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Developmental language disorders.

Developmental language disorders are among the most common disorders of childhood referred to the pediatric neurologist. This article presents an overview of developmental language disorders, a discussion of the definition of developmental language disorders, potential causal factors, and a description of possible subtypes of language disorders in children. The article concludes with a review of the pediatric neurologist's role in developmental language disorders and recommendations for assessment and management.

Child↗

Clomipramine in adults with pervasive developmental disorders: a prospective open-label investigation.

The purpose of this investigation was to determine the short-term efficacy and tolerability of clomipramine in a consecutive series of adults with pervasive developmental disorders (PDDs). Thirty-five adults with PDDs (DSM-IV), 16 of whom were nonverbal, entered a 12-week prospective open-label trial of clomipramine. The initial sample included 18 patients with autistic disorder, 6 patients with Asperger's disorder, and 11 patients with pervasive developmental disorder not otherwise specified (PDDNOS). Behavioral ratings were obtained at baseline and after 4, 8, and 12 weeks of clomipramine. Eighteen (55%) of the 33 patients who completed the trial were categorized as treatment responders based on scores of "much improved" or "very much improved" on the Clinical Global Impression (CGI) global improvement item (p < 0.001). Ten (63%) of 16 patients with autistic disorder, 2 (33%) of 6 patients with Asperger's disorder, and 6 (55%) of 11 patients with PDDNOS were considered responders to clomipramine treatment. In those 18 patients, clomipramine significantly reduced total repetitive thoughts and behavior (p < 0.001) and also aggression (p < 0.001), and improved some aspects of social relatedness, such as eye contact and verbal responsiveness (p < 0.001). Change in these specific symptom clusters over time was not related to DSM-IV subtype of PDD. The level of autistic behavior, as measured by the Autism Behavior Checklist (ABC) score, and full-scale intelligence quotient (IQ) were not significantly associated with global treatment response. Whereas clomipramine was well tolerated by most patients, 13 had clinically significant adverse effects. Three patients had seizures during clomipramine treatment, including 2 who had prior seizure disorders and were taking anticonvulsants. Of the 32 patients who had no history of prior seizures, only 1 had a seizure during clomipramine treatment. There were no adverse cardiovascular or extrapyramidal effects. All responders continued on clomipramine after completion of the study. The results of this open-label trial suggest that clomipramine may be an effective drug for reducing repetitive thoughts and actions and aggressive behavior and for improving some elements of social behavior, such as eye contact and verbal responsivity in adults with PDDs. Careful monitoring of adverse effects, particularly seizures, is warranted. Although an electroencephalogram (EEG) is not mandatory in patients with PDD prior to clomipramine treatment, we recommend that patients with PDD and a history of seizures be treated initially with a selective serotonin uptake inhibitor rather than with clomipramine. The findings of this study require replication in a double-blind placebo-controlled investigation before definitive statements of efficacy and tolerability can be made.

Adolescent↗

[Evaluation of children with developmental disorders by evoked potentials and event-related potentials].

INTRODUCTION AND DEVELOPMENT: Evoked potentials evaluation of children with central nervous system developmental disorders o dysfunctions is better than clinical and imaging assessment for determination of: 1) conceptional age, 2) auditory and visual threshold, 3) integrity of sensory pathways, 4) neurologic motor outcome, and 5) course and evaluation of treatment of neurometabolic disorders. CONCLUSIONS: However, it is not the same for predicting or making the diagnosis of the majority of primary developmental disorders (autism, receptive and expressive language disorders, learning disability, attention deficit disorders and Gilles de la Tourette syndrome.

Asphyxia Neonatorum↗

Predicting language outcome in infants with autism and pervasive developmental disorder.

BACKGROUND: To examine longitudinal associations between diagnosis, joint attention, play and imitation abilities and language outcome in infants with autism and pervasive developmental disorder. METHODS AND PROCEDURES: Experimental measures of joint attention, play and imitation were conducted with a sample of infants with autism spectrum disorder at age 20 months. Language outcome was assessed at age 42 months. A within-group longitudinal correlational design was adopted. OUTCOMES AND RESULTS: Language at 42 months was higher for children with a diagnosis of pervasive developmental disorder than for children with a diagnosis of autism. Language at follow-up was also positively associated with performance on experimental measures of joint attention and imitation, but not with performance on experimental measures of play and 'goal detection' at 20 months, nor with a non-verbal intelligence quotient, although these associations were not examined independent of diagnosis. However, floor effects on the measure of play at 20 months and the small sample size limit the conclusions that can be drawn. CONCLUSIONS: Individual differences in infant social-communication abilities as well as diagnosis may predict language outcome in preschoolers with autism spectrum disorders. Attention should be directed at assessing these skills in 2- and 3-year-old children referred for a diagnosis of autism spectrum disorder. Imitation and joint attention abilities may be important targets for early intervention.

Attention↗

Case study: corticosteroid treatment of language regression in pervasive developmental disorder.

The authors describe a child whose language and behavior regressed at 22 months and in whom pervasive developmental disorder was later diagnosed. At 6 years, he displayed a profound receptive-expressive aphasia accompanied by behavioral disturbances characterized by hyperactivity, impaired social interactions, tantrums, gestural stereotypies, and echolalia. A single-photon emission computed tomography scan and steady-state auditory evoked potentials suggested bitemporal and left frontal pathophysiology. The overall profile resembled Landau-Kleffner syndrome, but no electroencephalographic disturbance was evident. Corticosteroid treatment resulted in amelioration of language abilities and behavior. These findings suggest that the factors underlying language regression in pervasive developmental disorder can, in special circumstances, be amenable to pharmacological treatment.

Adrenal Cortex Hormones↗

Insecure and disorganised attachment in children with a pervasive developmental disorder: relationship with social interaction and heart rate.

This study on children with a Pervasive Developmental Disorder (PDD; N = 32), children with developmental language disorder (N = 22), and normally developing children (N = 28) sought to answer questions concerning attachment and autistic behaviour. We could replicate the finding that children with a PDD are able to develop secure attachment relationships to their primary caregiver. Children with PDD who had an insecure attachment showed fewer social initiatives and responses than children with PDD who had a secure attachment, even when the insecurely and securely attached PDD children were matched on chronological and mental age. Children with both a PDD and mental retardation were more often classified as disorganised. Three findings suggested that a disorganised attachment does not merely reflect the presence of "autistic" behaviour: (1) children with PDD did not reveal higher rates of a disorganised attachment than matched comparison children; (2) having a PDD diagnosis and having a disorganised attachment were found to be associated with opposite effects on an ethological measure of level of behavioural organisation; and (3) a disorganised attachment but not a PDD diagnosis was associated with an increase in heart rate during parting with the caregiver and a decrease in heart rate during reunion.

Child↗

Genetic and neurological evaluation in a sample of individuals with pervasive developmental disorders.

With the aim of analyzing which complementary tests are relevant in the diagnostic evaluation of individuals with pervasive developmental disorders, a protocol of clinical and laboratory evaluation was applied in 103 outpatients. The protocol included chromosomal analysis, screening for inborn errors of metabolism, cytogenetic and molecular study of the FRAXA, FRAXE, and FRAXF mutations, EEG, SPECT, and magnetic resonance imaging study. Eighty-four subjects concluded the complementary tests and were classified either as having autism, atypical autism or Asperger syndrome according to the DSM-IV criteria. Sixteen individuals, all bellonging to the two autistic groups, presented genetic or enviromental factors that may have lead to the behavioral disorders, showing the importance of diagnostic evaluation in this group of conditions. Neuroimaging and EEG findings were non-specific and occurred in similar proportion among the groups, being considered of relative low significance in the diagnostic evaluation of individuals with pervasive developmental disorders.

Adolescent↗

Are pervasive developmental disorders and attention-deficit/hyperactivity disorder distinct disorders?

We studied the relationship between patients with attention-deficit/hyperactivity disorder (ADHD) and those with pervasive developmental disorders (PDD), using the High-Functioning Autism Spectrum Screening Questionnaire (ASSQ) and ADHD Rating Scale-IV. The ASSQ scores of the PDD group and the ADHD group were significantly higher than the control group. Furthermore, the PDD group scored higher than the ADHD group. Both groups also showed higher scores than the control group in all three domains, that is, restricted and repetitive behavior, social interaction, and communication problem. The PDD and the ADHD group showed no significant difference in the domains of communication problem, and restricted and repetitive behavior. The PDD group had a higher score than the ADHD group only in the social interaction domain. In total score, inattention score, and hyperactivity/impulsivity score on the ADHD Rating Scale-IV, both groups were significantly higher than the control group. Between the ADHD and the PDD groups, there was no significant difference in the three scores. The patients with strictly diagnosed ADHD had many PDD-related symptoms, and the patients with PDD had many ADHD-related symptoms. It therefore seems difficult to make a distinction between ADHD and PDD by using the present diagnostic criteria in the DSM-IV. We should evaluate each patient in terms of both sets of criteria.

Adolescent↗

Multiple complex developmental disorder delineated from PDD-NOS.

The objective of this study was to identify behavioral differences between children with multiple complex developmental disorder (MCDD) and those with pervasive developmental disorder-not otherwise specified (PDD-NOS). Twenty-five children (6-12 years) with MCDD and 86 children with PDD-NOS were compared with respect to psychiatric co-morbidity, psychotic thought problems and social contact problems using the child behavior checklist/4-18, the Dutch version of the diagnostic interview schedule for children-Version IV, the child and adolescent functional assessment scale, and the autism diagnostic observation schedule-generic. MCDD was associated with anxiety disorders, disruptive behavior, and psychotic thought problems. PDD-NOS was associated with deficits in social contact. MCDD differs from autistic disorder, and can also be delineated from PDD-NOS.

Anxiety Disorders↗

Early-onset schizophrenia as a progressive-deteriorating developmental disorder: evidence from child psychiatry.

The developmental perspective as reflected by investigations of childhood and early-onset schizophrenia has become a major research area during recent years and contributed much to the understanding of schizophrenia at all ages. This paper reviews clinical features, neurobiological and neuropsychological findings in childhood and adolescent onset schizophrenia including some results of studies of the author on age at onset, premorbid symptoms, treatment and course. Childhood-onset schizophrenia is a rare disorder with a prevalence of one child in 10,000 before the age of 12 and a remarkable increase around puberty and early adolescence. Developmental events and precursors of schizophrenia cover a wide range of dysfunctions and disturbances including elevated rates of soft neurological signs and birth complications, slow habituation and high baseline autonomic activity, high rate of developmental disorders of speech and/or language and overall and specific cognitive deficits. Brain morphological studies and intelligence testing as well as investigations of the course provide evidence of deterioration. Therefore, early-onset schizophrenia can be understood as a progressive-deteriorating developmental disorder.

Adolescent↗

Treatment strategies for a case of concurrent pervasive developmental disorder and cerebral gigantism.

A 4-year-old girl presented for a psychiatric evaluation with reported episodes of clumsiness, aggressiveness, lack of relatedness, and temper tantrums. Her evaluation disclosed multiple developmental deficits, including cognitive, affective, and social lags. Given her individual history and her specific constellation of symptoms as well as a familial history indicative of developmental impairment, the child was diagnosed as having pervasive developmental disorder. This diagnosis reflects the expanded nosology for autism, as specified in DSM-III-R. Subsequently, after a genetic evaluation, the child was found to have an organic central nervous system deficit in the form of cerebral gigantism, a neural disorder. The recognition of an organic impairment in this case contributed to an understanding of the pervasive developmental disorder symptomatology and facilitated the formulation of an appropriate therapeutic protocol that addressed both developmental and neurological components.

Brain Diseases↗

Teaching toilet skills in a public school setting to a child with pervasive developmental disorder.

An 8-year old boy with Pervasive Developmental Disorder (PDD) was taught toileting skills within a public elementary school. During baseline, he never urinated successfully in the toilet and wore a disposable diaper throughout the day. The training program included scheduling a toileting opportunity at a time that increased the likelihood of urination and providing positive reinforcement when voiding in the toilet occurred. Toileting skills were established rapidly and were maintained when another bathroom visit was added to the toileting schedule, primary reinforcement was eliminated, and the boy no longer wore a disposable diaper. Issues related to behavioral support programming within inclusive educational settings are discussed.

Behavior Therapy↗

Hemizygosity at the elastin locus in a developmental disorder, Williams syndrome.

Williams syndrome (WS) is a developmental disorder affecting connective tissue and the central nervous system. A common feature of WS, supravalvular aortic stenosis, is also a distinct autosomal dominant disorder caused by mutations in the elastin gene. In this study, we identified hemizygosity at the elastin locus using genetic analyses in four familial and five sporadic cases of WS. Fluorescent in situ hybridization and quantitative Southern analyses confirmed these findings, demonstrating inherited and de novo deletions of the elastin gene. These data indicate that deletions involving one elastin allele cause WS and implicate elastin hemizygosity in the pathogenesis of the disease.

Adult↗

Occupational therapy with children with pervasive developmental disorders.

OBJECTIVE: Although the prevalence of children with pervasive developmental disorders (PDD) has increased, empirical data about the role and practices of occupational therapists have not been reported in the literature. This descriptive study investigated the practice of occupational therapists with children with PDD. METHOD: A survey was mailed to 500 occupational therapists in the Sensory Integration Special Interest Section or School System Special Interest Section of the American Occupational Therapy Association in eastern and midwestern United States. The valid return rate was 58% (292 respondents). The survey used Likert scale items to measure frequency of performance problems observed in children with PDD, performance areas addressed in intervention, perceived improvement in performance, and frequency of use of and competency in intervention approaches. RESULTS: The respondents primarily worked in schools and reported that in the past 5 years they had served an increasing number of children with PDD. Most respondents provided direct services and appeared to use holistic approaches in which they addressed multiple performance domains. They applied sensory integration and environmental modification approaches most frequently and believed that they were most competent in using these approaches. Respondents who reported more frequent use of and more competence in sensory integration approaches perceived more improvement in children's sensory processing. Respondents who reported more frequent use of and more competence in child-centered play perceived more improvement in children's sensory integration and play skills.

Adult↗

When is development disordered? Developmental psychopathology and the harmful dysfunction analysis of mental disorder.

One goal of developmental psychopathology is to understand the origins and course of mental disorders. I argue that pursuit of this goal requires a valid conceptual understanding of disorder and that this understanding can be provided by the "harmful dysfunction" analysis of the concept of disorder. The harmful dysfunction analysis holds that a disorder is a condition that is both harmful according to social values and caused by an internal dysfunction, that is, by a failure of an internal mechanism to perform a function for which it was naturally selected. This analysis explains why many of the distinctive features of developmental psychopathology are appropriate to the study of disorder. It is argued that the harmful dysfunction analysis is a necessary supplement to other proposed criteria for disorder, such as developmental deviation or predictive validity.

Adolescent↗

Use of risperidone in pervasive developmental disorders: a case series.

A series of 14 children and adolescents (ages 9-17 years, 10 males) were treated with risperidone for pervasive developmental disorder. The rationale for using an atypical neuroleptic agent is based on its ability to target both positive and negative symptoms of schizophrenia. It was postulated that symptoms similar to the positive and negative symptoms of schizophrenia may be observed in the pervasive developmental disorders and might respond favorably to risperidone. Twelve of the 14 youths had been treated previously with several psychotropic drugs, often concurrently. Risperidone was initiated at a starting dose of 0.25 mg twice daily and increased in 0.25 mg/day increments every 5-7 days. Optimal dosages ranged from 0.75 to 1.5 mg daily in divided doses. Thirteen of the 14 youths appeared to benefit from risperidone. Improvement in functionality on the Children's Global Assessment Scale was demonstrated in 13 of 14 cases. Disruptive behaviors, when present, markedly decreased on risperidone. Ten patients showed a marked reduction in agitation and anxiety. Social awareness improved markedly in 10 patients, moderately in 3, and only slightly in 1. All but 1 patient demonstrated a lessening in obsessional behaviors. Effects on attention were uniformly positive. Side effects were minimal at the dosages used in this study; 5 patients had initial sedation. Neither extrapyramidal side effects nor agitation was observed in any case. Ten of 14 youths could be managed with risperidone monotherapy. During the follow-up period (mean 7 months), none of the patients experienced a major relapse while taking risperidone. Positive and negative symptoms, as typically characterized in schizophrenia, were both found to improve equally well with risperidone treatment. Based on these findings, a prospective clinical trial with a randomized controlled design is warranted.

Adolescent↗

Long-term group psychotherapy for children with pervasive developmental disorders: evidence for group development.

This article highlights the clinical progress of three long-term psychotherapy groups for children with pervasive developmental disorders. Unique aspects of working with such children are addressed as well as their movement through the phases of group development. Clinical observations suggested that these groups did in fact pass through observable phases of cohesion, differentiation, and intimacy. Further, these children demonstrated noticeable social development both inside and outside the groups. They identified strongly with their groups, and after the settling-in period, there was remarkably little resistance to participation, especially given the children's tendencies for social avoidance. Vignettes are provided to illustrate both group developmental phases and noteworthy psychological experiences of the clinical process with children who have been diagnosed with pervasive developmental disorders.

Adolescent↗

Familial factors influence level of functioning in pervasive developmental disorder.

OBJECTIVE: To determine whether siblings with pervasive developmental disorders (PDD) tend to have the same type and number of PDD symptoms or a similar level of functioning. METHOD: The familial correlations for PDD subtype, symptom totals, adaptive behaviors, and nonverbal IQ were calculated for 94 children with PDD from 46 families. RESULTS: On variables measuring PDD symptoms, only impairments in nonverbal communication and verbal/nonverbal status tended to run true within families. There was no familial aggregation of PDD subtype. In contrast, measures of nonverbal IQ and adaptive behaviors in socialization and communication showed a moderate degree of familial resemblance. The degree of familial resemblance did not change if the analysis was restricted only to those families in which both affected children met criteria for autism. CONCLUSION: Insofar as the familial resemblance seen in PDD is due to genetic factors, these data provide some evidence that higher- and lower-functioning PDD children may arise from separate genetic mechanisms. Current gene-mapping studies of PDD may need to take this evidence of genetic heterogeneity into account.

Activities of Daily Living↗