Asperger’s Syndrome and Pervasive Developmental Disorders

Joseph P. Damore JR., MD

INTRODUCTION

Pediatric primary care providers are in the enviable position of watching children grow and develop from infancy through childhood, adolescence, and often beyond. Perhaps no other specialty affords clinicians with the opportunity to witness such unparalleled change. Those who work in pediatric primary care also observe many children of various ages and developmental stages. As a result, they become acquainted early on with the broad range of normal variations that characterize such development.

Often in such practice, providers will encounter a child who presents with behavioral or emotional symptoms that are outside the range of normal development. It is not uncommon for the pediatric practitioner to be the first to diagnose children with attentional deficits, hyperactivity, or other difficulties, such as mood disorders and anxiety. Less frequently, providers may encounter and appropriately diagnose a child with autism. Usually, such diagnoses are made in the presence of clear-cut symptoms or during particularly severe decompensations in behavior. Often, however, children with less profound behavioral disorders “fall through the cracks” of pediatric watchfulness. Busy primary care providers may overlook these children, who often present with odd mannerisms, deficits in social skills, or remarkable facility with language or memory. This is particularly common when such characteristics do not create problems at home or school. Even when problems occur, these children are often misdiagnosed and treated for the component parts of their syndrome (Perry, 1998).

Though the category of pervasive developmental disorders includes such problems as autism, Rett’s disorder, and childhood disintegrative disorder, this chapter focuses specifically on the presentation, diagnostic criteria, and treatment of children with Asperger’s syndrome and pervasive developmental disorder not otherwise specified (PDD NOS). The reasons for this are twofold. First, discussion of all the pervasive developmental disorders is beyond the scope of this text. Secondly, the sequellae of disorders such as autism and Rett’s disorder are so profound that they usually are easily identified at an early age and thus referred early for treatment. Cases of Asperger’s syndrome and PDD NOS, however, are often misdiagnosed or undiagnosed. Though rare, they are more likely to be seen in pediatric primary care practice. This chapter is not an exhaustive discussion of these syndromes; rather, it will enable readers to become familiar with the presentation and treatment of these disorders.

PATHOLOGY

Asperger’s Syndrome

In 1944, the Viennese child psychiatrist Hans Asperger, while studying the capacity for group formation in children, first described a cohort of children who demonstrated social isolation coupled with odd and unusual behavior (Szatmari, Tuff, Finlayson, & Bartolucci, 1990). This condition was first described as autistic psychopathy, terminology that led to misunderstanding because of the similarity of the term psychopathy to sociopathy. For this reason, the disorder became known as Asperger’s syndrome. This disorder describes children with eccentricities of speech, nonverbal communication, social interaction, motor coordination, and skills and interests. Most of these children demonstrate a strong interest in repetitive activities and a strong desire to avoid changes in their environment. Children with Asperger’s syndrome usually acquire the capacity for language at the same age as unaffected children, though the content of the speech tends to be repetitive, pedantic, and loquacious (Wing, 1981). Similarly, they demonstrate difficulty in initiating and maintaining two-way conversation. Children with Asperger’s syndrome exhibit a paucity of emotional expression and facial gestures. Quite commonly, these youngsters become intensely interested in unusual subjects, such as floor plans for buildings, fish, or machinery (Szatmari et al., 1990; Howlin & Asgharian, 1999).

They also tend to excel at board games. These children are reported to be clumsy and uncoordinated and often demonstrate problems with drawing and writing. Their posture and gait may be described as odd (Wing, 1981).

Wing (1981) suggested that Asperger’s syndrome should be considered part of an autistic continuum (ie, a mild variant of autism in relatively intelligent children). Though in agreement with the notion that Asperger’s syndrome and autism share features, other authors later proposed that those with Asperger’s syndrome demonstrate no cognitive or language delays (Tantum, 1988).

Baron-Cohen and Wheelwright (1999) examined the behavior of children with Asperger’s syndrome. In surveying 92 parents of such children, they found that the obsessive behaviors of Asperger’s syndrome are not in fact random acts but are organized around the notion of what they term “folk physics,” meaning a fascination with how things work. This finding is in contrast with what the authors term “folk psychology,” which means a curiosity about how people work.

Few studies have been able to demonstrate a link between Asperger’s syndrome and pathologic conditions, though associations with neurologic disorders and amino-aciduria have been reported (Tantum, 1988). Sobanski, Marcus, Hennighausen, Hebebrand, and Schmidt (1999) describe disturbed eating behavior and, hence, significantly decreased body mass index (BMI) in male children with Asperger’s syndrome compared with healthy control children. Of 36 subjects, 13 children had BMIs below the 10th percentile, while three had BMIs under the third percentile. Their findings suggest that providers should evaluate any child with Asperger’s syndrome for eating problems as well.

Some studies suggest an association between Asperger’s syndrome and lesions of the right cerebral hemisphere (Tantum, 1988). Interestingly, Wing (1981) observed that approximately half of children affected with this disorder had a history of prenatal, postnatal, or perinatal complications, though other investigators did not observe such findings in other samples (Szatmari, Bremner, & Nagy, 1989). Though several studies suggest a genetic etiology to the disorder, no firm genetic relationship has been established (Gillberg, 1989).

Pervasive Developmental Disorder Not Otherwise Specified

Unlike Asperger’s Syndrome, little is known about PDD NOS as a distinct clinical entity. Various euphemisms for the disorder include atypical autism and high functioning autism, because most children with PDD NOS demonstrate some or most characteristics seen in children with autism (Howlin & Asgharian, 1999).

In a study of 390 children younger than 5 years conducted by Dahl, Cohen, and Provence (1986), 24 children demonstrated odd behavior, eccentric thinking, and impairments in social interaction. In a similar study, Levine and Demb (1987) reported that of 18 preschool children diagnosed with PDD NOS, only about one third had an IQ in the normal or borderline range. More than two thirds of the children in Levine’s sample had a history of abnormal speech acquisition. More than 90% of these children had abnormal speech characterized by echolalia, abnormalities of prosody, and pronoun reversal. In addition, Levine and Demb noted these children to be markedly provocative or clinging with their parents, and almost all demonstrated ritualistic behaviors. Other authors have described the consistency of these findings over time as well (Baron-Cohen & Wheelwright, 1999; Berument, Rutter, Lord, Pickles, & Bailey, 1999; Coplan, 2000).

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