1. Behavioral
development, DQ, IQ
Dr. Ravi Soni

2. Introduction
• Maturation of different biological functions-
milestones
• Anticipated at a particular age
• Due consideration given to environmental and
social factors

3. Gross Motor
Age in months Activity
3 Neck holding
5 Roll over
6 Sits with support
8 Sits without support, crawling,
9 Stands with support
12 Creeps, walks but falls, stands without support
15 Walks alone, creeps upstairs
18 Running
24 Walks stairs 2 feet at a time
36 Walks up stairs alternate feet, rides tricycle
48 Walks down stairs alternate feet, hopping

4. Fine Motor
Age in months Activity
4 Bidextrous reach
6 Unidextrous reach and transfer, biscuit to mouth (Mouthing)
9 Immature pincer
12 Mature pincer, feeds from cup with spilling
15 Imitates Scribbles, 2 blocks, picks up glass and drinks
18 Scribbles, 3 blocks, feeds with spoon without spilling
24 Copies straight line, 6 blocks
36 Copies circle
48 Copies plus
60 Copies triangle
72 Copies rectangle
84 Copies diamond

5. Language
Age in months Activity
1 Alerts to sounds
3 Cooing
4 Laughs aloud
6 Mono syllables
9 Bi syllables
12 1-2 words with meaning
18 8-10 words
24 2 word sentences
36 Gender identity, full name
48 Story telling, songs, poems
60 Asks meaning of words

6. Personal Social skills
Age in months Activity
2 Social smile
3 Recognizes mother
6 Stranger anxiety
9 Waves bye-bye
12 Plays ball game
18 Copies parents in task
24 Asks for food
36 Shares toys
48 Plays in a group
60 Dressing, undressing, shoe lace tying

7. Developmental Quotient
Up to 6 yrs. Screening tools:
DA/CA *100  Denver II
 <20- Profound DD  Bharatraj DST
 20-34- Severe DD  Phataks’ Baroda DST
 35-49- Moderate DD  Trivandrum DST
 50-69- Mild DD Formal Tests:
 70-79- Borderline dev.  Gessell development
 80-84- Below average dev. schedules
 85-114- Average dev.  Nancy Bayley scale of
 >115- Above average dev. development
Social / Adaptive scales:
 Vineland social maturity
scale

8. DQ:

9. Intelligence Quotient
 >6 yrs.  Screening tests:
 MA/CA *100  Peabody Picture vocabulary
test III
 Cut off- 70
 Draw a person task
 Kaufman brief intelligence
test
 Formal tests:
 Wechsler intelligence test
 Stanford Binet test
 Binet Kamat test

10. MENTAL RETARDATION/
INTELLECTUAL DISABILITY
Dr. Ravi Soni

11. Definitions
 1910: Presence of a mental defect, inability to manage
ordinary affairs
 Idiots: Mental age 2 yrs. or younger
 Imbeciles: Mental age 2-7 yrs.
 Morons: Mental age 7-12 yrs.
 1959 (AAMR): 3 diagnostic criteria
 Sub-average intellectual functioning (1 SD below; IQ<=85)
 Impairment in adaptive behavior
 Onset < 16 yrs.
 5 degrees- Borderline, mild, moderate, severe and profound
 1973:
 IQ<=70, 2SD below
 Onset < 16 yrs
 No borderline category

12. AAIDD- 2002 definition
Significant limitations in
 Intellectual functioning and
 Adaptive behavior- conceptual, social and practical
adaptive skills
 Onset < 18 yrs.
Assumptions essential
 Limitations in the context of community environments
 Considering cultural and linguistic diversity
 Limitations co exist with strengths
 Limitations: Provision of support
 Strengths: To improve life functioning

13. DSM IV TR (2000):
 Significantly Sub-average intellectual functioning IQ<= 70
 Deficits in Adaptive behavior in at least two of the
following areas: communication, self care, home living,
social/interpersonal skills, use of community resources,
self direction, functional academic skills, work, leisure,
health and safety.
 Onset before 18 years
 Mild: 50-55 to 70
 Moderate: 35-40 to 50-55
 Severe: 20-25 to 35-40
 Profound: <20-25
 Mental Retardation, severity unspecified

14. History:
 1st century- Avicenna- various levels of intelligence
 2nd century- Talmud- “ Shoteh”- wanders alone, tears
clothes, sleeps in cemetery
 1534- Fitz Herbert- earliest Intelligence test-
 17th century- John Locke- differentiated MR from
Mental Illness
 1800- Pinel- “Moral treatment of Mental Patients”
 Seguin- First need school in Paris, Seguin Form Board,
1st President of AAMR

15. History:
 1905- Binet and Simon- First version of the
intelligence test
 Penrose- 1st scientific study on ID
 Gessell- Schedule for development
 Change in terminology
• Shoteh
• Idiots, Imbeciles, Morons
• Mental Retardation
• Intellectual disability

16. Classification
Degree of MR with IQ Adult attainment
Literacy ++ (6th grade)- educable
Mild (50-69) Self-help skills ++
85% Good speech ++
Semi-skilled work +
Literacy + (2nd-3rd grade)
Self-help skills ++-Trainable
Moderate (35-49)
Domestic speech +
10%
Unskilled work with or without
supervision +
Assisted self-help skills +
Severe (20-34)
Minimum speech +
3-4%
Assisted household chores +
Speech: Utterances of words +/-
Profound (Below 20)
Self-help skills +/-
1-2%
Sensory- motor impairments+

17. • Prevalence:
• Mild MR sometimes goes unrecognized until
middle childhood.
• M:F ratio
• 40% between 4 and 18 years of age met
criteria for at least one psychiatric disorder.

18. Prenatal causes
 Maternal Toxins
 FAS, FHS
 Others:
 Toxemia, IUGR, Radiation, Trauma
 Familial MR

19. Prenatal causes
 Genetic causes
 Chromosomal Aberrations:
 Trisomy 21, 13, Cri-du-Chat Syndrome
 Micro deletions:
 Angelmans’ Prader-Willi, Williams’ Syndrome, Rubinstein- Tabyi
syndrome
 Monogenic Mutations:
 Tuberous Sclerosis, Fragile X syndrome, Metabolic disorders
 Malformations:
 Holoprosencephaly, Lissencephaly, Neural tube defects
 Maternal infections:
 TORCH, HIV

20. Perinatal causes
 Infections:
– Meningitis, herpes
 Labor complications
– Trauma, Asphyxia
 Others
– Hypoglycemia, Hyperbilirubinemia, Seizures

21. Postnatal Causes
 Infections:
– Meningitis, herpes
 Toxins:
– Lead poisoning
 Others:
– CVA, Tumors, Trauma

22. Environmental and sociocultural
factors
• Significant deprivation of nutrition and nurturance
• Poor medical care, poor maternal nutrition
prenatally
• Teenage pregnancies
• Poor postnatal care, malnutrition, exposure to toxic
substances ( lead ), physical trauma
• Family instability, multiple but inadequate
caretakers
• Incapacitating mental disorder in parent

23. Associated Psychiatric problems
(Dual Diagnosis)
 Axis I + Axis II Disorders
 Brain damage or dysfunction + social and family
factors - psychiatric disorders
 3 to 5 times more frequent than in general
population
 Full range of psychiatric disorders
 Source of high parental stress and social
embracement

24. Highly Prevalent Psychiatric
Symptoms
Hyperactivity
Short attention span
Self injurious behaviors
Repetitive stereotypal behaviors

25. Psychiatric illness in ID
Assessment:
Difficult representation of sample
Inappropriate developed tests, criteria- Difficulties in
using diagnostic criteria, scales and tools etc. due to deficits in abstract
thinking and poor communication skills (intellectual distortion)
Diagnostic overshadowing
Information from the patients unreliable
Cognitive impairments
 communicative skills- behavioral responses
like aggression, irritability

26. Personality styles and Traits
Negative self image
Low self esteem
Poor frustration tolerance
Interpersonal dependence
Rigid problem solving

27. Psychiatric illness in ID
Psychological factors
 Low self image
 Outer-directedness, learned helplessness
 Sense of isolation and inadequacy
 Repeated failures and disappointment
Environmental factors:
 Social rejection and stigma, peer attitudes, abuse
potential
Medical:
 Seizures, sensory, motor impairment, medication side
effects

28. Behavioral phenotypes
 Specific behaviors characteristically associated
with specific genetic conditions
 Nyhan 1972
 Does not mean only genetic determinism
 But a combination of genetic, environmental,
social and biological factors

29. Behavioral Phenotypes: Examples
Behavior Syndrome
Hyperphagia, obsessions & Prader-Will syndrome
compulsions, skin-picking
Hand-wringing Rett syndrome
Self mutilation Lesch-Nyan syndrome
Inappropriate laughter Angelman syndrome
Cat cry Cri-du-chat syndrome
Social anxiety, gaze aversion Fragile X syndrome
Psychosis VCFS, Prader-Willi Syndrome

30.  Externalizing disorders: manifested in children’s outward
behavior rather than their internal thoughts and feelings.
 ADHD
 Oppositional defiant disorder
 Conduct disorder
 Mixed presentations
 ODD and Conduct disorder are considered as ‘Disruptive Behavior’.
 Internalizing disorders:
 Anxiety disorder
 Phobias, generalized, panic
 Separation anxiety disorder
 Social anxiety disorder
 Depression
 Obsessive compulsive disorder

31. Mental retardation and Psychosis
 Hallucinatory behavior, fearfulness, paranoia,
withdrawn behavior, negative Symptoms, catatonic
Symptoms, disorganized speech, disorganized
thought, psychomotor agitation, aggression, Self
Injurious Behaviors are frequently reported
 Genetic syndromes such as VCFS and Prader-Willi
may present with MR and psychosis

32. Mental retardation and Psychosis
(cont)
 Unusual manifestations:
 Staring to side
 Nodding and gesticulating as if listening to some one
 Shadow boxing with unseen others
 Covering eyes or ears as if shutting out stimuli
 Placing unusual wrappings around neck, wrist or ankles
 Inspecting food with new and out-of-context intensity
 Grimacing or wincing as if smelling or tasting something
foul

33. Mental retardation and Affective
disorders
 Affective disorders in all forms do occur
 Classical criteria may not be elicited, instead behavioral
equivalents are commonly seen
 Depressive equivalents : irritability, unexplained temper
tantrums or aggression
 Vegetative, affective, motor, and behavioral symptoms are
common and thinking and perceptual symptoms are less
common
 Atypical presentations such as mixed episodes, rapid cycling
are common

34. Mental retardation and Affective
disorders (Cont)
Unusual manifestations
 Mania: excessive laughing, clapping, over familiarity,
wandering, talking about marriage, sexual disinhibition
e.g. hugging people of opp. sex, excessive use of
cosmetics, talking authoritatively, demanding special
foods, drinking too much fluids, using bad language,
stubbornness, singing & dancing, and collecting
rubbish
 Depression: Clinging to mother, weeping, being dull,
talking less than usual, sleep and app disturbances,
withdrawn, aches & pains

35. Mental retardation and ADHD
 ADHD is reported as common co morbid psychiatric
disorder with a prevalence rate of 8.7 – 16%
 Children with mild MR scored more on dimension
of ‘disruptive behavior’
 Multiple co-morbidity is common
Mental retardation and PDD
 Around 75% of children with PDD meet the criteria
for MR
 Common in some genetic conditions such as fragile
X, tuberous sclerosis and PKU

36. Instruments specific to this
population:
 PAS-ADD: Psychiatric Assessment Schedule for Adults
with Developmental Disability (Moss et al, 1998)
 RSMB: Reiss Screen for Maladaptive Behavior (Reiss,
1988)
 PIMRA: Psychopathology Inventory for Mental
Retardation in Adults (Senatore et al, 1985)
 DBC: Developmental Behavior Checklist

37. Common Associated Physical Problems
 Seizure disorder
 Cerebral palsy
 Visual impairment
 Hearing impairment
 Congenital heart disease
 Cleft lip and cleft palate
 Nutritional deficiencies
 Recurrent infections
 Feeding disorders
 Skin problems
 Dental problems

38. Important clinical questions
 Reasons for consultation
 Developmental delay : global vs. restricted
 Severity of delay or retardation
 Detectable causes
 Associated medical problems
 Associated psychiatric problems
 Assessment of awareness amongst family
 Parental expectations
 What and how to disclose

39. Clinical evaluation
History taking
Physical examination
 head-to-toe examination
 look for sensory impairment
 major congenital anomalies
 minor congenital anomalies (4 or more MCAs -
prenatal diagnosis)
Psychological assessment
Physical investigations
Comprehensive diagnosis

42. Common syndromes:
Syndrome Key features
Downs syndrome Typical facies, short stature, slanting
eyes, simian crease, cup-shape ears,
clinodactyly, CHD,
Fragile X syndrome Elongated triangular face, protruding
or prominent ears, macro-orchidism
Angelman syndrome Dysmorphic face - wide mouth, large
tongue, thin upper lip, seizures, ataxia
Prader-Willi syndrome Obesity, short stature, small hands/
feet, hypotonia
Tuberous sclerosis Sebaceous adenomas, ash-leaf
macules, shagreen patches

43. Common syndromes:
Syndrome Key features
MPS I& II Typical facies, coarse skin, skeletal
anomalies, corneal clouding,
hepatosplenomegaly
Phenylketonuria Light colored hair, abnormal smell of
urine, microcephaly and seizures
Autosomal recessive Severe congenital microcephaly with
microcephaly mild to mod MR
Rubinstien Taybi syndrome Prominent beak-shaped nose, broad
thumb and hallux
Cong Hypothyroidism Lethargy, growth failure, coarse and
dry skin, constipation, feeding
problems, prominent abdomen,
bradycardia

44. Downs Syndrome

45. Fragile X syndrome

46. Angelmann Syndrome:

47. Prader Willi Syndrome:

48. William Syndrome:
Showing low set and posteriorly
Elfin Facies: rotated ears:

49. Tuberous Sclerosis:

50. Cri Du Chat Syndrome

51. Rubinstein Taybi Syndrome:

52. Course and Prognosis

53. MANAGEMENT
BIO-PSYCHO-SOCIAL MODAL

54. Management principles
 Collect good baseline information including pre-morbid
states
 Detailed history of evolution of symptoms including onset,
precipitating factors etc.
 Encourage family and individual to speak and listen to them
genuinely
 Careful observation and analysis of behavioral profile
 Plan for an individualized comprehensive multi-modal
intervention package
 Use the knowledge and support from care takers and family
(collateral history)
 Regular periodical reviews

55. Management principles (Cont)
 Early detection &  Developmental
intervention Assessment including
 Parent counseling & IQ
training, Parent  Skills training (e.g.
management training Social, communication)
 Pharmacological Rx  Age appropriate
 Behavior modification concepts development
 Individual counseling
 Normalization
 Habilitation

56. Normalization
• Mid 1800 – Institutionalization
• After mid – 1900 – Deinstitutionalization, With the
philosophy of ‘Normalization’ in living situations and
‘Inclusion’ in educational settings.
• “The education for all Handicapped Children Act”
passed in 1975 mandates the public school system to
provision of appropriate educational service to all
children with disabilities.
• Currently provision for all children, including those
with disabilities, ”within the least restrictive
environment” is mandated by law.

57. Management

58. Investigations
 Urine screen for abnormal metabolites: Phenyketonuria,
homocysteinuria, galactosemia, MPS (Heparan Sulfate)
 Thyroid function test: Hypothyroidism
 Advanced metabolic tests (Gas chromatographic Mass
Spectroscopic (GCMS), tandem mass spectroscopy
(TMS):Wide range of neuro-metaboloic disorders such as
fatty acid oxidation disorders, aminoacidopathies, urea
cycle disorders and organic acidurias
 Enzyme studies: Tay-Sachs disease (Hexosaminidase),
metachromatic leukodystrophy (Sulphatase A)
 Karyotyping: Down syndrome, other chromosomal
disorders

59. Investigations
 FISH: Prader -Willi syndrome, William syndrome, Sub-
telomeric deletions
 Molecular genetics : Fragile X syndrome (FMR1 mutation),
Rett syndrome (MECP2 mutation),
 Brain imaging: Tuberous sclerosis, lissencepahly,
Holoprosencephaly
 EEG: Epileptic encephalopathies such as West syndrome
( infantile spasms, Hypsarrhythmia, deve. regression )
 Visual evaluation: Cataract, Optic atrophy, cortical
blindness, refractive error
 Immunologic tests (Ig M antibodies): TORCH infections

60. Pharmacotherapy
 Around 20-45% of persons with MR are given
psychotropics (Unwin GL, 2008)
 Persons with MR respond to psychotropic
medications in ways similar to the typically
developing individuals
 Rates of response tend to be poorer and
occurrence of side effects tends to be more
frequent
 Greater monitoring, use of lower doses and
slower dosage increments seem to be essential
steps

61. Pharmacotherapy (Cont)
Circumstances for the use of
medication:
 Failure of non-drug interventions
 Risk of harm to self or others or property
 High frequency/severity of behavioral
problem
 To treat underlying psychiatric disorders

62. Some guidelines while starting a
medication:
 Prior to prescription:
 Medical and psychosocial causes for behavioral disorder must be ruled
out
 Psychiatric diagnoses should be seriously tried
 The least intrusive and most positive interventions should be used
 When medication is prescribed:
 It should be integral part of treatment plan
 It should not diminish the patients’ functional status
 The lowest effective dose should be tried
 Dose reduction should be considered periodically unless clinically
contraindicated
 Adverse effects should be monitored periodically

63. Pharmacotherapy (Cont)
 Anti-psychotics:
 Atypicals are preferred over typicals
 EPS and sedation are more frequently reported
 Frequent monitoring and rationalization are must
 Try to taper, stop and reassess the need to continue
 May not require for prolonged durations
 Anti cholinergic medication can be used if individual
requires
 Anti convulsants:
 Seizures- common co morbid condition
 Sodium valproate
 Carbamazepine contraindicated in myoclonic seizures
 Phenobarbitone- hyper activity

64. Pharmacotherapy (Cont)
Rx of ADHD
 Psycho stimulants: Methylphenidate,
amphetamines
 Most frequently prescribed medication in this
population
 IQ above 50 – better response
 Greater risk for side effects such as tics and social
withdrawal, worsening of seizures
 Clonidine may be used in some children with
hyper arousal, aggressive hyperactivity and tics

65. Aggression and its various causes
Medical illness Treat medical condition
Pre-seizure irritability Anticonvulsants
Mood disorders SSRIs or mood stabilizers
Task related anxiety CBT and anti anxiety drugs
Associated with delusions Anti-psychotics
Inability to express needs Teach functional
communication skills

66.  Aggression and self injurious behavior:
lithium, naltrexone, carbamazepine,
valproate, risperidone.
 Stereotypical motor movements: anti
psychotics, SSRIS.
 Explosive rage behavior: Beta blockers,
antipsychotics.

67. BM Techniques for building new
skills:
 Goal specification: Specified description of desired behavior
to be learnt, based on current skills level and needs
 Task analysis: Breaking activity into sequential steps; number
of steps depends on child’s learning capacity
 Rewarding: Pleasant event following a given behavior; can be
material (food) or social (praise, attention); should be
immediate, consistent, appropriate and contingent
 Modeling: Showing how, or demonstrating, so that the child
imitate and learn
 Chaining: Breaking the task into small steps and teaching one
after another

68. BM Techniques for building new
skills:
 Back chaining: Teaching the last step first and
then going backwards
 Forward chaining: Teaching the first step first
 Prompting: Assisting the child verbally or
physically (hand over hand, gesturing,
pointing) and gradually fading the assistance

69. BM techniques to eliminate unwanted
behaviors
 Disregarding: Ignoring the behavior (as if it is not occurring at
all) but continuing the attention to child
 Ignoring: Ignoring both the child and behavior
 Limit-setting: Clearly communicating what is acceptable and
unacceptable behaviors to child and enforcing these
 Blocking: Preventing the behavior from being completed
(example aggression).
 Time-out (from positive reinforcement): Removal of
attention and reinforcement contingent upon occurrence of a
specified maladaptive / undesirable behavior

70. BM techniques to eliminate unwanted
behaviors
 Differential reinforcement of other behavior: Noticing and
rewarding the child while he or she shows desirable behavior
or when undesirable behavior is absent (catching the child
being good and praising)
 Over-correction: Child has to not only restore but do
something more to set right whatever damage or disturbance
that has occurred as a result of undesirable behavior

71.  I like living. I have sometimes been wildly,
despairingly, acutely miserable, racked with sorrow,
but through it all I still know quite certainly that just
to be alive is a grand thing.
-- Agatha Christie

72. Thank you

73. MCQ
Which is the most common inherited cause
of learning disability?
 Downs Syndrome
 Phenylketonuria
 Fragile X Syndrome
 Patau Syndrome

74. MCQ
In which of the following disorders ‘Gaze
Aversion’ is present other than Fragile X
Syndrome?
 ADHD
 Cry Du Chat Syndrome
 Autism
 Downs Syndrome

75. MCQ
Which of the following is not a X-linked
inherited syndrome?
 Retts Syndrome
 Lesch-Nyhan Syndrome
 Niemann-Pick Disease
 Fabry’s Disease

76. MCQ
Which of the following disorders or
behaviors are more common in patients with
Mild Intellectual Disability?
 Autistic behaviors
 Self injurious behaviors
 Disruptive and Conduct disorder Behaviors
 Repetitive stereotypal behaviors