Behavioral development, DQ, IQ

G SRINATH

Introduction Maturation of different biological functions-

milestones Anticipated at a particular age Due consideration given to environmental and

social factors

Gross MotorAge 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

Fine MotorAge in months

Activity

4 Bidextrous reach

6Unidextrous reach and transfer, biscuit to mouth (Mouthing)

9 Immature pincer

12 Mature pincer, feeds from cup with spilling

15Imitates 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

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

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

Developmental Quotient Up to 6 yrs. DA/CA *100

<20- Profound DD 20-34- Severe DD 35-49- Moderate DD 50-69- Mild DD 70-79- Borderline dev. 80-84- Below average

dev. 85-114- Average dev. >115- Above average

dev.

Screening tools: Denver II Bharatraj DST Phataks’ Baroda DST Trivandrum DST

Formal Tests: Gessell development

schedules Nancy Bayley scale of

development Social / Adaptive scales:

Vineland social maturity scale

DQ

Intelligence Quotient >6 yrs. MA/CA *100 Cut off- 70

Screening tests: Peabody Picture

vocabulary test III Draw a person task Kaufman brief

intelligence test Formal tests:

Wechsler intelligence test

Stanford Binet test Binet Kamat test

MENTAL RETARDATION/ INTELLECTUAL DISABILITY

G SRINATH

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

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

DSM IV TR (2000) Sub-average intellectual functioning IQ<= 70 Deficits in Adaptive behavior (Conceptual, Social

and Practical skills) Onset before 18 years

Mild: 50-55 to 70 Moderate: 35-40 to 50-55 Severe: 20-25 to 35-40 Profound: <20-25 Undiagnosed

ICD 10 Arrested or incomplete development of the

mind Impairment of skills- cognitive, language,

motor and social abilities Development period No flexibility as in DSM

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

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

ClassificationDegree of MR with IQ Adult attainment

Mild (50-69)85%

Literacy ++ (6th grade)- educableSelf-help skills ++Good speech ++Semi-skilled work +

Moderate (35-49)10%

Literacy + (2nd-3rd grade)Self-help skills ++-TrainableDomestic speech +Unskilled work with or without supervision +

Severe (20-34)3-4%

Assisted self-help skills +Minimum speech +Assisted household chores +

Profound (Below 20)1-2%

Speech: Utterances of words +/-Self-help skills +/-Sensory- motor impairments+

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

Maternal Toxins FAS, FHS

Others: Toxemia, IUGR, Radiation, Trauma

Familial MR

Prenatal causes

Perinatal causes Infections:

Meningitis, herpes Labor complications

Trauma, Asphyxia Others

Hypoglycemia, Hyperbilirubinemia, Seizures

Postnatal Causes Infections:

Meningitis, herpes Toxins:

Lead poisoning Others:

CVA, Tumors, Trauma

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

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

Psychological factors Full range of personality Low self image Outer-directedness, learned helplessness

Environmental factors: Social rejection and stigma, peer attitudes, abuse

potential Medical:

Seizures, sensory, motor impairment, medication side effects

Psychiatric illness in ID

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

Behavioral phenotypes: Examples

Behavior Syndrome

Hyperphagia, obsessions & compulsions, skin-picking

Prader-Will syndrome

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

Externalizing disorders: ADHD Oppositional defiant disorder Conduct disorder Mixed presentations

Internalizing disorders: Anxiety disorder Phobias, generalized, panic Separation anxiety disorder Social anxiety disorder Depression Obsessive compulsive disorder

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

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

Mental retardation and Affective disorders

Affective disorders in all forms do occur Classical criteria may not be elicited, instead

behavioral equivalents are commonly seen 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

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, talking in English, stubbornness, singing & dancing, and collecting rubbish

Depression: Clinging to mother, weeping, being dull, talking less than usual, sleep and app disturbances, withdrawn, aches & pains

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

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

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

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

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

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

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 microcephaly

Severe congenital microcephaly with 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

Course and Prognosis

MANAGEMENTBIO-PSYCHO-SOCIAL MODAL

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

Management principles (Cont) Early detection &

intervention Parent counseling &

training, Parent management training

Pharmacological Rx Behavior

modification

Developmental Assessment including IQ

Skills training (e.g. Social, communication)

Age appropriate concepts development

Individual counseling Normalization Habilitation

Management

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

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

Nerve Biopsies: MCL Visual evaluation: Cataract, Optic atrophy, cortical

blindness, refractive error Immunologic tests (Ig M antibodies): TORCH infections

Investigations

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

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

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

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

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

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

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

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

BM Techniques for building new skills

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

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

BM techniques to eliminate unwanted behaviors

References Psychiatry 3rd edition- Tasman CTP- 9th edition- Kaplan and Saddock Textbook of Pediatrics- O P Ghai- 7th edition

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