERE. 50
  • 51. FRAGILE X SYNDROME IS BELIEVED TO OCCUR IN ABOUT 1 OF EVERY 1,000 MALES AND 1 OF EVERY 2,000 FEMALES. 51
  • 52. PRADER-WILLI SYNDROME • PRADER-WILLI SYNDROME IS POSTULATED TO RESULT FROM A SMALL DELETION INVOLVING CHROMOSOME 15, USUALLY OCCURRING SPORADICALLY. • ITS PREVALENCE IS LESS THAN 1 OF 10,000. • PERSONS WITH THE SYNDROME EXHIBIT COMPULSIVE EATING BEHAVIOR AND OFTEN OBESITY, MENTAL RETARDATION, HYPOGONADISM, SMALL STATURE, HYPOTONIA, AND SMALL HANDS AND FEET. • CHILDREN WITH THE SYNDROME OFTEN HAVE OPPOSITIONAL AND DEFIANT BEHAVIOR. 52
  • 54. CRI-DU-CHAT SYNDROME • CHILDREN WITH CAT'S CRY SYNDROME LACK PART OF CHROMOSOME 5. 54
  • 55. • SEVERE RETARDATION • MICROCEPHALY • LOW-SET EARS • OBLIQUE PALPEBRAL FISSURES • HYPERTELORISM • MICROGNATHIA. • THE CHARACTERISTIC CAT-LIKE CRY CAUSED BY LARYNGEAL ABNORMALITIES THAT GAVE THE SYNDROME ITS NAME GRADUALLY CHANGES AND DISAPPEARS WITH INCREASING AGE. • DIFFICULTY SWALLOWING AND SUCKING • LOW BIRTH WEIGHT AND POOR GROWTH • HYPERACTIVE, AGGRESSIVE, AND REPETITIVE MOVEMENTS 55
  • 56. KLINEFELTER’S SYNDROME & TURNER’S SYNDROME • TURNER SYNDROME OCCURS IN 1/2500 LIVE BIRTHS. IT IS CAUSED BY A PARTIAL OR COMPLETE ABSENCE OF ONE OF THE X CHROMOSOMES IN A FEMALE. • IN THE CASE OF KLINEFELTER SYNDROME, THE MALE HAS 2 X CHROMOSOMES AND 1 Y CHROMOSOME 56
  • 57. KLINEFELTER’S SYNDROME & TURNER’S SYNDROME 57
  • 59. • CAUSED BY THE ABSENCE OF MATERIAL ON THE SEVENTH PAIR OF CHROMOSOME. • THEIR UNUSUAL SENSITIVITY TO SOUND MAKES THEM COMPETENT IN MUSIC AND LANGUAGE DESPITE OF THEIR LOW IQ LEVEL. WILLIAM’S SYNDROME
  • 60. WILLIAM’S SYNDROME PHYSICAL FEATURES • SHORT STATURE • ELFINLIKE FACIES • BROAD FOREHEAD • DEPRESSED NASAL BRIDGE • STELLATE PATTERN OF THE IRIS • WIDELY SPACED TEETH • FULL LIPS • RENAL AND CARDIOVASCULAR ABNORMALITIES • THYROID ABNORMALITIES • HYPERCALCEMIA BEHAVIOURAL FEATURES • ANXIETY • HYPERACTIVITY • OUTGOING • SOCIABLE • VERBAL SKILLS > VISUAL SPATIAL SKILLS 60
  • 61. CORNELIA DE LANGE SYNDROME • MULTIPLE GENES HAVE BEEN ASSOCIATED WITH THE CONDITION • SLOW GROWTH BEFORE AND AFTER BIRTH • INTELLECTUAL DISABILITY THAT IS USUALLY SEVERE TO PROFOUND • SKELETAL ABNORMALITIES INVOLVING THE ARMS AND HANDS • DISTINCTIVE FACIAL FEATURES • ARCHED EYEBROWS THAT OFTEN GROW TOGETHER IN THE MIDDLE (SYNOPHRYS) • LONG EYELASHES • LOW-SET EARS • SMALL, WIDELY SPACED TEETH • SMALL, UPTURNED NOSE. • MANIFEST SELF-INJURIOUS BEHAVIOR 61
  • 62. 62
  • 63. RETT'S DISORDER • RETT'S DISORDER IS HYPOTHESIZED TO BE AN X-LINKED DOMINANT SYNDROME THAT IS DEGENERATIVE AND AFFECTS MAINLY FEMALES. 63
  • 64. • DECELERATION OF HEAD GROWTH BETWEEN AGES 5 AND 48 MONTHS • LOSS OF PREVIOUSLY ACQUIRED PURPOSEFUL HAND SKILLS BETWEEN 5 AND 30 MONTHS WITH THE SUBSEQUENT DEVELOPMENT OF STEREOTYPED HAND MOVEMENTS (E.G., HAND-WRINGING OR HAND WASHING) • LOSS OF SOCIAL ENGAGEMENT EARLY IN THE COURSE ( ALTHOUGH OFTEN SOCIAL INTERACTION DEVELOPS LATER) • APPEARANCE OF POORLY COORDINATED GAIT OR TRUNK MOVEMENTS • SEVERELY IMPAIRED EXPRESSIVE AND RECEPTIVE LANGUAGE DEVELOPMENT WITH SEVERE PSYCHOMOTOR RETARDATION 64
  • 65. NEUROFIBROMATOSIS • ALSO CALLED VON RECKLINGHAUSEN'S DISEASE • NEUROFIBROMATOSIS IS THE MOST COMMON OF THE NEUROCUTANEOUS SYNDROMES CAUSED BY A SINGLE DOMINANT GENE, WHICH MAY BE INHERITED OR BE A NEW MUTATION. • THE DISORDER OCCURS IN ABOUT 1 OF 5,000 BIRTHS. • CHARACTERIZED BY • CAFE AU LAIT SPOTS ON THE SKIN AND BY NEUROFIBROMAS • OPTIC GLIOMAS • ACOUSTIC NEUROMAS, CAUSED BY ABNORMAL CELL MIGRATION. • MILD MENTAL RETARDATION OCCURS IN UP TO ONE THIRD OF THOSE WITH THE DISEASE. 65
  • 67. TUBEROUS SCLEROSIS • TUBEROUS SCLEROSIS IS THE SECOND MOST COMMON OF THE NEUROCUTANEOUS SYNDROMES; A PROGRESSIVE MENTAL RETARDATION OCCURS IN UP TO TWO THIRDS OF ALL AFFECTED PERSONS. • IT OCCURS IN ABOUT 1 OF 15,000 PERSONS AND IS INHERITED BY AUTOSOMAL DOMINANT TRANSMISSION. 67
  • 68. VOGT’S TRIAD • MENTAL RETARDATION • EPILEPSY • ADENOMA SEBACEUM 68
  • 70. ADRENOLEUKODYSTROPHY • THE MOST COMMON OF SEVERAL DISORDERS OF SUDANOPHILIC CEREBRAL SCLEROSIS, ADRENOLEUKODYSTROPHY IS CHARACTERIZED BY • DIFFUSE DEMYELINATION OF THE CEREBRAL WHITE MATTER RESULTING IN VISUAL AND INTELLECTUAL IMPAIRMENT • SEIZURES • SPASTICITY • PROGRESSION TO DEATH. • THE DISORDER IS TRANSMITTED BY A SEX-LINKED GENE LOCATED ON THE DISTAL END OF THE LONG ARM OF THE X CHROMOSOME. 70
  • 71. 71
  • 72. INBORN ERRORS OF METABOLISM 72
  • 73. PHENYLKETONURIA PKU IS TRANSMITTED AS A SIMPLE RECESSIVE AUTOSOMAL MENDELIAN TRAIT AND OCCURS IN ABOUT 1 OF EVERY 10,000 TO 15,000 LIVE BIRTHS. 73
  • 74. CLINICAL FEATURES • SEVERELY RETARDED, BUT SOME ARE REPORTED TO HAVE BORDERLINE OR NORMAL INTELLIGENCE. • ECZEMA • CONVULSIONS OCCUR IN ABOUT A THIRD OF ALL PATIENTS. • THEY FREQUENTLY HAVE TEMPER TANTRUMS • OFTEN DISPLAY BIZARRE MOVEMENTS OF THEIR BODIES AND UPPER EXTREMITIES, INCLUDING TWISTING HAND MANNERISMS • A MUSTY ODOR IN THE CHILD'S BREATH, SKIN OR URINE, CAUSED BY TOO MUCH PHENYLALANINE IN THE BODY • FAIR SKIN AND BLUE EYES, BECAUSE PHENYLALANINE CANNOT TRANSFORM INTO MELANIN — THE PIGMENT RESPONSIBLE FOR HAIR AND SKIN TONE • ABNORMALLY SMALL HEAD (MICROCEPHALY) 74
  • 75. MAPLE SYRUP URINE DISEASE • AN INBORN ERROR OF METABOLISM CAUSED BY DEFECTIVE OXIDATIVE DECARBOXYLATION OF KETO ACIDS OF LEUCINE, ISOLEUCINE, AND VALINE. • THESE BRANCHED-CHAIN AMINO ACIDS ARE PRESENT IN THE BLOOD AND URINE IN HIGH CONCENTRATIONS. • MANIFESTATIONS OF DISEASE INCLUDE FEEDING DIFFICULTIES, PHYSICAL AND MENTAL RETARDATION, AND A URINE ODOR SIMILAR TO THAT OF MAPLE SYRUP; NEONATAL DEATH IS COMMON. 75
  • 76. LESCH-NYHAN SYNDROME • LESCH-NYHAN SYNDROME IS A RARE DISORDER CAUSED BY A DEFICIENCY OF AN ENZYME INVOLVED IN PURINE METABOLISM. • THE DISORDER IS X-LINKED; PATIENTS HAVE MENTAL RETARDATION, MICROCEPHALY, SEIZURES, CHOREOATHETOSIS, AND SPASTICITY. 76
  • 78. • GALACTOSEMIA - INABILITY OF THE BODY TO USE SIMPLE SUGAR GALACTOSE • HUNTER SYNDROME – DEFECTIVE BREAKDOWN OF CHEMICAL MUCOPOLYSACCHARIDE. • TAY-SACHS DISEASE – ABSENCE OF HEX-A ENZYME. 78
  • 80. RUBELLA (GERMAN MEASLES) • THE MAJOR CAUSE OF MENTAL RETARDATION CAUSED BY MATERNAL INFECTION. • THE CHILDREN OF AFFECTED MOTHERS MAY SHOW SEVERAL ABNORMALITIES, INCLUDING CONGENITAL HEART DISEASE, MENTAL RETARDATION, CATARACTS, DEAFNESS, MICROCEPHALY, AND MICROPHTHALMIA. • THE INCIDENCE RISES TO ALMOST 50 PERCENT WHEN THE INFECTION OCCURS IN THE FIRST MONTH OF PREGNANCY. 80
  • 81. CYTOMEGALIC INCLUSION DISEASE • SOME CHILDREN ARE STILLBORN, AND OTHERS HAVE JAUNDICE, MICROCEPHALY, HEPATOSPLENOMEGALY, AND RADIOGRAPHIC FINDINGS OF INTRACEREBRAL CALCIFICATION. • THE DIAGNOSIS IS CONFIRMED BY POSITIVE FINDINGS OF THE VIRUS IN THROAT AND URINE CULTURES. 81
  • 82. SYPHILIS • SYPHILIS IN PREGNANT WOMEN WAS ONCE THE MAIN CAUSE OF VARIOUS NEUROPATHOLOGICAL CHANGES IN THEIR OFFSPRING, INCLUDING MENTAL RETARDATION. • TODAY, THE INCIDENCE OF SYPHILITIC COMPLICATIONS OF PREGNANCY FLUCTUATES WITH THE INCIDENCE OF SYPHILIS IN THE GENERAL POPULATION. 82
  • 83. • TOXOPLASMOSIS • IT CAUSES MILD OR SEVERE MENTAL RETARDATION AND, IN SEVERE CASES, HYDROCEPHALUS, SEIZURES, MICROCEPHALY, AND CHORIORETINITIS. • HERPES SIMPLEX • THE HERPES SIMPLEX VIRUS CAN BE TRANSMITTED TRANSPLACENTALLY, IT IS ONE OF THE MOST COMMON MODE OF INFECTION DURING BIRTH. • MICROCEPHALY, MENTAL RETARDATION, INTRACRANIAL CALCIFICATION, AND OCULAR ABNORMALITIES MAY RESULT. 83
  • 84. ACQUIRED IMMUNE DEFICIENCY SYNDROME (AIDS) • MANY FETUSES OF MOTHERS WITH AIDS NEVER COME TO TERM BECAUSE OF STILLBIRTH OR SPONTANEOUS ABORTION. • OF INFANTS BORN INFECTED WITH THE HUMAN IMMUNODEFICIENCY VIRUS (HIV), UP TO HALF HAVE PROGRESSIVE ENCEPHALOPATHY, MENTAL RETARDATION, AND SEIZURES WITHIN THE FIRST YEAR OF LIFE. 84
  • 85. PRENATAL USE OF TOXIC SUBSTANCES 85
  • 86. FETAL ALCOHOL SYNDROME • A TYPICAL PHENOTYPIC PICTURE OF FACIAL DYSMORPHISM THAT INCLUDES HYPERTELORISM, MICROCEPHALY, SHORT PALPEBRAL FISSURES, INNER EPICANTHAL FOLDS, AND A SHORT, TURNED-UP NOSE. • THE ENTIRE SYNDROME OCCURS IN UP TO 15 PERCENT OF BABIES BORN TO WOMEN WHO REGULARLY INGEST LARGE AMOUNTS OF ALCOHOL. 86
  • 87. PRENATAL DRUG EXPOSURE • COCAINE • HEROIN • DIAZEPAM • PHENOBARBITAL • CHLORPROMAZINE 87
  • 88.
  • 89. • THE INTELLECTUAL DISABILITY USUALLY RANGES FROM SEVERE TO PROFOUND. • THERE IS NO SPECIFIC TREATMENT AND LIFE EXPECTANCY IS LOW. MICROCEPHALUS HOLOPROSENCEPHALY (HPE, ONCE KNOWN AS ARHINENCEPHALY) IS A CEPHALIC DISORDER IN WHICH THE PROSENCEPHALON (THE FOREBRAIN OF THE EMBRYO) FAILS TO DEVELOP INTO TWO HEMISPHERES.
  • 90. • RESULTS FROM AN ACCUMULATION OF CEREBROSPINAL FLUID INSIDE OR OUTSIDE THE BRAIN. • THE DEGREE OF INTELLECTUAL DISABILITY DEPENDS ON HOW EARLY THE CONDITION IS DIAGNOSED AND TREATED. HYDROCEPHALUS
  • 92. COMPLICATIONS OF PREGNANCY • TOXEMIA OF PREGNANCY AND UNCONTROLLED MATERNAL DIABETES RESULT IN MENTAL RETARDATION. • MATERNAL MALNUTRITION DURING PREGNANCY OFTEN RESULTS IN PREMATURITY AND OTHER OBSTETRICAL COMPLICATIONS. • VAGINAL HEMORRHAGE, PLACENTA PREVIA, PREMATURE SEPARATION OF THE PLACENTA, AND PROLAPSE OF THE CORD CAN DAMAGE THE FETAL BRAIN BY CAUSING ANOXIA
  • 94. • PREMATURE INFANTS AND INFANTS WITH LOW BIRTHWEIGHT ARE AT HIGH RISK FOR NEUROLOGICAL AND INTELLECTUAL IMPAIRMENTS THAT APPEAR DURING THEIR SCHOOL YEARS. • INFANTS WHO SUSTAIN INTRACRANIAL HEMORRHAGES OR SHOW EVIDENCE OF CEREBRAL ISCHEMIA ARE ESPECIALLY VULNERABLE TO COGNITIVE ABNORMALITIES. • AMONG CHILDREN WITH VERY LOW BIRTHWEIGHT, 20 PERCENT HAS SIGNIFICANT DISABILITIES, INCLUDING CEREBRAL PALSY, MENTAL RETARDATION, AUTISM, AND LOW INTELLIGENCE WITH SEVERE LEARNING PROBLEMS. 94
  • 96. • NUTRITIONAL PROBLEMS • ADVERSE LIVING CONDITIONS • INADEQUATE HEALTH CARE • LACK OF EARLY COGNITIVE STIMULATION ENVIRONMENTAL AND PSYCHOSOCIAL PROBLEMS
  • 97. • CHILD ABUSE AND NEGLECT • TRAUMATIC BRAIN INJURY • VIRAL INFECTIONS LIKE MENINGITIS OR ENCEPHALITIS • HEAVY METAL POISONING ESP. LEAD POISONING ENVIRONMENTAL AND PSYCHOSOCIAL PROBLEMS
  • 99. COMMON COMORBID PSYCHIATRIC DISORDERS • INTELLECTUAL DISABILITY IS A SIGNIFICANT RISK FACTOR FOR PSYCHOPATHOLOGY IN GENERAL. • PSYCHIATRIC DISORDERS ARE THREE TO FOUR TIMES HIGHER IN PEOPLE WITH AN ID DIAGNOSIS THAN IN THE GENERAL POPULATION. • THE PREVALENCE OF PSYCHIATRIC DISORDERS IN INDIVIDUALS WITH ID IS ESTIMATED TO BE BETWEEN 30% AND 60% 99
  • 100. • i. INTERNATIONAL STUDIES • ERIC EMERSON ET AL (2007) IN A STUDY CONDUCTED IN BRITAIN FOUND THE PREVALENCE OF PSYCHIATRIC DISORDERS WAS 36% AMONG CHILDREN WITH INTELLECTUAL DISABILITY AND 8% AMONG CHILDREN WITHOUT • II. INDIAN STUDIES • A STUDY WAS CONDUCTED BY C.R.J. KHESS ET AL (1998), AT THE CHILD PSYCHIATRIC UNIT (CPU.) OF THE CENTRAL INSTITUTE OF PSYCHIATRY (C.I.P.) OVER A ONE YEAR PERIOD THE STUDY SAMPLE CONSTITUTED OF 60 PATIENTS, FOUND PSYCHIATRIC COMORBIDITY WAS PRESENT IN 34 (56.67%) PATIENTS. 100
  • 101. • WHEN ASSESSING FOR PSYCHIATRIC DISORDERS IN PEOPLE WITH AN ID, ASSESSMENT PROCEDURES MUST BE MODIFIED TO TAKE INTO ACCOUNT ASSOCIATED DISORDERS, SUCH AS COMMUNICATION DISORDERS, AUTISM SPECTRUM DISORDER, AND MOTOR, SENSORY, OR OTHER CO-OCCURRING CONDITIONS. • KNOWLEDGEABLE INFORMANTS ARE ESSENTIAL DURING ASSESSMENT TO IDENTIFY CHANGES IN IRRITABILITY, MOOD REGULATION, INCREASED AGGRESSION, EATING PROBLEMS, SLEEP PROBLEMS, AND CHANGES IN ADAPTIVE BEHAVIOUR AT WORK, AT HOME, AND IN THE COMMUNITY SETTINGS. 101
  • 102. THE MOST COMMON PSYCHIATRIC COMORBIDITIES ASSOCIATED WITH THE INTELLECTUALLY DISABLED ARE : • ATTENTION-DEFICIT/HYPERACTIVITY DISORDER • AUTISM SPECTRUM DISORDER • ANXIETY DISORDERS • BIPOLAR AND RELATED, DISORDER • DEPRESSIVE DISORDER • SCHIZOPHRENIA SPECTRUM AND OTHER PSYCHOTIC DISORDER • FEEDING AND EATING DISORDER • DISRUPTIVE, IMPULSE CONTROL AND CONDUCT DISORDERS • SOMATIC SYMPTOM & RELATED DISORDER 102
  • 103. ATTENTION-DEFICIT/HYPERACTIVE DISORDERS • THE PREVALENCE OF ADHD IN CHILDREN WITH ID IS ESTIMATED TO BE BETWEEN 8.7% AND 16%, COMPARED WITH 5% IN THE GENERAL POPULATION. • FOR PERSONS WITH ID, THE DIAGNOSIS OF ADHD DEPENDS UPON THE PRESENCE OF SYMPTOMS EXCESSIVE FOR AN INDIVIDUAL’S MENTAL AGE OR REMARKABLE COMPARED WITH PEERS WHO HAVE SIMILAR LEVELS OF RETARDATION. 103
  • 104. • A STUDY CONDUCTED IN THE CHILD PSYCHIATRY UNIT OF A TERTIARY PSYCHIATRIC HOSPITAL BY KHESS R.J. ET AL.78 EVALUATED THAT (14%) OF THE STUDY SUBJECTS HAD COMORBID HYPERKINETIC DISORDER. • A STUDY CONDUCTED IN THE DEPARTMENT OF PSYCHIATRY, SMCH, INCLUDING 100 ID PATIENTS, SHOWED THE PREVALENCE OF ADHD TO BE 11% 104
  • 105. AUTISM SPECTRUM DISORDER • IT HAS BEEN FOUND THAT INCREASED SEVERITY OF ID IS POSITIVELY CORRELATED WITH THE INCIDENCE OF AUTISM. • IN FACT, IT CAN SOMETIMES BE DIFFICULT TO DISTINGUISH AUTISM FROM SEVERE ID. • HISTORICALLY, THE PREVALENCE OF INTELLECTUAL DISABILITY IN AUTISM IS ESTIMATED AT 70%, BUT RECENT STUDIES, SUGGEST THAT THE PREVALENCE OF INTELLECTUAL DISABILITY IN AUTISM-SPECTRUM CONDITIONS MAY BE CONSIDERABLY LOWER. • IT HAS BEEN SUGGESTED THAT THE ASSOCIATION BETWEEN AUTISM AND INTELLECTUAL DISABILITY MAY BE INFLATED BECAUSE OF CLINICAL ASCERTAINMENT BIAS. 105
  • 106. • Cooper and colleagues FOUND THE PREVALENCE OF AUTISM SPECTRUM DISORDER TO BE 4.4% IN PERSONS WITH ID. • A STUDY CONDUCTED IN THE CHILD PSYCHIATRY UNIT OF A TERTIARY PSYCHIATRIC HOSPITAL BY Khess r.J. Et al. EVALUATED THAT (11%) OF THE STUDY SUBJECTS HAD AUTISM. • IN OUR STUDY WE FOUND THE PREVALENCE TO BE 3% 106
  • 107. ANXIETY DISORDERS • FOR PEOPLE WITH INTELLECTUAL DISABILITY, THE PREVALENCE OF ANXIETY DISORDERS HAS BEEN SHOWN TO BE MUCH HIGHER. • THE VARIABILITY OF PREVALENCE IS FROM 1% TO 25%. THIS MAY BE DUE TO REDUCED COGNITIVE ABILITIES AND INCREASED VULNERABILITY TO ENVIRONMENTAL DEMANDS. • IN OUR STUDY WE FOUND THE PREVALENCE TO BE 3% • AN INDIVIDUAL WITH INTELLECTUAL DISABILITY MAY NOT BE ABLE TO IDENTIFY SUBJECTIVE ANXIETY AS AN UNDERLYING CAUSE OF DISTRESS AND THE RESULTING AGITATION AND AGGRESSION MAY BE SUGGESTIVE FOR A DIAGNOSIS OF IMPULSE CONTROL DISORDER RATHER THAN REFLECTING THE UNDERLYING ANXIETY. 107
  • 108. • FREQUENT SYMPTOMS OF ANXIETY IN INTELLECTUALLY DISABLED PERSONS INCLUDE AGGRESSION, AGITATION, SELF-INJURY, AND INSOMNIA. THESE SYMPTOMS ARE OFTEN DIFFICULT TO DISTINGUISH FROM IMPULSE CONTROL DISORDERS. • ANXIETY DISORDERS OR POSTTRAUMATIC STRESS DISORDER MAY PRESENT IN RESPONSE TO FRUSTRATION OR THREATENING SITUATIONS; FEARFULNESS AND AVOIDANT BEHAVIOURS. • PANIC ATTACKS MAY RESEMBLE RAGE OUTBURSTS, AND RESULT IN AGGRESSION OR SELF-INJURY. 108
  • 109. BIPOLAR AND RELATED DISORDER • AFFECTIVE DISORDERS IN ALL FORMS DO OCCUR IN PERSONS WITH MENTAL RETARDATION. • THE PRESENCE AND DEGREE OF MENTAL RETARDATION MODIFY MANIFESTATIONS OF THESE DISORDERS. • DIAGNOSIS IS DIFFICULT BECAUSE OF THE ABSENCE OF CLASSICAL MANIFESTATIONS AND FREQUENT OCCURRENCE OF INTER-EPISODE BEHAVIOUR DISTURBANCES 109
  • 110. • A STUDY CONDUCTED IN THE CHILD PSYCHIATRY UNIT OF A TERTIARY PSYCHIATRIC HOSPITAL BY KHESS R.J. ET AL. EVALUATED THAT (8%) OF THE STUDY SUBJECTS HAD COMORBID MOOD DISORDER. • IN OUR STUDY WE FOUND THE PREVALENCE TO BE 6% 110
  • 111. DEPRESSIVE DISORDER • OWING TO INTELLECTUAL AND COMMUNICATION LIMITATIONS, IT IS DIFFICULT TO USE THE PSYCHIATRIC DIAGNOSTIC INTERVIEW AND APPLY STANDARD DIAGNOSTIC CRITERIA TO THIS POPULATION. THUS, DEPRESSION MAY BE MISDIAGNOSED OR UNDERDIAGNOSED. • THE POINT PREVALENCE OF DEPRESSION AMONG ADULTS WITH INTELLECTUAL DISABILITY IS ESTIMATED TO BE 4% (COOPER & BAILEY, 2001). • IN OUR STUDY WE FOUND THE PREVALENCE TO BE 4% 111
  • 112. SCHIZOPHRENIA SPECTRUM AND OTHER PSYCHOTIC DISORDER • INDIVIDUALS WITH INTELLECTUAL DISABILITY EXHIBIT THE SAME TYPES OF PSYCHOTIC DISORDERS AS ADULTS OF NORMAL INTELLIGENCE, ALTHOUGH AN ACCURATE DIAGNOSIS IS OFTEN DIFFICULT TO MAKE. • DIAGNOSTIC OVERSHADOWING, FOR EXAMPLE, IN WHICH ABNORMAL BEHAVIOURS ARE ASSUMED TO BE THE RESULT OF ID RATHER THAN POTENTIAL COMORBID PSYCHOPATHOLOGY, MAY OBSCURE IDENTIFICATION OF PSYCHIATRIC CONDITIONS. • A STUDY BY PENROSE REPORTED THAT 3.8% OF 1280 INDIVIDUALS WITH INTELLECTUAL DISABILITY HAD SCHIZOPHRENIA AND 1.9% HAD AFFECTIVE PSYCHOSES. 112
  • 113. • C.R.J. KHESS ET AL (1998) IN HIS STUDY REPORTED THAT 11.76% OF THE PATIENTS WERE FOUND TO HAVE PSYCHOSIS, BUT NONE FULFILLED THE CRITERIA FOR SCHIZOPHRENIA • IN OUR STUDY WE FOUND THE PREVALENCE TO BE 14% 113
  • 114. FEEDING AND EATING DISORDER • PICA AND RUMINATION SEEMS TO BE THE MOST COMMON EATING DISORDER IN THE INTELLECTUALLY DISABLED. • THE INGESTION OF NON-NUTRITIVE SUBSTANCES, REGURGITATION AND RUMINATION OCCURS WITH GREATER FREQUENCY AS THE SEVERITY OF COGNITIVE DISABILITY INCREASES. • ANOREXIA NERVOSA AND BULIMIA NERVOSA ARE RELATIVELY UNCOMMON IN THE INTELLECTUALLY DISABLED 114
  • 115. • DESPITE DIAGNOSTIC AND METHODOLOGICAL DIFFICULTIES, PSYCHOPATHOLOGY AND EATING DISORDER RESEARCH STUDIES SUGGEST THAT 3-42% OF INSTITUTIONALIZED ADULTS WITH ID AND 1-19% OF ADULTS WITH ID IN THE COMMUNITY HAVE DIAGNOSABLE EATING DISORDERS (Gravestock S) • IN OUR STUDY WE FOUND THE PREVALENCE TO BE 6% 115
  • 116. DISRUPTIVE, IMPULSE CONTROL AND CONDUCT DISORDERS • IMPULSE CONTROL DIFFICULTIES REPRESENT A MAJOR FEATURE OF A NUMBER OF BEHAVIOURAL AND MENTAL DISORDERS IN CHILDREN, YOUTH, AND ADULTS WITH INTELLECTUAL DISABILITIES (ID). • Khess R.J. et al.IN THEIR STUDY EVALUATED THAT (2%) OF THE STUDY SUBJECTS HAD COMORBID CONDUCT DISORDER. • IN OUR STUDY WE FOUND THE PREVALENCE RATE TO BE 3% 116
  • 117. SOMATIC SYMPTOM & RELATED DISORDER • SOMATIC SYMPTOM & RELATED DISORDER IS LARGELY COMMON IN PATIENTS WITH ID. • MOSTLY, IT IS SEEN IN PATIENTS WITH MILD AND MODERATE ID. • HOWEVER, THE PREVALENCE RATE IS NOT SO WELL RESEARCHED. • IN OUR STUDY, WE FOUND THE PREVALENCE TO BE 5% 117
  • 119. • SLAYTER (2010) REVIEWED HEALTHCARE BILLING CLAIMS AND CONCLUDED THAT 2.6% OF ALL PEOPLE WITH ID HAD A DIAGNOSABLE SUBSTANCE ABUSE DISORDER. • OTHER ESTIMATES USING DIFFERENT METHODOLOGIES VARY WIDELY, RANGING AS HIGH AS 26% (STRAIN, BUCCINO, BROONER, SCHMIDT, & BIGELOW, 1993). • WHEN A PERSON WITH ID ALSO HAS A MENTAL ILLNESS OR A DUAL DIAGNOSIS, THE ESTIMATES OF CO-OCCURRING SUBSTANCE ABUSE RANGE FROM 7% TO 20% (SINCLAIR, 2004). • THE RESEARCH IN THIS AREA IS SPARSE, BUT THE NUMBERS ARE ALARMING. 119
  • 120. • PEOPLE WITH ID WHO ARE SUBSTANCE ABUSERS SHARE SOME UNIQUE TRAITS. • AS A GROUP, THEY TEND TO BEGIN DRINKING ALCOHOL A COUPLE YEARS LATER THAN THEIR PEERS WITHOUT ID (SLAYTER). • THEY ARE LESS LIKELY TO SEEK HELP FOR THEIR PROBLEM AND EVEN WHEN THEY DO, THE RESOURCES THAT ARE HELPFUL FOR THE GENERAL PUBLIC FALL SHORT OF MEETING THEIR NEEDS. • ADDITIONALLY, THIS GROUP IS AT GREATER RISK OF COMPLICATIONS FROM DRINKING BECAUSE THEY TEND TO BE PRESCRIBED MEDICATIONS FOR OTHER CONDITIONS, SUCH AS SEIZURES, METABOLIC DISORDERS, AND CO- OCCURRING MENTAL ILLNESS THAT MIGHT NEGATIVELY INTERACT WITH ALCOHOL AND DRUGS. 120
  • 121. 121 SEIZURE DISORDER IN INTELLECTUAL DISABILITY
  • 122. SEIZURE DISORDER IN INTELLECTUAL DISABILITY • WHEN CONSIDERED ACROSS THE SPECTRUM OF INTELLECTUAL DISABILITY, THE FREQUENCY AND SEVERITY OF EPILEPSY VARIES INVERSELY WITH THE LEVEL OF MENTAL RETARDATION (ROBERTS, 1986). • AMONG INDIVIDUALS WITH MILD RETARDATION, THE DISTRIBUTION OF VARIOUS SUBTYPES OF SEIZURES APPROXIMATES THE GENERAL POPULATION. • AMONG INDIVIDUALS WITH SEVERE/PROFOUND RETARDATION, INCREASED RATES OF UNDERLYING NEUROLOGICAL ABNORMALITIES ARE MANIFESTED BY A HIGHER FREQUENCY OF MIXED SEIZURE DISORDERS, AND AN ONSET OF SEIZURES IN INFANCY OR EARLY CHILDHOOD (RATEY & DYNEK, 1996; ROBERTS, 1986). 122
  • 123. • COMMUNITY-BASED STUDIES OF EPILEPSY IN ADULTS WITH ID SHOW A PREVALENCE OF 16–26%. BOWLEY, C. ET AL (2000). • IN OUR STUDY WE FOUND THE PREVALENCE RATE TO BE 22% 123
  • 124. TO BE CONTINUED….. IN PART - II • MANAGEMENT OF INTELLECTUAL DISABILITY • SERVICES AND SUPPORT • LEGAL ASPECTS 124