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Mental Retardation




                                   Dr. Kalpana Malla
                                       MD Pediatrics
                           Manipal Teaching Hospital

Download more documents and slide shows on The Medical Post [ www.themedicalpost.net ]
Definition (AAMD)
• American Association on Mental Deficiency :
       Mental retardation refers to significantly
  sub average general intellectual
  functioning, existing concurrently with deficits
  in adaptive behavior and manifested during
  the developmental period
• Intellectual functioning is defined by
  Intelligent Qutient (I.Q)
 IQ = Mental Age/Chronological Age X100
Wechsler Adult Intelligence Scale
                (WAIS)
       Class                        IQ
•   Profound MR --------------------- <20
•   Severe MR ----------------------- 20-34
•   Moderate MR ------------------- 35-49
•   Mild MR -------------------------- 50-69
•   Boderline MR --------------------70-79
•   Low average---------------------80-89
•   Average --------------------------90-109
•   High average ------------------110-119
•   Superior -------------------------120-129
•   Genious ------------------------ > 130
Revised definition -1992

•   Does not consider IQ
•   Considers defects in adaptive skills- It is
    characterized by significantly subaverage
    intellectual functioning, existing concurrently with
    related limitations in two or more of the following
    applicable adaptive skill areas:
1.Communication
2. Self-Care- getting dressed, using the bathroom, and
    feeding oneself
3. Home Living
Revised definition -1992

4. Social Skills - peers, family members, spouses,
5. Community Use
6. Self-Direction
7. Health And Safety
8. Functional Academics
9. Leisure And Work
• Mental retardation manifests before age 18.
• Two areas of deficits - intellectual functioning and
  adaptive skills :



• Intellectual skills, the ability to solve problems
  related to academics; usually estimated by an IQ
  test
• Adaptive skills, skills needed to adapt to one’s
  living environments; usually estimated by an
  adaptive behavior survey
Alternative terms
•   Developmental delay
•    Developmental disability
•   Intellectual disability
•   Mental handicap
•   Learning difficulty
•   Idiots
•   Morons
•   Mental Defectives
•   Feeble-minded
•   Fools
•   Evolutionary Degenerates
Causes by time.

• Prenatal (12%) -occurs during fetal
                 development
• Perinatal (6%) -causes at birth
• Postnatal (4%) - occurring after birth, can
               be biological or psychosocial
• 78% unknown
• Prenatal :
A)Structural defects – hydrocephalus,spina
  bifida,craniosynostosis, microcephaly
B)Genetic conditions
    1. chromosomal disorders –down
         syndrome ,fragile X syndrome
         Klinfelters syndrome
Prenatal
2.Inborn errors of metabolism –
   a) Amino acid - Phenylketonuria, Maple syrup urine
                 Ds,Homocystinuria,Hartnup ds
  b) Carbohydrate metabolism –glactosemia,Fructose
             intolerance, Glycogen storage ds, MPS
  c) Lipid metabolism - lipidosis, Tay sachs Ds,
C. Endocrine – cretinism
Prenatal
D) Maternal – TORCH infection
          - Fetal alcohol syndrome,
            Teratogenic agents, irradiation,
            Toxemia of pregnancy
Causes by time
Perinatal
• Birth Asphyxia
• Birth trauma
• Intracranial hge
• Low birth weight
• Prematurity
• Torch
• Meningitis at birth
Causes by time
Postnatal-
• Infection -Meningitis, Encephalitis
• Toxic – kernicterus ,Lead intoxication
• Trauma - Head injuries ,ICHge ,Child abuse and
  neglect
• Endocrine –hypoglycemia ,hypocalcemia,
  dyselectrolytemia,hypothyroidism
• Gross PEM
Postnatal……..
• Behavioral injuries - Social and family,
  interactions
• Educational availability & supports that
  promote mental development of adaptive
  skills
• Social, behavioral, and educational often
  overlap these are sometimes referred to as
  cultural-familial mental retardation
Causes by severity

• Mild MR is more influenced by cultural and
  family environment – PHYSIOLOGICAL MR
• More severe MR is more likely to stem from
  genetic and other organic factors –
  PATHOLOGICAL MR
Syndromes associated MR
• Down syndrome -MR, slanted eyes, single
  palm crease, hypotonia, short stature
• Fragile X Syndrome – in males, thought to be
  most common hereditary cause of MR
• Tay-Sachs Disease - inherited metabolic
  disorder which leads MR, paralysis, dementia,
  or blindness
Syndromes associated MR

• Williams syndrome –mild to mod MR (social
  deficits in special
  skills, reading, math, writing), heart
  defects, elfin facial features
• Prader-Willi syndrome - MR varies, mostly
  mild MR, obesity
Signs

•   Have trouble speaking
•   Find it hard to remember things
•   Have trouble understanding social rules
•   Have trouble learn specific subject
•   Have trouble solving problems
•   Have trouble thinking logically
•   Disable to self care
•   Persistence of infantile behaviour
•   Unable to take higher education
Mild MR
• Learning disabilities ,hyperactivity, short
  attention span, distractibility, poor
  concentration, poor memory, impulsiveness,
  clumsy movements, disturbed sleep
• Emotional instability, low frustration tolerance
• Mild disability –
   May not be obvious in early childhood
   In School - have poor academic performance (
  learning disability)
  In adults - considered as "slow" rather than retarded
Moderate MR
• Academic skills – 2nd grade level
• Problem with social work
• Behaviorable age -8 yrs
obvious within 1st year of life
 - will face difficulty in school, at home, and in the
   community
 - Need special school, but they can still progress to
   become functioning members of society. As adults
   they may live with their parents, in a supportive
   group home
Severe MR
•   Little or no speech
•   Limited abilities to manage self care
•   Require high supervision
•   Behaviourable age – 3 yrs
Diagnosis
• According to the latest edition of the
  Diagnostic and statistical Manual of Mental
  Disorders there are three criterias
1. IQ below 70
2.Significant limitations in two or more areas of
  adaptive behavior
3. Evidence that the limitations became
  apparent in childhood
Assessment
• Assess intellectual and adaptive skills
• Professional administered IQ tests
           - Stanford-Binet – 2yrs - adult
           - Wechsler Intelligence Scale for
              Children (WISC-III) – 6- 17 yrs
            - Kaufman Assessment Battery for
              Children (K-ABC)
Prenatal Screening
• Amniocentesis (fluid)
• Chorionic villus sampling (CVS) (tissue)
• Sonography (visual)
• Maternal serum screening (MSS) (blood)
Investigations
•   No routine workup
•   Clinical judgement
•   No identifyable cause in most cases
•   Some – chromosomal, metabolic, encephalopathy
•   Investigations – TORCH screening
                  Karyotyping
                  thyroid function test
                   metabolic screening
                    CT/MRI
                    EEG
Treatment



• Require patience, good will, unlimited time
• Caring rather than curing
• Educating rather than medicating
Instruction Using Behavioral
                Principles

• Caregivers are trained to teach children
  positive behaviors and reduce
 negative behaviors effectively
 and humanely
Drug Therapies

• No specific drugs but some symptoms can be
  controlled
• Neuroleptic drugs to reduce aggressive and
  antisocial behavior (phenothiazines)
• antipsychotic drugs( risperidone)
• Antidepressant drugs can improve sleep, possibly
  help reduce self-injurious behavior, reduce
  depression
Mainstreaming

• Placing children with MR in regular classrooms
  to “normalize” their behavior and give them
  more opportunities
• Students with MR achieve high academic gains
  where they are more fully included in general
  classrooms
Institutionalization

• Reserved for the least capable children with
  the gravest disabilities
Milder MR is usually      More severe MR is
    treated with              treated by:
•   1. Behavioral           • 1. Behavior Therapy
    Instruction             • 2. Drugs to control
•   2. Early Intervention     aggression and self-
    Programs                  injurious behavior
•   3. Special Education    • 3. Either home care or
•   4. Mainstreaming          institutionalization
Treatable /Preventable causes of MR
•   Hypothyroidism
•   Severe PEM
•   Perinatal asphyxia
•   Preterm /LBW
•   Meningitis, encephalitis
Prevention
• Primary intervention before it
  occurs…(vaccines for rubella)
• Secondary intervention soon after detection
  (lead screening, PKY screening)
• Tertiary intervention to reduce long term
  effects (early education intervention)
ASSOCIATED PROBLEMS
•   Self injury       •   Eating problems
•   Pica              •   Poisoning
•   CP                •   Sexual abuse
•   Epilepsy          •   Learning disorders
•   Toilet probs      •   Behavior problems
•   Sleep disorders
Thank you
Download more documents and slide shows on The Medical Post
               [ www.themedicalpost.net ]

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Mental Retardation

  • 1. Mental Retardation Dr. Kalpana Malla MD Pediatrics Manipal Teaching Hospital Download more documents and slide shows on The Medical Post [ www.themedicalpost.net ]
  • 2. Definition (AAMD) • American Association on Mental Deficiency : Mental retardation refers to significantly sub average general intellectual functioning, existing concurrently with deficits in adaptive behavior and manifested during the developmental period
  • 3. • Intellectual functioning is defined by Intelligent Qutient (I.Q) IQ = Mental Age/Chronological Age X100
  • 4. Wechsler Adult Intelligence Scale (WAIS) Class IQ • Profound MR --------------------- <20 • Severe MR ----------------------- 20-34 • Moderate MR ------------------- 35-49 • Mild MR -------------------------- 50-69 • Boderline MR --------------------70-79 • Low average---------------------80-89 • Average --------------------------90-109 • High average ------------------110-119 • Superior -------------------------120-129 • Genious ------------------------ > 130
  • 5. Revised definition -1992 • Does not consider IQ • Considers defects in adaptive skills- It is characterized by significantly subaverage intellectual functioning, existing concurrently with related limitations in two or more of the following applicable adaptive skill areas: 1.Communication 2. Self-Care- getting dressed, using the bathroom, and feeding oneself 3. Home Living
  • 6. Revised definition -1992 4. Social Skills - peers, family members, spouses, 5. Community Use 6. Self-Direction 7. Health And Safety 8. Functional Academics 9. Leisure And Work • Mental retardation manifests before age 18.
  • 7. • Two areas of deficits - intellectual functioning and adaptive skills : • Intellectual skills, the ability to solve problems related to academics; usually estimated by an IQ test • Adaptive skills, skills needed to adapt to one’s living environments; usually estimated by an adaptive behavior survey
  • 8. Alternative terms • Developmental delay • Developmental disability • Intellectual disability • Mental handicap • Learning difficulty • Idiots • Morons • Mental Defectives • Feeble-minded • Fools • Evolutionary Degenerates
  • 9. Causes by time. • Prenatal (12%) -occurs during fetal development • Perinatal (6%) -causes at birth • Postnatal (4%) - occurring after birth, can be biological or psychosocial • 78% unknown
  • 10. • Prenatal : A)Structural defects – hydrocephalus,spina bifida,craniosynostosis, microcephaly B)Genetic conditions 1. chromosomal disorders –down syndrome ,fragile X syndrome Klinfelters syndrome
  • 11. Prenatal 2.Inborn errors of metabolism – a) Amino acid - Phenylketonuria, Maple syrup urine Ds,Homocystinuria,Hartnup ds b) Carbohydrate metabolism –glactosemia,Fructose intolerance, Glycogen storage ds, MPS c) Lipid metabolism - lipidosis, Tay sachs Ds, C. Endocrine – cretinism
  • 12. Prenatal D) Maternal – TORCH infection - Fetal alcohol syndrome, Teratogenic agents, irradiation, Toxemia of pregnancy
  • 13. Causes by time Perinatal • Birth Asphyxia • Birth trauma • Intracranial hge • Low birth weight • Prematurity • Torch • Meningitis at birth
  • 14. Causes by time Postnatal- • Infection -Meningitis, Encephalitis • Toxic – kernicterus ,Lead intoxication • Trauma - Head injuries ,ICHge ,Child abuse and neglect • Endocrine –hypoglycemia ,hypocalcemia, dyselectrolytemia,hypothyroidism • Gross PEM
  • 15. Postnatal…….. • Behavioral injuries - Social and family, interactions • Educational availability & supports that promote mental development of adaptive skills • Social, behavioral, and educational often overlap these are sometimes referred to as cultural-familial mental retardation
  • 16. Causes by severity • Mild MR is more influenced by cultural and family environment – PHYSIOLOGICAL MR • More severe MR is more likely to stem from genetic and other organic factors – PATHOLOGICAL MR
  • 17. Syndromes associated MR • Down syndrome -MR, slanted eyes, single palm crease, hypotonia, short stature • Fragile X Syndrome – in males, thought to be most common hereditary cause of MR • Tay-Sachs Disease - inherited metabolic disorder which leads MR, paralysis, dementia, or blindness
  • 18. Syndromes associated MR • Williams syndrome –mild to mod MR (social deficits in special skills, reading, math, writing), heart defects, elfin facial features • Prader-Willi syndrome - MR varies, mostly mild MR, obesity
  • 19. Signs • Have trouble speaking • Find it hard to remember things • Have trouble understanding social rules • Have trouble learn specific subject • Have trouble solving problems • Have trouble thinking logically • Disable to self care • Persistence of infantile behaviour • Unable to take higher education
  • 20. Mild MR • Learning disabilities ,hyperactivity, short attention span, distractibility, poor concentration, poor memory, impulsiveness, clumsy movements, disturbed sleep • Emotional instability, low frustration tolerance
  • 21. • Mild disability – May not be obvious in early childhood In School - have poor academic performance ( learning disability) In adults - considered as "slow" rather than retarded
  • 22. Moderate MR • Academic skills – 2nd grade level • Problem with social work • Behaviorable age -8 yrs obvious within 1st year of life - will face difficulty in school, at home, and in the community - Need special school, but they can still progress to become functioning members of society. As adults they may live with their parents, in a supportive group home
  • 23. Severe MR • Little or no speech • Limited abilities to manage self care • Require high supervision • Behaviourable age – 3 yrs
  • 24. Diagnosis • According to the latest edition of the Diagnostic and statistical Manual of Mental Disorders there are three criterias 1. IQ below 70 2.Significant limitations in two or more areas of adaptive behavior 3. Evidence that the limitations became apparent in childhood
  • 25. Assessment • Assess intellectual and adaptive skills • Professional administered IQ tests - Stanford-Binet – 2yrs - adult - Wechsler Intelligence Scale for Children (WISC-III) – 6- 17 yrs - Kaufman Assessment Battery for Children (K-ABC)
  • 26. Prenatal Screening • Amniocentesis (fluid) • Chorionic villus sampling (CVS) (tissue) • Sonography (visual) • Maternal serum screening (MSS) (blood)
  • 27. Investigations • No routine workup • Clinical judgement • No identifyable cause in most cases • Some – chromosomal, metabolic, encephalopathy • Investigations – TORCH screening Karyotyping thyroid function test metabolic screening CT/MRI EEG
  • 28. Treatment • Require patience, good will, unlimited time • Caring rather than curing • Educating rather than medicating
  • 29. Instruction Using Behavioral Principles • Caregivers are trained to teach children positive behaviors and reduce negative behaviors effectively and humanely
  • 30. Drug Therapies • No specific drugs but some symptoms can be controlled • Neuroleptic drugs to reduce aggressive and antisocial behavior (phenothiazines) • antipsychotic drugs( risperidone) • Antidepressant drugs can improve sleep, possibly help reduce self-injurious behavior, reduce depression
  • 31. Mainstreaming • Placing children with MR in regular classrooms to “normalize” their behavior and give them more opportunities • Students with MR achieve high academic gains where they are more fully included in general classrooms
  • 32. Institutionalization • Reserved for the least capable children with the gravest disabilities
  • 33. Milder MR is usually More severe MR is treated with treated by: • 1. Behavioral • 1. Behavior Therapy Instruction • 2. Drugs to control • 2. Early Intervention aggression and self- Programs injurious behavior • 3. Special Education • 3. Either home care or • 4. Mainstreaming institutionalization
  • 34. Treatable /Preventable causes of MR • Hypothyroidism • Severe PEM • Perinatal asphyxia • Preterm /LBW • Meningitis, encephalitis
  • 35. Prevention • Primary intervention before it occurs…(vaccines for rubella) • Secondary intervention soon after detection (lead screening, PKY screening) • Tertiary intervention to reduce long term effects (early education intervention)
  • 36. ASSOCIATED PROBLEMS • Self injury • Eating problems • Pica • Poisoning • CP • Sexual abuse • Epilepsy • Learning disorders • Toilet probs • Behavior problems • Sleep disorders
  • 37. Thank you Download more documents and slide shows on The Medical Post [ www.themedicalpost.net ]