Childhood Disorders and Management-Presentation.ppt

Intellectual disability and it's management is a very serious subject that the people working in mental health field and also public should know.

1. CHILDHOOD DISORDERS AND ITS
MANAGEMENT
Binny Joseph
(Consultant Clinical Psychologist)

2. What’s in a Name?
Different Names….
Say few?

3. Learning Objectives
Participants will be able to:
1)Define 4 levels of severity of mental retardation.
2)Identify the primary comorbid Axis I disorders.
3)Describe 6 risk factors for mental retardation.
4)Identify the 3 most common causes of mental
retardation.
5)Define behavioral phenotypes for 5 “common”
mental retardation syndromes.

4. Definition
Deficits in IQ and adaptive functioning
IQ of 70 or below
–Measured by standard scales
Wechsler, Stanford-Binet, Kaufman
Impairments in Adaptive Functioning
–Effective coping with common life demands
–Ability to meet standards of independence
–Measured by standard scales
Vineland, AAMR Adaptive Behavior Scale

5. Degrees of Severity
Mild Mental Retardation
–IQ: 50-55 to approximately 70
Moderate Mental Retardation
–IQ: 35-40 to 50-55
Severe Mental Retardation
–IQ: 20-25 to 35-40
Profound Mental Retardation
–IQ: Less than 20-25

6. AAIDD Proposed Classification
Based upon the intensity of supports
needed, as opposed to IQ (the traditional
system):
–Intermittent Support
–Limited Support
–Extensive Support
–Pervasive Support

8. Mild Mental Retardation
Previously referred to as “educable”
Largest segment of those with MR (85%)
Typically develop social/communication skills
during preschool years, minimal impairment in
sensorimotor areas, often indistinguishable from
“typicals”until later age
By late teens acquire skills up to approximately
the 6
th
grade level

9. Moderate Mental Retardation
Previously referred to as “trainable”
About 10% of those with MR
Most acquire communication skills during early
childhood years
Generally benefit from social/vocational training and with
moderate supervision can attend to personal care
Difficulties recognizing social conventions which
interferes with peer relations in adolescence
Unlikely to progress beyond the 2
nd
grade academically
Often adapt well to life in the community in supervised
settings (performing unskilled or semiskilled work)

10. Severe Mental Retardation
3 –4% of those with MR
Acquire little or no communicative speech in
childhood; may learn to talk by school age and be
trained in elementary self-care skills
Can master sight reading “survival”words
Able to perform simple tasks as adults in closely
supervised settings
Most adapt well to life in the community, living in
group homes or with families

11. Profound Mental Retardation
1 –2% of those with MR
Most have an identifiable neurological
condition that accounts for their MR
Considerable impairments in sensorimotor
functioning
Optimal development may occur in a highly
structured environment with constant aid

12. Stage Child’s Progress Normal Development Delayed Development: No.
Achieved
1. Responds to name / voice 1-3 months 4th month
2. Smiles at others 1-4 months 6th month
3. Holds head steady 2-6 months 6th month
4. Sits without support 5-10 months 12th month
5. Stands without support 9-14 months 18th month

13. Stage Child’s Progress Normal Development Delayed Development:
6. Walks well 10-20 months 20th month
7. Talks in 2-3 word sentences 16-30 months 3rd year
8. Eats/drinks by self 2-3 years 4th year
9. Tells his name 2-3 years 4th year
10. Has toilet control 3-4 years 4th year
11. Avoids simple hazards 3-4 years 4th year
Other Factors
12. Has fits Yes/ No
13. Has physical disability–what? Yes /No

14. Prevalence
1% (1 –3% in developed countries)
The prevalence of MR due to biological factors is
similar among children of all SES; however,
certain etiological factors are linked to lower SES
(e.g., lead poisoning & premature birth)
More common among males (1.5:1)
In cases without a specifically identified biological
cause, the MR is usually milder; and individuals
from lower SES are over-represented

15. Psychiatric Features
No specific personality type
Lack of communication skills may predispose to
disruptive/aggressive behaviors
Prevalence of comorbid Axis I disorders is 3-4
times that of the general population
The nature of Axis I disorders does not appear to be
different between “typicals”and those w/MR
Patients with MR and comorbid Axis I disorders
respond to medications much the same as those
without MR

16. Predisposing Factors
No clear etiology can be found in about 75% of
those with Mild MR and 30 –40% of those with
severe impairment
Specific etiologies are most often found in those
with Severe and Profound MR
No familial (ancestral) pattern (although certain
illnesses resulting in MR may be heritable)

17. Predisposing Factors (2)
Heredity (5% of cases)
–Autosomal recessive inborn errors of metabolism (e.g., Tay-Sachs,
PKU)
–Single-gene abnormalities with Mendelian inheritance and variable
expression (e.g., tuberous sclerosis)
–Chromosomal aberrations (e.g., Fragile X)
Early Alterations of Embryonic Development (30% of
cases)
–Chromosomal changes (e.g., Downs)
–Prenatal damage due to toxins (e.g., maternal EtOH consumption, -
EtOH(stands for ethyl alcohol, or ethanol). This clear substance is
found in alcoholic drinks such as beer, wine and liquor. infections)

18. Predisposing Factors (3)
Environmental Influences (15-20% of cases)
–Deprivation of nurturance, social/linguistic and other
stimulation
Pregnancy & Perinatal Problems (10% of cases)
–Fetal malnutrition, prematurity, hypoxia, viral and
other infections, head-trauma, infections
General Medical Conditions Acquired in Infancy
or Childhood (5% of cases)
–Infections, trauma, poisoning (e.g., lead)

19. Disability
Low birth weight is the strongest predictor of
disability
Male children and those born to black women and
older women in the USA are at increased risk for ID
Lower level of maternal education is also
independently associated with degree of disability

20. Screening of Intellectual Disability
Screening
Screeningisaprocedureforaninitialidentificationofpersons
withmentalretardationandforafollowupwithassessment.
(i) Pre-natal Procedures
(ii) Neonatal and Post-natal Screening and Diagnostic Procedure
Blood and urine examinations are conducted in the neonatal period in
all suspected cases and with a previous history of mentalretardation
in the family.

21. Most Commonly Associated
Axis I Disorders
ADHD
Mood Disorders
Pervasive Developmental Disorders
Stereotypic Movement Disorders

22. Attention Deficit Hyper Activity
Disorder (ADHD)
Inattention Symptoms (6 of 9):
 Careless mistakes
 Attention difficulty
 Listening problem
 Loses things
 Fails to finish things
 Organizational skills lacking
 Reluctance in tasks requiring sustained mental effort
 Forgetful in Routine activities
 Easily Distracted

23. Attention Deficit Hyper Activity
Disorder (ADHD)
Hyperactive-Impulsive Sx(6 of 9):
Runs about or is restless
Unable to wait his/her turn
Not able to play quietly
On the go
Fidgets with hands or feet
Blurts out answers
Staying seated is difficult
Talks excessively
Tends to interrupt

24. ADHD Co-morbid Conditions
Other Disruptive Behavior Disorders such as:
-Oppositional Defiant Disorder:
Pervasive pattern of negativistic, defiant,
disobedient, and hostile behaviors toward authority
figures
-Conduct Disorder:
Repetitive pattern of violating the basic rights of
others and/or societal laws

25. ADHD Co-morbid Conditions
-Mood disorders, such as depression or bipolar
disorder; check family history; co-morbid teens have
higher rates of suicide
-Anxiety disorders–25% or more
-Learning disorders–up to 60%, especially
Reading Disorder (Writing, Reading, Mathematics)

26. Management of ADHD
Psycho-educational Interventions, such as cognitive-
behavioral therapy, to improve impulse control, and
parent management training
Special Educators
Classroom strategies and modifications
Parent Education and Empowerment
Pharmacotherapy

27. Etiology
At least 500 causes now known
Many MR syndromes have been related to the
X-chromosome
Most common cause of MR:
(1)Down’s Syndrome (most common genetic cause)
(2)Fragile X Syndrome (accounts for 40% of all X-
linked syndromes; most common inherited cause)
(3)Fetal EtOH Syndrome (most common attributable
cause)
together these 3 account for 30% of all identified
cases of MR

28. AUTISM
1943-Leo Kanner
“Extreme aloneness from the beginning of life and
anxiously obsessive desire for the preservation of
sameness.”

29. Autism-Symptoms
Severe abnormality of reciprocal social relatedness
Severe abnormality of communication development
Restricted, repetitive behavior, patterns of behavior,
interests, imagination
Early onset (before 3-5 years)

30. Autism-Other observed behaviors
Lack of awareness of feelings of others
Bizarre speech patterns
Lack of spontaneous and make-believe play
Preoccupation with parts of objects
Repetitive motor movements
Marked distress over changes

31. Screening Model for Infantile Autism
Is child’s eye-to-eye contact normal?
Is he/she comforted by proximity/body contact?
Does he/she often smile or laugh unexpectedly?
Does he/she prefer to be left alone?

32. Systematic Feature Examination
Hand stereotypies (strange
looking or posturing)
Stiff gaze, avoidance of
Little reaction to strong,
unexpected noise
Passive, obvious lack of interest
Resistance to change, Insist on
sameness
Strong attachments to objects;
Spins objects
Difficulty in mixing with others
Throw Temper Tantrums
Tend not to want to cuddle or be
cuddled
Over-sensitivity or under-
sensitivity to pain
No fears of danger

33. Autism-Sensory Processing
Painfully sensitive to certain sounds, textures, tastes, and
smells.
Either too sensitive or less sensitive than normal. Some
autistic have difficulty interpreting sensory information.
Like normal these experiences are not hallucinations but
based on real experiences.
Some avoid being touched, a gentle touch for most, will
hurt or shock autistics.
Some are insensitive to pain, and fail to notice injuries.

34. Emotions
Take major emergencies in stride but become upset
over minor disruption.
Unemotional, but can be very emotional when
things are important to them.
More candid and expressive with their emotions
than normal people.
Small amount will have difficulty regulating their
emotions. Individual will have verbal outburst,
usually in strange or overwhelming environment.

35. Communications
Problem with semantic-pragmatic component, take a statement or question in a
literal way.
Ex.) "I'd like coffee with my cereal“
Repeating things that have been heard (echolalia)
Inability to understand body language, tone of voice
Some autistics are mute
Difficult in sustaining a conversation. No normal "give and take" in a
conversation
Autistics tend to go on with their favorite subjects and do not give the
other person a chance to talk.
People with autism might stand too close to the other person.

36. Attention
Trouble handling multiple stimuli of
attention.
Very narrow focused attention, can not keep
up with more than one thing at a time.
Shifting attention is a slow process, usually
involves pauses or moments of delay.

37. Autism-Management
Improving Communication Skills
–Music Therapy
Speech Development
–Art Therapy
Non-verbal, Symbolic Expression
–Animal Therapy
Physical and Emotional Benefits
Pharmacotherapy (Comorbid ADHD

38. Autism-Management
Need of Assessment to know the severity
Reinforcement
Behavioural Modification Techniques
Shaping
Social Adaptive Skill Training

39. Down’s Syndrome
MostcommonchromosomalabnormalityleadingtoMR(1.2/1000births)
(Downsyndrome(DSorDNS),alsoknownastrisomy21,isa
geneticdisordercausedbythepresenceofallorpartofathirdcopyof
chromosome21.Itisusuallyassociatedwithphysicalgrowthdelays,mildto
moderateintellectualdisability,andcharacteristicfacialfeatures)
Nondysjunctionofchromosome21
Relativestrengths:
–Visual(vs.auditoryprocessing)
–Socialfunctioning
Relativeweaknesses:
–Languageexpressionandpronunciation
Generallyviewedtosufferlessseverepsychopathologythanother
developmentallydelayedgroups
After40yearsofage,affectedindividualsnearlyalwaysdemonstrate
postmortemneuronaldefectsindistinguishablefromAlzheimer’sDisease

40. Behavior & Psychiatric Illness in Downs
Recent population based survey of social and
healthcare records found:
–Females had better cognitive abilities and speech
production compared with males
–Males had more behavioral troubles
–ADHD symptoms were often seen in childhood across
gender
–Depression was diagnosed more often in adults with
mild/moderate intellectual impairment
–Autistic behavior was most common in those with
profound intellectual disability
–Elderly often showed a decline in adaptive behavior
consistent with Alzheimer’s
•Maatta et al, 2006

41. Down’s Syndrome

42. Fragile X Syndrome
FMR-1 gene (>200 trinucleotide CGG repeats, Xq27.3)
An example of a “dynamic mutation”where more mutations
occur with successive generations
General problems: MR, mild CT dysplasia, & macro-
orchidism
Only 50% of females with the full mutation demonstrate IQs
in the borderline/mild MR range (vs. 100% of males)
Increases the risk for ADHD, autism (20-60%) & social
phobia
Increasing deficits in adaptive and cognitive functioning with
age
Relative strengths:
–Verbal long-term memory
Relative weaknesses:
–ST memory, VM integration, sequential processing, math & attn

43. Fragile X Syndrome

44. Fragile X Syndrome

45. Fetal EtOH Syndrome
Incidence > 1:1000
Irritable as infants, hyperactive as children (ADHD)
Teratogen amount: 2 drinks/day (smaller birth size), 4-6 drinks/day
(subtle clinical features), 8-10 drinks/day (full syndrome)
General problems: prenatal onset of growth deficiency, microcephaly,
short palpebral fissures
Syndrome can include:
–Facial deformities (ptosis of eyelid, microphthalmia, cleft lip [+/-palate],
micrognathia, flattened nasal bridge and filtrum, & protruding ears)
–CNS deformities (meningomyelocele, hydrocephalus)
–Neck deformities (mild webbing, cervical vertebral & rib abmormalities)
–Cardiac deformities (tetralogy of Fallot, coarctation of aorta)
–Other abnormalities (hypoplastic labia majora, strawberry hemangiomata)

46. Fetal EtOH Syndrome

47. Prader-Willi Syndrome
Deletion in chromosome 15 (15q11-13); freq 1:15000
60-80% w/microscopic deletion on paternal 15; remaining
PWS have 2 copies of maternal chromosome w/no
paternal chromosome (“uniparental disomy”)
Infantile hypotonia, (low muscle tone)hyperphagia/food
seeking, morbid obesity, small hands/feet, mild to
moderate MR
Relative stability in adaptive functioning during
adolescence and early adulthood
Relative strengths:
–Expressive vocabulary, LT memory, visual/spatial integration
and visual memory (unusual interest in jigsaw puzzles)
Relative weaknesses:
–Temper tantrums, emotional lability, mood symptoms (dx?),
anxiety, skin picking, OCD symptoms (>50% OCD)

48. Prader-Willi Syndrome

49. Prader-Willi Syndrome

50. Angelman Syndrome
SevereMR,seizures,ataxia(lackofvoluntarycoordination
ofmusclemovementsthatcanincludegaitabnormality,
speechchanges)
&jerkyarmmovements(puppet-likegait),absenceof
speech,andboutsoflaughter(aka“happypuppet”)
Deletioninchromosome15(15q11-13)
IncontrasttoPWS,allidentifiedcasesofdeletiontracedto
maternalchromosome15
–Illustrating“genomicimprinting,”(thefactthattheparentof
originofthedeletionatthesamelocusimpactsthephenotype;that
is,deletionofpaternal15q11-13resultsinPrader-Willibut
deletionofmaternal15q11-13resultsinAngelman.)

51. Angelman Syndrome

52. Williams Syndrome
MR, supravalvular aortic stenosis, “elfin-like”
facies, infantile hypercalcemia, and growth
deficiency
Deletion of elastin gene (7q11.23)
Relative strengths:
–Remarkable facility for recognizing facial features
–Loquacious, pseudo-mature “cocktail party speech”
Relative weaknesses:
–Increased risk for ADHD, Anxiety D/O

53. Williams Syndrome

54. Psychotropic Medications
No medications identified to treat MR nor to
address specific symptoms
No medications are FDA approved
Rates of medication use vary from 12 –40% in
institutions vs. 19 –29% in community settings
amongst current studies (excl anticonvulsants)
•Singh et al, 1997
More recent review found that 22.8% of MR
persons in group homes in the Netherlands were
prescribed psychotropic medications
•Stolker et al, 2002

55. Stimulants
ADHD is the most widely diagnosed psychiatric
disorder amongst children and adolescents with MR
Prevalence rates estimated to be 8.7 –16% (Emerson,
2003; Stromme & Diseth, 2000)
At least 20 RDBPC trials published involving MTP
with persons with MR; positive results range from 45 –
66%; lower than the rates found with non-MR
population
Positive predictors of response include IQ>50 and
higher baseline scores on parent/teacher ratings of
inattention and activity level
Limited data on other treatments for ADHD symptoms
•Handen et al, 2006

56. Conduct Disorder
Disorder of behaviourcharacterised by repetitive and persistent
pattern of dissocial, aggressive or defiant conduct.
-Affects 12% of boys and 7% of girls
-Most frequent reason for psychiatric hospital admissions for children
and adolescents
Oppositional Defiant Disorder in preschool years developing into a
serious conduct disorder by adolescence
This group has a 2-3 fold likelihood of becoming juvenile offenders

57. Conduct Disorder-Psychosocial
Correlates
Harsh punishment
Institutional living
Inconsistent parental
figures (living with
different relatives for
years)
Poor parental
monitoring in early
childhood
Parental conflict
Maternal depression
Paternal alcoholism

58. Conduct Disorder-Psychosocial
Correlates
Conduct Disorder develops as a result of biological
risk and childhood experiences, so there are
opportunities for early intervention
Treatment includes family therapy, behavior
management training, social skills group, and
teaching problem-solving skills

59. Generalized Anxiety Disorder
Mean age of onset between 10-13 years of age
Prevalence: Latency age 3%; adolescent 10%
Worry themes: Academics, natural disasters, social
life, physical assault, family, parents, friends…

60. Separation Anxiety Disorder
The most common anxiety disorder of childhood
Most commonly occurs at age 7 or 8 years, but may
occur in adolescence
Psychosocial theory is that angry feelings toward
parents are displaced, so the environment is
perceived as threatening
Developmentally inappropriate, excessive worry
concerning separation from those to whom the
youngster is attached,

61. Depression
1.Depressed mood (feels sad or empty) by self-report or observation
2.Diminished interest or pleasure in most activities
3.Weight gain or weight loss; in children, failure to make expected
weight gain
4.Insomnia or hyper-somnianearly every day
5.Psychomotor agitation or retardation nearly every day, observable
by others
6.Fatigue or loss of energy
7.Feelings of worthlessness or guilt (which may be delusional)
8.Inability to concentrate; indecisiveness
9.Recurrent thoughts of death

62. Management
Screening
District Early Intervention Center (DEIC)
Do the Registration
Get help of:
Clinical Psychologist
Psychiatrist
Pediatrist
Speech Therapist
Physio-therapist
Special Educationist
Optometrist-(Eye)

63. Management
Need of early assessment and intervention
Parental training
Counselling for parents and other family members
Group sharing
Need of Inclusive Education Centre

64. Management
Technology for Employment
Video-assisted training is being used for job training and job
skill development and to teach complex skills for
appropriate job behavior and social interaction.
Technology for Sports and Recreation
Toyscanbeadaptedwithswitchesandothertechnologiesto
facilitateplayforchildren.
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