The PDD Behavior Inventory PDDBI

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The PDD Behavior Inventory (PDDBI)
Ira L. Cohen, Ph.D. Chairman, Psychology
Dept. NYS IBR/DD Cohen and Sudhalter
(2005) Psychological Assessment Resources, Inc.
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PDD Behavior Inventory (PDDBI)

• Cohen, I.L., Schmidt-Lackner, S., Romanczyk, R.,
  and Sudhalter, V. (2003). The PDD Behavior
  Inventory A rating scale for assessing response
  to intervention in children with PDD. Journal of
  Autism and Developmental Disorders, 33(1), 31-45.
• Cohen, I.L. (2003). Criterion-related validity of
  the PDD Behavior Inventory. Journal of Autism and
  Developmental Disorders, 33(1), 47-53.
• Cohen, I.L., and Sudhalter, V. (2005). The PDD
  Behavior Inventory. Lutz, Fl Psychological
  Assessment Resources, Inc.

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Goals of Workshop

• Understanding why the PDDBI was developed and
  its uses
• Learning about autism and the related PDDs
• Learning about administration and scoring of the
  PDDBI
• Learning about the reliability and validity of
  the PDDBI
• Learning about interpretation of PDDBI score
  profiles and score discrepancies and their
  implications for diagnosis and intervention

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Why was the PDDBI Developed?

• I had clinical and research questions that could
  not easily be answered with rating scales
  developed to assess autism
• Childrens Psychiatric Rating Scale
• Childhood Autism Rating Scale
• Autism Behavioral Checklist
• Behavioral Summarized Evaluation scale
• Global Impression-Type Scales (CGI)
• Gilliam Autism Rating Scale
• Autism Diagnostic Interview-Revised
• Autism Diagnostic Observation Schedule-Generic

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Clinical Questions

• When a child with autism shows challenging
  behaviors . . . .
• Is it because he or she has autism? (i.e., other
  children with autism show similar problems at the
  same level of intensity)
• Is something else going on? (i.e., childs
  behavior is beyond what we would expect or is
  restricted to certain settings)
• But theres a problem
• Assessment tools for autism are not standardized
  on children with autism
• Assessment tools for autism are not standardized
  on different types of informants

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Research/Clinical Questions

• When a child is treated with medication and
  repetitive behaviors decrease. . . . .
• Is there also an improvement in social
  communication skills?
• Is there a decrease in social communication
  skills?
• But theres a problem
• Most assessment tools for autism dont assess the
  social communication skills that are important in
  distinguishing children with autism from
  typically developing children
• Instead, they emphasize their problems with
  communication
• None are standardized on well-diagnosed samples
  and none are age-normed

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Clinical Questions

• When a child with autism has difficulty
  communicating. . . .
• Is it because he or she has autism? (i.e., other
  children with autism show similar problems at the
  same skills level)
• Is something else going on? (i.e., childs
  communication is much worse than we would expect
  or is restricted to certain settings)
• But theres a problem
• Assessment tools for autism are not
  age-standardized on children with autism
• Assessment tools for autism are not standardized
  on different types of informants

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Problems with Existing Assessment Tools

• Except for the ADI-R and ADOS-G, all of the
  assessment tools focus exclusively on problem
  behaviors and do not reflect current research on
  behaviors that differentiate children with autism
  from other groups
• None of the assessment tools are age-normed
• Only one provides standard scores (GARS) but the
  diagnostic criteria defining the standardization
  sample are poorly described
• Except for the ADI-R and ADOS-G, all focus on
  behavior problems seen in the more severely
  affected cases
• None of the assessment tools are tailored to
  inputs from teachers/therapists (important for
  assessing generalization)

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PDD Behavior Inventory (PDDBI)

• The PDDBI can be used to assess response to
  intervention, assist in diagnosis and treatment
  planning, and help with research
• It
• Assesses both problem behaviors and appropriate
  social communication behaviors (important in
  assessing improvement)
• Is age-normed (because there is a need to assess
  change due to age from that due to treatment)
• Includes items that are based on the latest
  research on behaviors that discriminate autism
  from other conditions
• Is standardized on a well-diagnosed autism sample

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Uses of the PDDBI

• Clinical
• Assisting in Diagnosis and Treatment
  Recommendations
• Monitoring Changes at Follow-Ups, etc.
• Educational
• Assisting in Placement Decisions
• Assisting in Treatment Planning
• Monitoring Students Progress, etc.
• Research
• Measuring Response to Novel Treatments
• Identifying Meaningful Sub-Groups
• Assessing (Endo)phenotypes in Genetic Studies,
  etc.

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Assisting in Diagnosis

• Does the childs profile of domain scores look
  like someone his/her age with autism?
• Is the profile consistent with your observations?
• Does the profile suggest an alternate and/or
  co-morbid diagnosis that needs to be considered
  (diagnostic overshadowing?)?
• Do the domain profiles of parent and teacher
  agree?
• If not, which scores differ?
• If they differ, does this say something about
  diagnosis (e.g., Selective Mutism)?

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Assisting in Placement Decisions

• Is the childs problem behavior profile typical
  of someone his/her age with autism?
• If not, are some scores so high that a special
  treatment setting may be necessary?

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Treatment Planning

• Is the childs social-communication behavior
  profile typical of someone his/her age with
  autism?
• If not, do domain scores suggest some other
  diagnosis should be considered, e.g., Aspergers?

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Research

• The PDDBI can be helpful and is being used for
  measuring meaningful change as a result of
  intervention (e.g., medication, ABA, dietary,
  etc.) for people in the autism spectrum
• For groups (e.g., Are people in my school
  improving? Is my intervention associated with
  improvement?)
• For individuals (Has this person improved?)
• If so, in what areas?
• If so, is it a meaningful decrease in autism
  traits?
• It is also being used in large scale genetics
  studies to identify genes associated with certain
  types of autistic behaviors

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Some Research Programs Using PDDBI

• Arizona State University
• Arkansas Childrens Hospital Research Unit
• ASD-Canadian American Research Consortium
• Baylor College of Medicine
• Binghamton University
• Carlos Albizu University
• Cleveland Clinic Center for Autism
• Columbia University - Psychiatric Institute
• Massachusetts General Hospital
• M.I.N.D. Institute
• Mount Sinai Hospital Seaver Center (Manhattan)
• National Institute of Mental Health (NIMH)
• Ohio State University
• Royal Prince Alfred Hospital, Sydney, Australia
• St. Marys Hospital (Wisconsin)
• University of California San Diego
• University of Illinois
• University of North Carolina Chapel Hill
• Washington State University

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PDDBI

• As will be shown, we have found the PDDBI to be
  both reliable and valid
• It can be used for assessing children on the
  autism spectrum who are between 18 months and
  12-1/2 years of age

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Autism and the Related PDDs
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http//www.time.com/time/covers/1101030120
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Autism and the Related PDDs
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Earliest Description of Autism?

• If a woman gives birth and the infant rejects
  the mother
• Summa Izbu IV 42
• Ancient Mesopotamian medical text (translated by
  M. Coleman, M.D.)

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Leo Kanners Observations (1943)(Kanner, L.
Autistic disturbances of affective contact.
Nervous Child, 2, 217-250.)Sample 8 boys 3
girls

• inability to relate themselves in the ordinary
  way to people and situations from the beginning
  of life
• Of 8 speaking children, none used language to
  convey meaning
• echolalia and delayed echolalia
• affirmation by repetition
• literalness
• personal pronouns are repeated as heard

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Kanners Observations (continued)

• Excellent rote memories
• all powerful need for being left undisturbed
• loud noises and moving objects reacted to with
  horror
• anxiously obsessive desires for the maintenance
  of sameness
• routines
• furniture arrangements

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Kanners Observations (continued)

• Monotonous and repetitive motions and verbal
  utterances
• Good relation to objects - not to people
• intelligent physiognomies

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Modern Descriptions of Autism

• Kanner (1943)
• British Working Party (1963)
• Rimland (E-1 and E-2 Scales) (1964)
• Rutter (1972)
• Ritvo and Freeman (NSAC) (1977)
• DSM III (First use of PDD term) (1980)
• DSM-III-R (1987)
• DSM-IV (1994)

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Diagnostic History of PDD

• DSM III (1980)
• Pervasive Developmental Disorder
• Infantile Autism
• Childhood Onset Pervasive Developmental Disorder
• Atypical Pervasive Developmental Disorder
• DSM III-R (1987)
• Pervasive Developmental Disorder
• Autistic Disorder
• Pervasive Developmental Disorder - NOS

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Current Nosology

• DSM-IV (1994)
• Pervasive Developmental Disorder
• Autistic Disorder
• Childhood Disintegrative Disorder
• Retts Disorder
• Aspergers Disorder
• Pervasive Developmental Disorder NOS
• All represent the autism spectrum

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Autistic Disorder (DSM-IV)

• 1) Qualitative impairment in social interaction
  (Problems with eye contact, facial expression,
  body posture, gestures, peer relationships,
  sharing interests, emotional reciprocity)

2) Qualitative impairments in communication
(Delay or lack of language, problems with
conversational desire/skill, stereotyped
language, problems with social and imaginative
play) 3) Restricted repetitive and stereotyped
patterns of behavior, interests and activities
(Preoccupations, inflexible adherence to routines
or rituals, stereotyped movements, preoccupation
with parts of objects) 4) Onset prior to 3 years
Not Retts or Disintegrative
http//news.sie.edu
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Candle fixation at birthdays
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Co-morbid Features

• Anxiety Problems and Anxiety Disorders
• Hyperactivity Common
• Sleeping, Eating, and, sometimes, GI Disturbances
  
• Incongruous Mood States and Mood Disorders
• Self-Injurious Behaviors Sometimes Seen
• Savant Skills in Small Percentage
• Tics Sometimes Seen
• Epilepsy in 30 to 40 by adulthood
• Genetic Syndromes

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PDD-NOS

• Also known as Atypical Autism
• Criteria not met for one of the other PDDs due to
  age of onset, or atypical symptoms, or
  sub-threshold symptoms or all of these
• There is severe and pervasive impairment in
  development of reciprocal social interaction
  skills and impairment in communication skills OR
  presence of stereotyped behaviors, interests, and
  activities

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Aspergers Disorder
?

• Same characteristics as Autistic Disorder, but
• No general language delay (single words by 2
  years communicative phrases by 3 years)
• No delay in cognitive development or self-help
  skills or curiosity about the environment
• Not other PDD or schizophrenia

Jerry Espenson Boston Legal
?
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Retts Disorder

• Normal pre- and peri-natal development
• Normal psychomotor development up to 5 mos.
• Normal HC at birth-HC deceleration 5-48 mos.
• Loss of purposeful hand skills (hand wringing)
• Loss of social engagement
• Poorly coordinated gait and trunk movements
• Severe language disorder and retardation
• Breathing abnormalities common
• Due to MECP2 gene mutation ? absence of MECP2
  protein ? absence of gene suppression
• Leaky genes

http//www.rettsyndrome.org.uk
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Childhood Disintegrative Disorder

• Normal development first 2 years
• Loss of skills before 10 years in at least 2
• Expressive or receptive language
• Social or adaptive skills
• Bowel/bladder control
• Play
• Motor skills
• Abnormalities in at least 2
• Qualitative social interaction
• Qualitative impairment in communication
• Repetitive behaviors, restricted interests
• Not other PDD or schizophrenia

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Differential Diagnosis Issues

• Receptive-Expressive Language Disorder
• Mental Retardation without PDD
• ADHD
• Deafness/Hearing Impairment
• Selective Mutism
• Reactive Attachment Disorder

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Autism/PDD-NOS Characteristics

• Most are males (about 75-50)
• Developmental delay is common (about 70)
• Parents recognize problems around 18 months,
  sometimes with loss of skills
• Enlarged head circumference sometimes seen in
  younger children (about 37)
• Genes play a strong role in etiology

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What Causes Autism?
Genetic Known/Unknown
Pre/Post-Natal Brain Development/Function
Autisms (Disorders/Syndromes) (Autism is an
etiologically heterogeneous disorder, as is the
case with mental retardation)
Pre/Post-Natal Environment (viruses, hormones,
neurotransmitters, etc.)
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Known Genetic Conditions Associated With Autism

• Fragile X Syndrome
• About 2 to 8 in males or females with autism
• About 15 of fragile X males have autism
• Other Genetic Disorders/Conditions
• Untreated Phenylketonuria (PKU)
• Tuberous Sclerosis in about 3 of cases
• Angelmans Syndrome
• Chromosome 15q11-13 Duplications (maternal
  origin) (Cook, et al., 1997) - Same region as
  Prader-Willi (maternal) and Angelmans (paternal)
  Deletion Syndromes

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Genetics of Autism

• Twin studies (Bailey, et al 1995)
• 60 concordance for autism in 25 MZ twins None
  in DZ
• 92 concordance for cognitive impairment in MZ
  twins 10 in DZ twins

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The Broader Phenotype

• Autism, per se, may not be inherited
• Rather, there appears to be a Spectrum of social
  and language problems inherited in some families.

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Genetic Factors in Autism

• Family Studies
• Risk of Autism in siblings of proband 5 to 9
• Risk of Autism itself in the population about
  0.5
• Risk of Aspergers or PDDNOS in siblings 3
• Risk of other social or communication impairments
  or restricted interests 20 in siblings
• Risk of Mood Disorders is elevated in family
  (siblings, parents, extended family)

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Other Medical Issues

• There is very little evidence for the role of the
  following in causing autism
• Heavy metals such as mercury
• Vaccines such as MMR and DPT
• Gastro-intestinal problems
• Many of these issues are currently being
  investigated at various centers
• The role of immune system problems and CNS
  inflammation in autism are also major questions

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Oxidative Stress Problems in Autism? Levels of
transferrin and ceruloplasmin (antioxidants) are
lower in children with autism who lost skills
relative to sibs
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• Administration and Scoring

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Administration

• Can be completed at home, school, or clinicians
  office (should be free from distractions)
• Ensure confidentiality in reporting
• Clinician should indicate with an X or check mark
  whether informant is to complete standard or
  extended form
• Standard if primary concern is with autism
  diagnosis-related behaviors (e.g., prevalence
  studies)
• Extended if concern is with autism behaviors and
  more generic behavior issues

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General Issues in Administration

• Give an estimate of amount of time needed to
  complete the PDDBI (about 20-40 minutes depending
  on standard or extended form)
• Review scoring for
• Question marks (review item with respondent)
• Missing responses or multiple responses
• Missing dates (birth dates and current date)

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PDDBI Domains

• Domains were conceptually organized as follows
• Approach/Withdrawal Dimension
• Social Communication Skills
• Domains assess behaviors important for autism
  (Standard Form) and for associated behavior
  problems that are not unique to autism (Extended
  Form)
• Different versions were created for parents and
  teachers (a generic term that includes teachers,
  speech therapists, aides, ABA instructors, etc.)

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PDD Behavior Inventory (PDDBI)

• Approach-Withdrawal Problems (Repetitive,
  Ritualistic Pragmatic Problems)
• Sensory/Perceptual Approach Behaviors
• Ritualisms/Resistance to Change
• Social Pragmatic Problems
• Semantic/Pragmatic Problems
• Arousal Regulation Problems
• Specific Fears
• Aggressiveness
• Composite Scores
• (Receptive)/Expressive Communication Skills
• Social Approach Behaviors
• Expressive Language
• Learning, Memory and Receptive Language
• Composite Scores
• Autism Composite Score

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Domains and Item Scoring

• A nested approach was used for each domain
• Each domain in the PDDBI is made up of a subset
  of different clusters
• For example, the Sensory/Perceptual Approach
  Behaviors domain has 5 clusters in the parent
  version tapping a variety of repetitive behaviors
• Each cluster consists of 4 or more exemplars and
  each is rated on a Likert scale with the
  following options
• 0 (Does Not Show Behavior) 1 (Rarely Shows
  Behavior)
• 2 (Sometimes/Partially Shows Behavior)
• 3 (Usually/Typically Shows Behavior) and ?
  (Dont Understand)
• Each domain is scored (the raw score) by summing
  the ratings, taking missing items into account
• Standard scores are computed from the tables and
  entered on the Summary Sheet

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PDD Behavior Inventory (PDDBI)Scoring System
(T-Scores)

• Each domain and composite was age-normed and
  according to a T-score system (mean50 SD10)
• The higher the T-scores for the
  Approach-Withdrawal domains and the Autism
  Composite Score, the more severe or discrepant
  that childs scores are from the average child
  with autism
• The higher the T-score for the Receptive/Expressi
  ve Social Communication Abilities domains, the
  better that childs skills are relative to the
  average child with autism

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Domain Profile Form

• Standardized T-scores (refer to tables or
  software) can be plotted on the Profile Form

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Cluster Score Summary Table

• Cluster scores within domains can be
  qualitatively examined along an ordinal dimension
  for their clinical importance

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Test Materials

• Parent Form (PDDBI-P PDDBI-PX)
• PDDBI-P (124 Items)
• PDDBI-PX (188 items)
• Teacher Form (PDDBI-T PDDBI-TX)
• PDDBI-T (124 Items)
• PDDBI-TX (180 items) Score Summary Sheets
• Profile Forms
• (PDDBI-SP software)
• XExtended (if concern is with autism behaviors
  and with more generic behavior issues)

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Appropriate Populations

• Any child with a Pervasive Developmental Disorder
• Ages 18 months through 12 years, 5 months
• English speaking informants
• Flesch-Kincaid Reading Level Grade 4.7
• Gunning Fog Index 7.8 (Readers Digest level)

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Selecting Raters

• Parent
• Parent or legal guardian with the most recent and
  frequent contact over the previous 6 months
  (ideally both parents)
• Teacher
• Teacher or other professional (speech therapist
  teachers aide, etc.) must have had at least
  daily contact for at least one month or more than
  4 weeks of several days per week contact

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Approach-Withdrawal Problems Clusters
(Repetitive, Ritualistic Pragmatic Problems)

• Sensory/Perceptual Approach Behaviors
• (Head to Body Arrangement) - SENSORY
• Visual Behaviors
• Non-Food Taste Behaviors
• Touch Behaviors (PDDBI-P)
• Noise Making Behaviors (PDDBI-T)
• Proprioceptive/Kinesthetic Behaviors
• Repetitive Manipulative Behaviors
• Gait-Based Kinesthetic Behaviors (PDDBI-T)

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Approach-Withdrawal Problems Clusters
(Repetitive, Ritualistic Pragmatic Problems)

• Ritualisms/Resistance to Change (RITUAL)
• Resistance to Change in the Environment
• Resistance to Change in Schedules/Routines
• Rituals
• Social Pragmatic Problems (SOCPP)
• Problems with Social Approach
• Social Awareness Problems
• Inappropriate Reactions to the Approaches of
  Others

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Approach-Withdrawal Problems Clusters
(Repetitive, Ritualistic Pragmatic Problems)

• Semantic/Pragmatic Problems (SEMPP)
• Aberrant Vocal Quality When Speaking
• Problems with Understanding Words (e.g.,
  echolalia)
• Verbal Pragmatic Deficits (e.g., problems with
  conversations or perseverative language)

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Approach-Withdrawal Problems Clusters

• Arousal Regulation Problems (AROUSE)
• Kinesthetic Behaviors
• Reduced Responsiveness
• Sleep Regulation Problems (PDDBI-P)
• Specific Fears (FEARS)
• Sadness When Away From Caregiver(PDDBI-P)
• Anxiousness When Away From Caregiver(PDDBI-P)
• Auditory Withdrawal Behaviors
• Fears and Anxieties
• Social Withdrawal Behaviors

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Approach-Withdrawal Problems Clusters

• Aggressiveness (AGG)
• Self-Directed Aggressive Behaviors
• Incongruous Negative Affect
• Problems when CaregiverReturns from an Outing or
  Vacation
• Aggressiveness Toward Others
• Overall Temperament Problems

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(Receptive)/Expressive Communication Skills
Clusters

• Social Approach Behaviors (SOCAPP)
• Visual Social Approach Behaviors
• Positive Affect Behaviors
• Gestural Approach Behaviors
• Responsiveness to Social Inhibition Cues
• Social Play Behaviors
• Imaginative Play Behaviors
• Empathy Behaviors
• Social Imitative Behaviors
• Social Interaction Behaviors (PDDBI-P)

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(Receptive)/Expressive Communication Skills
Clusters

• Expressive Language (EXPRESS)
• Vowel Production
• Consonant Production
• Diphthong Production
• Expressive Language Competence
• Verbal Affective Tone
• Pragmatic Conversational Skills

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Receptive/Expressive Communication Skills Clusters

• Learning, Memory, and Receptive Language (LMRL)
• General Memory Skills
• Receptive Language Competence
• Associative Learning (PDDBI-T)

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Composite Scores

• Approach-Withdrawal Problems (AWP)
• Repetitive, Ritualistic Pragmatic Problems
  (REPRIT)
• Receptive/Expressive Social Communication Skills
  (REXSCA)
• Expressive Social Communication Skills (EXSCA)
• Autism
• (SENSORYRITUALSOCPPSEMPP)
• (SOCAPP EXPRESS)

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Discrepancy Scores

• ?Social Pragmatic Problems Social Approach
  Behaviors?
• ?Semantic/Pragmatic Problems Expressive
  Language?
• ?Parent - Teacher?

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Development and Standardization
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You can observe a lot by just watchingYogi
Berra
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Development

• Items were selected based on observation and by
  review of research studies
• The items were chosen to best represent the
  cluster to which they were assigned

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Percent with ADI or ADOS confirmed Autism 92
(PDDBI-P) 89 (PDDBI-T)
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Reliability

• Internal Consistency (Alpha)
• Stability (Test-Retest)
• Interrater

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Internal Consistency
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Internal ConsistencyG. Leonard Burns, Ph.D.,
Washington State University, Pullman, WA

• Mother Father Teacher Aide
• Approach-Withdrawal Problems (.81-.90)
  (.85-.92) (.82-.92) (.81-.92)
• (.83-.90) (.81-.89)
• Receptive/Expressive (.92-.98) (.94-.98)
  (.95-.99) (.93-.95)
• (.91-.95) (.95-.97)

PDDBI Manual Data
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Stability
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Interrater (Teacher-Teacher)
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Interrater (Parent-Teacher)
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InterraterG. Leonard Burns, Ph.D., Washington
State University, Pullman, WA

• Mother-Father Teacher-Aide Mother-Teacher
  
• Approach-Withdrawal (.44-.59)
  (.46-.63) (.20-.55)
• (.40-.83) (.23-.55)
• Receptive/Expressive (.70-.85) (.59-.89)
  (.65-.82)
• (.85-.92) (.51-.82)

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Validity

• Internal Structure (intercorrelation matrices)
• Construct (principal components analyses)
• Developmental
• Criterion-Related
• Clinical

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Internal Structure
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Internal Structure
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Construct Validity
86
Factor 1 Confirms Social Communication
Dimension Factor 2 Confirms Approach-Withdrawal
Dimension
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Developmental Validity
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Criterion-Related Validity

• Childhood Autism Rating Scale (CARS)
• Nisonger Child Behavior Rating Form (CBRF)
• Vineland Adaptive Behavior Scales
• Griffiths Mental Development Scales

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Clinical Validity

• ADI-R (AGRE Definitions)
• Autism
• Not Quite Autism
• Broad Spectrum
• ADOS-G
• Autism
• Spectrum
• Not Spectrum
• Vineland Adaptive Functioning Level
• Adequate/Moderately Low
• Mild/Moderate
• Severe/Profound
• Seizure Disorders

http//www.agre.org/agrecatalog/algorithm.cfm
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• Clinical Interpretation

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PDDBI Profiles

• For clinical and research purposes, it is
  important to examine the overall profile of
  scores, as well as the magnitude of the composite
  scores, for both parent and teacher observations
• Such profiles can provide important information
  about the child, and identify behaviorally-defined
  sub-groups
• Remember that the PDDBI is standardized on an
  autism sample.

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Case 1 - Michael

• Visit 1 (23 months of age)
• Vineland
• Communication 6 months
• Socialization 11 months
• Motor Skills 23 months
• ADOS-G Autism
• Visit 2 (28 months of age After 25 hrs/wk of ABA
  and O.T.)
• Vineland
• Communication 21 months
• Socialization 20 months
• Motor Skills 28 months
• ADOS-G Autism Spectrum Disorder

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Case 4 - Albert

• IQ (SB-5) 103
• Vineland
• Communication 89
• Daily Living Skills 60
• Socialization 59
• Extreme anxiety noted on first observation
• History of aggressiveness as presenting problem
  with PDD
• Positive family history for anxiety and
  depression
• Medication
• Visit 1. 5 years, 11 months of age -
  Dextroamphetamine
• Visit 2. 7 years of age - Olanzapine (Zyprexa)
  became manic on an SSRI with d-amphetamine
• Final Diagnoses BP II Social Anxiety Disorder
  Aspergers Disorder

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Case 5 - Ted

• Boy, 10 years of age with Fragile X Syndrome
• Vineland
• Communication 63
• Daily Living Skills 46
• Socialization 65
• Medication Methylphenidate (Ritalin)
• History of delayed milestones

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Teds Cluster Scores
SOCPP
SEMPP
SOCAPP
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Case 6 - Huda

• Girl, age 3 years, 2 months
• Retts Disorder
• Vineland
• Communication 43
• Daily Living Skills 42
• Socialization 50
• Motor Skills 39
• ADOS-G Autism

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Retts Disorder

• Normal pre- and peri-natal development
• Normal psychomotor development up to 5 mos.
• Normal HC at birth-HC deceleration 5-48 mos.
• Loss of purposeful hand skills (hand wringing)
• Loss of social engagement
• Poorly coordinated gait and trunk movements
• Severe language disorder and retardation
• Breathing abnormalities common
• Due to MECP2 gene mutation ? absence of MECP2
  protein ? absence of gene suppression
• Leaky genes

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Case 7 JM

• 4 year, 10 month old boy with Costello Syndrome
• Grand mal at 3 months of age stopped breathing
• Currently petit mal seizures
• Tested positive for autism on the ADOS-G
• Parent report data provided by Dr. G. Hintz
  (Wisconsin)

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Costello Syndrome

• Rare multi-organ disorder of unknown etiology
• Physical characteristics
• Growth delay
• Short stature
• Excessive skin on neck, palms, fingers, soles
• Characteristic facial appearance
• Macrocephaly
• Low set ears
• Thick ear lobes and lips, wide nostrils
• Cognitive delay
• Behavior Warm, sociable, and humorous

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Do PDDBI Domains Reflect Improvement?
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Discriminating PDD from Non-PDD Cases
PDD (n475) vs. Not PDD (n50 language impaired,
emotional problems, typically developing, etc.) -
PDDBI-P Autism Composite Area under curve0.90
(/- .023) 95 CI .86-.95
119
Summary

• The PDDBI is a new reliable and valid tool for
  measuring treatment effects assisting in
  diagnosis, placement, and treatment planning and
  analyzing behavioral sub-groups
• It is sensitive to shifts in diagnostic status
• It correlates well with other measures of autism
  and adaptive skills

120
PPV and NPV Sensitivity and Specificity90
121
PPV and NPV Sensitivity and Specificity90
122
PPV and NPV Sensitivity and Specificity90