Pervasive Developmental Disorders A case study

Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification. Cochlear implant surgery June of 1999 at age 4. Use is consistent. Student is committed to use. Speech and listening skills not improving. Relies on sign language for meaningful communication. Entered Special Needs Preschool at age 3 and has been receiving services since.

Concerns Andy has always engaged in self stimulating behaviors. When he entered preschool, he was orally and tactically defensive. Not able to adjust to unexpected deviations in routine. Tends to obsess over objects being in line, rituals. Repeats himself. Learns rote material with little repetition, but cannot generalize. Easily distracted; not able to divide attention. These behaviors are obstacles to Andys instruction. I wanted to gain an understanding to be better able to serve him and to address issues as needed.

Andy is currently receiving services for his hearing loss. Because an official diagnosis would not provide additional services, it is not being pursued. However, to provide effective services, an understanding of PPD is essential. Of particular concern was his self-stimulating behavior. He is currently mainstreamed in a first grade class. This behavior is noticeable to the other students and becomes the target of their teasing.

Behavior: Crossing arms for self-stimulation when excited. Intervention: Began with touching arms and telling him to put his arms down. The cue was lessoned to a meaningful look at his arms. BASELINE INTERVENTION

Pervasive developmental disorder (PPD) is a broad term used to describe a set of symptoms that occur along a continuum of severity. This continuum is often referred to as Autism Spectrum Disorders (ASD).

The DSM-IV criteria consists of a set number of characteristics from three categories: A. (1) qualitative impairment in social interaction, (2) qualitative impairments in communication, (3) restricted, repetitive, and stereotyped patterns of behavior, interests, and activities, and, B. Delays or abnormal functioning in at least one of the following areas, with onset prior to age 3 years: (1) social interaction, (2) language as used in social communication, or (3) symbolic or imaginative play. C. The disturbance is not better accounted for by Rett's disorder or childhood disintegrative disorder.

Characteristics associated with PPD are: 1. Withdrawn behaviors 2. Unsocial behaviors 3. Problems using language to communicate 4. Repetitive patterns of behavior 5. Problems with imaginative play 6. Unable to deviate from schedule 7. Self-stimulating behavior

Some common disorders on this continuum are: 1. Aspergers Syndrome 2. Pervasive Developmental Disorder 3. Retts Syndrome 4. Childhood Disintegrative Disorder 5. Autism NICHD reports that 1in 500 people have some form of autism.

Aspergers Syndrome Unsocial behaviors Repetitive patterns of behavior Problems with imaginative play Good language skills Normal or near normal IQ

Pervasive Developmental Disorder Signs of developmental problems, but without the significant autism symptoms. Characteristics vary widely, but tend to be of a lesser degree than autism. Sometimes referred to as Pervasive Developmental Disorder-not otherwise specified, or PDD-NOS.

Retts Syndrome Has only been seen in girls. Development is normal until months, then language and motor abilities are lost. The development of the nervous system is not controlled leading to the loss of acquired skills. Normal milestones are stunted. Rate of progression is specific.

Current Research NIHs collaborative Program of Excellence in Autism has also identified a genetic origin for Retts Syndrome. Girls have two X chromosomes, therefore some genes are repeated. Randomly one gene will shut down and the remaining one will control the process. The disorder is the result of the mutation on the gene that makes methyl cytosine binging protein 2 (MECP2). The abnormality interferes with the biochemical regulator (MECP2) of the genes expression.

The defect prohibits a gene system from being shut down allowing too much of a good protein to be produced. During infancy a balance system is in place to decrease the interference of that protein. At the age of 2, the balance system no longer functions allowing the effects of the overproduction to surface. It is believed that the syndrome is limited to girls because males have only one MECP2 producing gene. Therefore, if it is mutant, they have no back up copy and die before birth.

Autism 1. Withdrawn behaviors 2. Unsocial behaviors 3. Problems using language to communicate 4. Repetitive patterns of behavior 5. Problems with imaginative play 6. Seizures (30%) 7. Most often seen in boys 8. Hypersensitivity to stimulation (visual, auditory, tactile), especially to being touched and loud noises 9. Little or no eye contact 10. Digestive disorders are common

11) Lack of eye contact 12)Inappropriate affect 13)Echolalia 14)Non-responsive to verbal cues 15)Inappropriate attachment to objects 16)Severe emotional outbursts without apparent cause

150 sibling pairs found strong evidence to indicate chromosomes 2 and 7 are involved with autism. Another possible location, although the correlation is not as strong, is chromosomes 16 and 17. Chromosome 7 is known to be linked to language disorders. Not exactly sure what is wrong on the gene to cause the disorder. In Fall of 2001, NIHs collaborative Program of Excellence in Autism identified a suspected chromosome for autism.

In the Fall of 2002, NIHs Collaborative Program of Excellence in Autism (10 multidisciplinary programs) identified a gene, HOXA1, that may predispose a person to autism.

Childhood Disintegrative Disorder Normal development to age 2 then rapidly lose acquired skills (language, self-help). Usually presents between months, but can happen up to age 10. Child loses interest in the environment. Very rare.

Interventions PDD is usually treated with either: behavioral therapy, pharmacological therapy, or a combination of both.

Behavioral Therapy One-on-one speech Communication therapy – Picture Exchange Communication System (PECS) Modeling to teach social, self-help skills, communication and learning This approach is generally effective, depending heavily on the intensity of the therapy and the severity of the child.

Pharmacological Therapy Usually used for seizures, self-injurious behaviors, digestive difficulties Secretin Therapy – A hormone involved in intestinal processes – A double blind study where each participant received both treatment and saline, using a form of secretin derived from pigs, found no difference between it and placebo. (Journal of the American Academy of Child and Adolescent Psychiatry, Nov 2001) A list of scientifically unreplicated therapies can be found at

PDD and deafness Hearing loss (HL) is difficult to detect in children with PDD. One of the hallmarks of PDD is non-responsiveness to auditory cues. This makes it difficult to know if the child is not hearing or just not responding. Oral communication is problematic without HL. Visual communication requires attending and eye contact. Typically people with PDD avoid both of these. Tactile defensiveness and over sensitivity to stimuli may prevent hearing aid fitting and use. Risk of damage and injury are increased in the presence of self-stimulating or injurious behaviors.

Resources National Institute of Child Health and Human Development General Information Autism Biomedical Information Network (Includes complete DSM-IV criterion) Autism society of AL Aspergers Disorder DSM-IV Personal Accounts and links

Resources, cont. Autism/PDD Resources Network Governers Council for Developmental Disabilities for GA Autism Society of Americas Greater GA Chapter GA Tools for Life Parent to Parent of GA parenttoparentofga.org GA State Resources at NICHCY Hands on Atlanta-Autism Society of America