1. Pervasive Developmental Disorders September 6, 2005 PSYC 4930

2. Pervasive Developmental Disorders  The current DSM-IV category of Pervasive Developmental Disorders includes several more severe forms of child psychopathology.  Historically disorders of this type have been referred to by a variety of labels such as atypical psychosis child psychosis symbiotic psychosis childhood schizophrenia infantile autism

3. Pervasive Developmental Disorders  Pervasive Developmental Disorders are characterized by impairments/difficulties in several areas: social interactions, communication skills, and stereotyped behaviors/interests/activities

4. Pervasive Developmental Disorders  At one time, conditions referred to as Pervasive Developmental Disorders were often thought to be reflective of Child Psychosis  However, PDD are not related to the psychotic conditions of adulthood (e.g. Schizophrenia), and are now referred to as "developmental" rather than "psychotic" disorders.

5. Pervasive vs. Specific Disorders  PDD are to be distinguished from "Specific Developmental Disorders" (e.g., reading, articulation, arithmetic, and language disorders).  This is because they are characterized by severe disturbances in many basic areas of development.  They may also be reflected in behaviors having no counterpart in normal development.  Thus, children with these conditions often display distorted rather than simply delayed development.

6. Pervasive Developmental Disorders  Several disorders are included under the DSM-IV heading of Pervasive Developmental Disorders: Rett's Disorder Childhood Disintegrative Disorder Asperger's Disorder Autistic Disorder  With the exception of autism, little is known regarding most of the other disorders in this category, although case studies describing each of these conditions can be found in the clinical literature.

7. Pervasive Developmental Disorders: Rett’s Disorder  This disorder, which was first described in the German medical literature in 1966 is a degenerative disorder manifested in both physical and behavioral symptoms  The disorder has its initial onset after a period of apparently normal early development.  Onset is usually thought to be toward the end of the first year or the beginning of the second year.  However, it can occur as early as 5 months or as late as 48 months.

8. Pervasive Developmental Disorders: Rett’s Disorder  Major symptoms include the following; a deceleration in normal head growth a loss of previously acquired hand movements the appearance of poorly coordinated gait and/or trunk movements  The loss of previously existing motor skills is usually accompanied by the development of stereotyped hand movements such as hand wringing or washing type movements

9. Pervasive Developmental Disorders: Rett’s Disorder  Other symptoms include developmental regression in both expressive and receptive language  This is usually accompanied by severe psychomotor retardation  Behavior is often characterized by autistic-like behaviors which include; stereotypic behaviors (e.g., hand movements noted above) a lack of sustained interest in persons and objects and a marked decrease in interpersonal contact

10. Rett’s Disorder: Prevalence and Course  Prevalence of Rett's disorder has not been well established, but it is thought to be much rarer than autism  Occurs only in females  Usually associated with severe mental retardation.  Marked by rapid deterioration after initial onset and a course which is chronic and often progressive  However, sometimes a renewed interest in social interactions may appear as the child becomes older

11. Rett’s Disorder: Etiology  Little is known regarding etiological factors  There are some neurological findings associated with Rett’s (e.g., motor symptoms, abnormal EEG, cortical atrophy on CT scans, cerebrospinal fluid abnormalities)  These factors suggested that there may be reason to view the disorder as a progressive degenerative brain disorder

12. Rett’s Disorder: Treatment  Very little information or research on the treatment of this condition  Possible treatments include: Behavioral approaches designed to deal with the behavioral deficits and excesses special education approaches to deal with cognitive impairments physical therapy to assist with the motor problems that result from this condition.

13. Pervasive Developmental Disorders: Childhood Disintegrative Disorder  Originally labeled as "dementia infantilis" by Heller (1930), Childhood Disintegrative Disorder is another severe and progressive disorder occurring after a period of normality  Usually develops after age two, and involves a rapid regression in behavior where there may be a loss of social, language, motor, and other skills (e.g., play, bladder and bowel control)

14. Childhood Disintegrative Disorder  Other symptoms include impairments in social interaction and communication as well as repetitive and stereotyped patterns of behavior or interests  The regression or disintegration seen in the disorder usually takes place over a period of six to nine months

15. Childhood Disintegrative Disorder vs. Autism  The obsessional and stereotypic behaviors and the impairment in social interactions that often accompany this disorder can resemble certain aspects of autistic disorder.  However, this disorder can be distinguished from autism by its later age of onset and the absence of other autistic features (e.g., language characteristics, desire for the maintenance of sameness)

16. Childhood Disintegrative Disorder  Childhood Disintegrative Disorder is very rare and more common in males than females  May develop slowly or suddenly (between ages of 2 and 10) and its course is variable  Sometimes, after the initial loss of skills, the condition will remain static, with some limited improvement in the area of social behavior  In other cases there is progressive deteoriation  Usually, however, problems in social interaction, communication, and behavior remain relatively constant over time

17. Childhood Disintegrative Disorder: Etiology  As with Rett's Disorder, little information is available regarding the etiology of this disorder  Central nervous system involvement plays a role  While these findings are suggestive, more definitive investigations into the etiology of this disorder remain to be done.

18. Childhood Disintegrative Disorder: Treatment  Multi-component approach similar to that suggested for the treatment of Rett's disorder might be useful  Use of behavioral approaches to modify problematic behavioral excesses and deficits, along with appropriate special educational approaches

19. Pervasive Developmental Disorders: Asperger’s Disorder  First published account of this disorder by Austrian psychiatrist Hans Asperger in 1944 who initially referred to the condition as "autistic psychopathy“  However, more recent authors have commented on the similarities between Autism and Asperger’s  Indeed, there is some debate as to whether this disorder is actually distinct from autistic disorder

20. Asperger’s Disorder  Essential features of the disorder: severe impairments in social interactions restricted and repetitive patterns of interests, activities and/or behaviors  No clinically significant delays in cognitive development, language development, or impairments in adaptive behavior, apart from those related to impairments in social interaction  While not considered a specific symptom of Asperger's Disorder, children with this condition are often delayed in meeting major motor milestones (e.g., crawling, walking) and are frequently characterized as clumsy

21. Asperger’s Disorder  Unlike autism, where social impairments seem to result from an intense desire to withdraw from or avoid social interactions, the social impairment in Asperger’s seems to result from a lack of social skills.  These children seem to have a marked inability to understand and use rules which typically guide social behavior.  The child may show significant problems with: nonverbal behaviors such as maintaining appropriate physical proximity to others while interacting making and sustaining eye contact appropriately using gestures, facial expression and other nonverbal behaviors to regulate social interactions

22. Asperger’s Disorder  Social behavior often appear quite egocentric and self-centered  Children with Asperger’s may frequently pursue their own highly personalized interests in social encounters without apparent awareness that the other person does not share similar interests  Behavior occurring within the context of two way social interactions often appears as inept, naive and peculiar

23. Asperger’s Disorder  Restricted and repetitive patterns of behaviors, interests, or activities are often striking and may be manifest in a variety of ways: Some may be preoccupied with specific activities (e.g., spinning objects) or become overly attached to certain objects or familiar places and become intensely upset when separated from them Others show an inflexible adherence to daily routines

24. Asperger’s Disorder  Commonly, children with this disorder show an extreme all-consuming involvement in some specific area of interest  The child may spend most of his/her time learning facts related to the area  They may collect things having to do with the area, and spend an enormous about of time talking to others about this area whether or not they are interested  While, investing a great deal of time learning about their area of interest, the child may have little understanding of the facts that they learn

25. Asperger’s Disorder  Unlike other PDD’s, in Asperger's Disorder, there is no clinically significant delay in cognitive development or in the area of language  the child may learn to speak at a normal age however, they may show marked peculiarities in language: may invent words use pronouns incorrectly repeat words or phrases over and over in a stereotyped manner  speech content is often overly pedantic, consisting of long one-sided discussions about the child's favorite topic  CASE EXAMPLE

26. Asperger’s Disorder: Prevalence  little data on prevalence, but children with Asperger's Disorder are quite rare  probably more rare than autism  more common in males than in females with sex ratios ranging from 4:1 to 9:1 being reported

27. Asperger’s Disorder: Etiology  Regarding etiology, the disorder was originally considered to have a genetic basis (Asperger, 1944).  While no formal studies to assess the possibility of genetic transmission have been published, Wing (1981) has provided relevant case study findings: of the 34 cases with this disorder that she studied, 5 of the 16 fathers and 2 of the 24 mothers had, "to a marked degree" behavior resembling that observed in their children  Although not definitive, some support for the role of biological factors in this disorder comes from the fact that these children sometimes show evidence of nonspecific neurological symptoms

28. Asperger’s Disorder: Course and Prognosis  given higher level of functioning (due to a lack of basic cognitive and language deficits) their prognosis appears much better than with other PDD’s  Indeed, Wing (1981) has presented case studies of individuals who were able to engage in gainful employment and function in a semi-independent manner.  Obviously, prognosis is intimately related to treatment and management approaches designed to deal with the child's difficulties

29. Asperger’s Disorder: Treatment  no treatment has been shown to modify the basic underlying impairment  treatment should include behavioral approaches focusing on enhancing the child's ability to function in social situations along with an educational program  psychotherapy may be useful as the person becomes aware of the degree to which social skills difficulties make it difficult to function

30. Pervasive Developmental Disorders: Autistic Disorder  Infantile autism was first described by Leo Kanner (l943) in his classic paper " Autistic Disturbances of Affective Contact"  In this article, Kanner highlighted the defining characteristics of 11 children seen in his child psychiatry practice at Johns Hopkins University  His views regarding this disorder have heavily influenced present day views of the disorder, emphasized a number of features

31. Autistic Disorder  Unlike certain other severe disorders of childhood, Kanner assumed autism to have an early onset.  He believed the disorder to be present from the beginning of life, or at least to become obvious during the first year  Indeed, he referred to it as an "inborn disturbance"  He felt that this early onset served to differentiate the disorder from other problems, which at that time, were judged to be manifestations of childhood psychosis

32. Autism: Social Deficits  Autistic children have a primary disturbance in social relationships and an apparent inability to relate to others.  They seem aloof, often oblivious to the presence of others, and are often described as being in a world of their own – “Like in a shell”, “Happiest when left alone”, “Acting as though people aren’t there”, etc.  Early life, infants may fail to show anticipatory posturing when the parent attempts to pick them up from the crib, and the failure of the infant to mold him or herself to the body of the parent

33. Autistic Disorder  Kanner suggested that the outstanding fundamental disorder is “the children’s [in]ability to relate themselves in the ordinary way to people and situations from the beginning of life”  He goes on to note that “this is not as in schizophrenic children or adults, a departure from an initially present relationships - it is not a “withdrawal” from formerly existing participation.”  There is from the start, an “extreme aloneness that whenever possible disregards, ignores, shuts out anything that comes to the child from the outside”.

34. Autistic Disorder  All autistic children show evidence of a severe language disorder  Many remain mute  Those that develop speech typically show unusual features such as echolalia (i.e., the repetition of what someone else has said), pronoun reversal (e.g., referring to oneself as "you" and to others as "I"), or atypical tone (e.g., monotone, inappropriate to content)  Even though some children with autism develop fairly large vocabularies, they usually cannot use speech to communicate with others

35. Autistic Disorder  Kanner noted that although some of his 11 cases developed language, they were no better able to communicate than were those who remained mute  children with autism who speak often have no difficulty naming objects and may have an aptitude for for learning previously constructed verbal materials such as poems, songs, and lists of things  Such learning, however, seems to be without any appreciation of the meaning of these words  There is usually minimal evidence of spontaneous speech that serves a communicative function

36. Autistic Disorder  Kanner and others have noted that autistic children seem to display an desire for the maintenance of sameness  children with autism often get upset when things in their environment are changed ‑ when routines are changed or when toys the child has left in a particular position are moved  This may result in a catastrophic reaction (e.g., tantrum, screaming) lasting until things are returned to their former state  This desire for sameness may lead some children to display a wide range of ritualistic behaviors

37. Autistic Disorder  Other behaviors frequently observed in children with autism: stereotyped behaviors (e.g., mouthing objects, flapping arms and hands, rocking, toe-walking). over responsive to environmental stimuli (e.g., light, sound, foods, clothing) under responsiveness and insensitivity to pain and in associated self ‑ injurious behaviors (e.g,. head banging, scratching, skin picking) In play, interest in parts of objects and little symbolic play  Usually associated with mental retardation  CASE EXAMPLE

38. Autistic Disorder: Prevalence  Although autism is a relatively rare disorder it has been difficult to determine its exact frequency of occurrence.  Varying rates of prevalence are due to: different diagnostic criteria frequently confused with other severe disorders of childhood not all children with autism come to the attention of researchers  Prevalence data from early studies suggested very low rates of occurrence, typically 4 or 5 cases per 10,000 children, and as low as 2 per 10,000 for "classic" cases

39. Autistic Disorder: Prevalence  Gillberg (1990) cites studies, employing differing diagnostic criteria that report figures ranging from 6.6 to 13.6 per 10,000.  The few studies using DSM criteria have reported rates on the order of 10 per 10,000.  The higher rates found in more recent studies has had to do, at least in part, with better detection of autistics among those with severe mental retardation  The disorder is more frequent in boys than in girls, with sex ratios ranging from 2.0 to 1 to 5.7 to 1

40. Autistic Disorder: Prognosis  In reviewing follow ‑ up studies of children with autism DeMyer, et al. (1981) note that as many as 60 to 70 percent live a life of complete or semi ‑ dependence, at home or in an institution  Only about 1-2% achieve normal levels of independence, while others show a borderline level of functioning  A better prognosis seemed to be associated with higher IQ (> 60)

41. Autistic Disorder: Prognosis  In a paper entitled "How far can autistic children go in matters of social adaptation?" Kanner (l973) reported on a follow ‑ up of 96 children with autism seen prior to l953  Although most did not fare as well, 11 of the 96 achieved a “favorable outcome” 3 obtained college degrees 3 went to junior college. 1 other was doing well in college 4 did not go beyond high school or special education

42. Autistic Disorder: Prognosis  Their occupations included accountant, duplicating machine operator, lab technician, bank teller, and several other types of unskilled work  Kanner noted that although these 11 children did show a “favorable outcome”, none seemed to show any interest in the opposite sex or marriage, suggesting continued problems in close relationships  Outcome was unrelated to having received psychiatric treatment  Best predictor seemed to be having useful speech by age 5

43. Etiology of Autism  Views regarding the causes of autism can generally be classified as psychogenic or biogenic in nature  Psychogenic theories characterized the parents of autistic children as cold, aloof, obsessional, refrigerator-like, and in other less than positive terms (see Kanner, l943)  Bettelheim (l967) suggested that negative maternal attitudes are of major importance in the development of this disorder (“refrigerator mother”)

44. Etiology of Autism  research designed to link family variables to autism has provided little support for psychogenic theories  DeMyer, et al. (1981) have noted that, "... Parents of autistic children have been found to display no more signs of mental or emotional illness than parents of children with organic disorders (with or without psychosis).”

45. Etiology of Autism  Growing evidence suggests that autism is a biologically based disorder  This point of view is supported by a wide range of studies that have in one way or another implicated the role of biological factors  The specific biological factors that cause this disorder have not been identified, although several candidates have been identified

46. Etiology of Autism  Autism has been shown to be related to biological problems such as: the development of seizure disorders abnormal EEG's congenital infections (e.g,.rubella) chromosomal abnormalities pregnancy and birth complications neurotransmitter abnormalities abnormal CT scans structural abnormalities of the left hemisphere genetic factors

47. Etiology of Autism  taken together, research findings provide strong support for a biogenic perspective  Indeed, after reviewing much of the literature related to neurobiological factors in autism, Gillberg (1990) has concluded that "autism is now regarded as a behaviorally defined syndrome of neurological impairment with a wide variety of underlying medical etiologies (p. 106)"  Again, the specific biological factors most relevant to the etiology of autism and their specific role remain to be uncovered

48. Autistic Disorder: Treatment  Due to cognitive and social impairments, most children with autism are unlikely to benefit from "talk therapies", although some authors have suggested that individual psychotherapy can be of some value in working with a small number of higher functioning children  While there is presently no "cure" for autism, behavioral approaches have achieved the most obvious success

49. Autistic Disorder: Treatment  Operant procedures (e.g., rewards, shaping) combined with modeling, have been found useful in teaching language, as well as other socially adaptive behaviors, and in decreasing many inappropriate behaviors  bringing about such behavioral changes requires skills which very few clinicians possess and an enormous amount of time

50. Autistic Disorder: Treatment  Lovaas (1987) conducted intensive, long-term, treatment program that focused on imitation, language development, the expression of appropriate emotions, and appropriate play behaviors  Of the 19 children participating in the program, it was possible to mainstream 8, and 7 were described as being indistinguishable from typical children  While the degree to which these children did in fact approach normality has been questioned, the results clearly highlight the potential contribution of behavioral treatments

51. Autistic Disorder: Treatment  Treatment and Education of Autistic and Related Communication Handicapped Children (TEACCH)  N.C. program for individuals with autism Includes education, communication, and behavior strategies for working with children with autism  Focuses on the building upon the strengths of children rather than just improving deficits

52. Autistic Disorder: Treatment  TEACCH structured teaching: Use of schedules Classroom structure (e.g., setting up clear areas for work/play, minimizing distractions) Use of visual cues (e.g., polaroids, color-coded tasks) Alternate enjoyable and less enjoyable activities Use minimal language or gestures  www.teacch.com

53. Autistic Disorder: Treatment  Some drugs may have beneficial effects for children with autism: Haloperidol has been shown to improve the learning ability of children with autism and make them more responsive to special education and behavior modification Fenfluramine, which reduces levels of serotonin in the blood, has shown some promise in improving social and intellectual functioning  While behavior therapy and drug treatments have both shown some promise in treating autism, there is research evidence to suggest that treatment should not be an either/or proposition

54. Living with Autistic Disorder: Dr. Temple Grandin  On cognitive processes: “Thinking in language and words is alien to me. I think totally in pictures. It is like playing different tapes in a video cassette recorder in my imagination. I used to think that everybody thought in pictures until I questioned many different people about their thinking processes. I have conducted an informal little cognitive test on many people. They are asked to access their memory of church steeples or cats. An object that is not in the person's immediate surroundings should be used for this visualization procedure. When I do this, I see in my imagination a series of "videos" of different churches or cats I have seen or known. Many "normal" people will see a visual image of a cat, but it is a sort of generalized generic cat image. They usually don't see a series of vivid cat or church "videos" unless they are an artist, parent of an autistic child, or an engineer. My "cat" concept consists of a series of "videos" of cats I have known. There is no generalized cat. If I keep thinking about cats or churches I can manipulate the "video" images. I can put snow on the church roof and imagine what the church grounds look like during the different seasons.”

55. Living with Autistic Disorder: Dr. Temple Grandin  “For me, there is no language based information in my memory. To access spoken information, I replay a "video" of the person talking.... To retrieve facts, I have to read them off a visualized page of a book or "replay the video" of some previous event. This method of thinking is slower. It takes time to "play" the videotape in my imagination.”

56. Living with Autistic Disorder: Dr. Temple Grandin  On communicating: “I screamed because it was the only way I could communicate.... I had the words I wanted to say in my mind, but I just could not get them out; it was like a big stutter. When my mother wanted me to do something, I often screamed. If something bothered me, I screamed. This was the only way I could express my displeasure. If I did not want to wear a hat, the only way I could communicate my desire not to wear the hat was to throw it on the floor and scream. Being unable to talk was utter frustration.”

57. Living with Autism: Temple Grandin, Ph.D.  http://www.npr.org/templates/story/story.php?storyId=4278538 http://www.npr.org/templates/story/story.php?storyId=4278538  Emergence: Labeled Autistic