Intellectual disability
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Intellectual disability

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Presented in Child Psychiatry, Niloufer Hospital in 2010.

Presented in Child Psychiatry, Niloufer Hospital in 2010.

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Intellectual disability Intellectual disability Presentation Transcript

  • 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 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
  • 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
  • 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
    • 1 st century- Avicenna- various levels of intelligence
    • 2 nd century- Talmud- “ Shoteh”- wanders alone, tears clothes, sleeps in cemetery
    • 1534- Fitz Herbert- earliest Intelligence test-
    • 17 th century- John Locke- differentiated MR from Mental Illness
    • 1800- Pinel- “Moral treatment of Mental Patients”
    • Seguin- First need school in Paris, Seguin Form Board, 1 st President of AAMR
  • History
    • 1905- Binet and Simon- First version of the intelligence test
    • Penrose- 1 st scientific study on ID
    • Gessell- Schedule for development
    • Change in terminology
      • Shoteh
      • Idiots, Imbeciles, Morons
      • Mental Retardation
      • Intellectual disability
  • Classification Degree of MR with IQ Adult attainment Mild (50-69) 85% Literacy ++ (6 th grade)- educable Self-help skills ++ Good speech ++ Semi-skilled work + Moderate (35-49) 10% Literacy + (2 nd -3 rd grade) Self-help skills ++-Trainable Domestic 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
    • MANAGEMENT
    • BIO-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 3 rd edition- Tasman
    • CTP- 9 th edition- Kaplan and Saddock
    • Textbook of Pediatrics- O P Ghai- 7 th edition
    • Thank you