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Pervasive Developmental Disorders. Nursing 864 September 24, 2009. Autism Spectrum Disorders Autism Asperger’s Syndrome PDD, NOS Rett’s disorder Childhood Disintegrative Disorder. Autism Spectrum Disorders. Prevalence Approximately 1/150 children 4.3 : 1 ratio males to females

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pervasive developmental disorders

Pervasive Developmental Disorders

Nursing 864

September 24, 2009

slide2
Autism Spectrum Disorders
    • Autism
    • Asperger’s Syndrome
    • PDD, NOS
  • Rett’s disorder
  • Childhood Disintegrative Disorder
autism spectrum disorders
Autism Spectrum Disorders
  • Prevalence
    • Approximately 1/150 children
    • 4.3 : 1 ratio males to females
    • Increase in prevalence
      • Causes
        • Better assessment and diagnostic tools
        • Improved recognition by health care providers
        • Increased public awareness
etiology
Etiology
  • Genetic
    • Multiple genes involved
    • Rate of occurrence in siblings 2-8%
    • Monozygotic twins – 60%
  • Syndromes and Related Health Problems
    • Occurs in less than 10%
    • Fragile X
    • Epilepsy
    • Tuberous sclerosis
    • Fetal alcohol syndrome
    • Mental retardation occurs in approximately 70% of children
    • Increased rate of perinatal complications in the mother
autism diagnosis dsm iv tr criteria
AutismDiagnosis – DSM- IV-TR criteria
  • Qualitative impairment in social interaction (at least 2)
    • Impaired nonverbal behaviors
    • Failure to develop peer relationships as same age level
    • Lack of seeking to enjoy interests or achievement
  • Qualitative impairment in communication (at least one)
    • Delay or lack of spoken language
    • Impaired ability to initiate or sustain conversation
    • Stereotyped and repetitive use of language
    • Lack of varied or spontaneous play
  • Restricted repetitive and stereotyped patterns of behavior, interests and activities (at least one)
    • Preoccupied with one or more stereotyped or restricted interest
    • Inflexible to nonfunctional routines or rituals
    • Stereotyped or repetitive movements
impaired social interaction
Impaired Social Interaction
  • Low rates or no initiation of social interaction
  • Little interest in other children
  • Trouble sustaining social interactions
  • Little shared interest
  • No joint attention
  • Does not imitate
  • Does not enjoy social games
  • No social smile
  • Little shared interest
  • Poor eye contact and rarely looks for reaction
communication deficits
Communication Deficits
  • Delay in language development – principal criteria for diagnosis
  • Difficulty putting meaningful sentences together
  • Nonverbal communication impaired
    • Inappropriate gestures
  • No response to name called (seems deaf)
  • Difficulty perceiving themes or intent
  • Does not point to request (proto-imperative)
  • Does not point to interest (proto-declarative)
  • Echolalia
  • Confused pronouns
  • Very literal and concrete
slide8
Restricted Range of Interests/Stereotyped
    • Preoccupation with topics or intense interest
    • Preoccupation with sensory experiences
    • Repetitive movements
    • Manipulate toys in ritualistic manner
    • Monotonous play
    • Spin, bang, line up toys
    • Rocking motions
    • Spinning body
    • Flap hands
    • Taste or smell unusual objects
    • Rigid with rules and resistant to transitions
asperger s syndrome
Asperger’s syndrome
  • Asperger’s syndrome
    • Qualitative impairment in social interaction (at least two)
    • Restricted repetitive and stereotyped pattern of behavior, interests and activities (at least one)
    • No clinically significant language delay
    • No clinically significant delay in cognitive development, self-help skills or adaptive behavior (other than social interaction)
pdd nos
PDD, NOS
  • Severe impairment in the development of reciprocal social interaction
    • Impaired verbal or nonverbal communication skills
    • Presence of stereotyped behavior, interests, and activities
    • Criteria are not met for other PDD
      • Late Onset
      • Atypical symptomatology
      • Subthreshold symptomatology
childhood disintegrative disorder
Childhood Disintegrative Disorder
  • Rare disorder
    • Occurs in less than 5/10,000
  • Occurs after at least two years of normal development
  • Generally is diagnosed around 4-5 years of age.
  • Occurs more frequently in males
  • Along with regression in social skills and communication, there is regression in motor skills
  • Etiology
    • Predisposition to genetic and environmental influences
  • Prognosis guarded
rett s syndrome
Rett’s Syndrome
  • Almost exclusively in females
  • Typically neurogenerative arrest
  • Etiology - Gene MECP2 located on the X chromosomes
  • Early clinical features
    • Deceleration of head growth
    • Period of developmental stagnation is followed by a period of regression
    • Loss of purposeful hand skills and oral language
    • Development of hand stereotypies and gait dyspraxia
  • Prognosis – 70% 35 year survival rate
theory of mind
Theory of Mind
  • The ability to understand the thoughts and intentions of others (mental states)
  • Perspective taking of others
  • It can determine how an individual acts and react
  • Lack of ability or reduced ability in Asperger’s and Autistic disorder
sally anne test theory of mind wimmer and perner 1983
Sally-Anne test (Theory of Mind) (Wimmer and Perner, 1983)
  • In the presence of the child, the experimenter uses two dolls, "Sally" and "Anne". Sally has a basket; Anne has a box.
  • The experimenters show a skit:
    • Sally puts a marble in her basket and then leaves the scene.
    • While Sally is away and cannot watch, Anne takes the marble out of Sally's basket and puts it into her box.
    • Sally then returns.
  • The children are asked where they think she will look for her marble.
  • Children are said to "pass" the test if they understand that Sally will most likely look inside her basket before realizing that her marble isn't there.
pathophysiology
Pathophysiology
  • Neuroanatomical Factors
    • Enlargement of gray and white matter cerebral volumes
      • Increased rate of head circumference emerges at about 12 months of age
      • Increased volumes in the temporal, parietal and occipital region
      • No differences in size in frontal lobe or cerebellum
    • Possible mechanisms
      • Increased neurogenesis
      • Decreased neuronal death
      • Increased production of nonneuronal brain tissue
pathophysiology1
Pathophysiology
  • Neurotransmitters
    • Increased brain-derived neurotrophic factor and other neurotrophins
    • Age –related serotonin synthesis capacity
      • These may contribute to abnormal brain growth and organization
screening and diagnosis
Screening and Diagnosis
  • Group of symptoms
    • Behavioral
    • No medical tests
    • Screening and diagnosis involved clinical judgment
    • Diagnosis requires presence of severe and pervasive impairment across domains
    • Not every socially awkward or eccentric child has ASD, but never wait and see
  • Targeted developmental screening – 9,18 & 30 months
  • Autism specific screening – 18 and 24 months
screening tools
Screening Tools
  • Level 1
    • Modified Checklist for Autism in Toddler (M-CHAT)
      • Screen as young as 18 months
      • Critical items
        • Peer interest
        • Pointing
        • Joint attention
        • Shared interest
        • Imitation
        • Responds to Name
screening tools1
Screening Tools
  • Level 2
    • Child Autism Rating Scale (CARS)
    • Gilliam Autism Rating Scale (GARS)
    • Gilliam Asperger’s Disorder Scale (GADS)
    • Social Communication Questionnaire (SCQ)
diagnostic tools
Diagnostic Tools
  • Level 3
    • Autism Diagnostic Observation Scale (ADOS)
    • Autism Diagnostic Interview – Revised (ADIR)
    • Preschool Language Scales (IV) – by SLP
    • Adaptive Ratings (i.e., Vineland)
    • Cognitive Testing
diagnostic evaluation
Diagnostic Evaluation
  • Multidisciplinary Team
    • Developmental Pediatrics, Psychology, Speech, Genetics, and Education
      • Medical/Developmental/Behavioral History
      • Structured Interview
      • Behavior Ratings Scales
      • Structured Direct Observation
      • Direct Interaction/Teaching
      • Functional Assessment
      • Standardized Testing (Speech, Genetics, Psychology)
other diagnostic tests
Other Diagnostic Tests
  • Used primarily for children with cognitive impairment
    • MRI – with MR
    • High-resolution chromosomes
      • Analysis of the number and structure of the chromosomes
    • Fragile X
    • DNA Microarray
      • Investigates the expression levels of thousands of genes simultaneously.
empirically supported treatments
Empirically Supported Treatments
  • Early Intensive Behavioral Intervention
    • Based on Applied Behavior Analysis
      • Systematic modifications of the environment based on principles of behavior identified through experimental analysis
    • Focuses on the purpose or the function of the behavior
    • Involves changing antecedents and consequences to change behavior
    • Uses principals of operant conditioning
slide24
Incidental Teaching
    • To help improve or elaborate language skills
    • Teaching occurs when child initiates communication
    • Must create communication temptations
    • Prompts help the child be successful
    • Involves labeling and describing that occurs in the adult-child interaction
slide25
Picture Exchange Communication System (PECS)
    • Augmentative communication
    • Picture exchange for teaching communication skills
    • Emphasizes teaching functional language
    • No evidence of children losing established speech
slide26
Discrete Trial Training
    • Precise teaching interactions that emphasize potent and frequent reinforcing consequences
    • Each skill is taught separately
    • Prompting helps insure responding and success
    • Emphasis on high rate of teaching interactions
slide27
Naturalistic Teaching Procedures
    • Teaching procedures that are embedded in their natural activities
    • Enhances the spontaneity and generalization of language, social and play skills
    • Demonstrated to be beneficial for children who are developmentally delayed or disadvantaged
guidelines for treatment
Guidelines for Treatment
  • Combination of ABA procedures
    • Best outcome between ages 2-5
    • Best outcome for 25 hours or more per week
    • Best outcome when functional communication is established by age 5
comorbid conditions
Comorbid Conditions
  • Behavioral
    • ADHD
    • Sleep disturbance
    • Disruptive behaviors
      • Temper tantrums
      • Aggression
      • Self-injury
    • Anxiety
      • Generalized, intense worries
      • Obsessions and compulsions
slide30
Neurologic
    • Seizures – 20-35%
    • Hypotonia
    • Gait Abnormalities
    • Microcephaly – associated with co-existing structural brain malformations
    • Macrocephaly
  • Orthopedic
    • Toe walking
slide31
Nutrition
    • Restricted food choices
    • Rituals
    • Poor motor skills
    • No evidence of dietary restrictions helpful in treatment (gluten or casein)
    • Pica
      • Monitor lead levels
medication management
Medication Management
  • Atypical Antipsychotics - Aggression
    • Risperdal – Only FDA approved medication for children with autism
    • Abilify
  • Stimulants- ADHD
  • Alpha-adrenergic antagonists –
    • Clonidine & Tenex – impulsivity and sleep
  • SSRI’s - anxiety
parent counseling
Parent Counseling
  • Safety
  • Nutrition
  • Advocacy in the School System – IEP
  • Bullying
  • Parenting Stress
  • Siblings
resources
Resources
  • Autism Action Partnership
    • www.autismaction.org
  • PTI Nebraska
    • www.pti-nebraska.org
  • First Signs
    • www.firstsigns.org
  • National Autism Association
    • http://www.nationalautismassociation.org/
  • Munroe-Meyer Institute
    • Center for Autism Spectrum Disorders
    • 559-2441