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Comprehension of indirect requests in adults with autistic disorders and mental retardation.

Eight adults with autistic disorders and 8 IQ-matched, mentally retarded (MR) subjects were given a task involving the comprehension of indirect requests. In one condition, requests were prefaced by instructions making their illocutionary force clear (I'm going to tell you to color some circles). In a second condition, the same requests were presented in an unstructured conversation. Although the performance of the MR subjects was better in both conditions, both groups performed similarly to normal 4- to 6-year-olds. In the unstructured condition, however, the autistic group's performance was significantly depressed, especially on sentences requiring greater semantic processing. The implications of these findings for understanding language and social deficits in autistic and similar individuals are discussed.

Adult↗

Bilateral medial prefrontal and temporal neocortical hypometabolism in children with epilepsy and aggression.

PURPOSE: To identify brain regions with abnormal function in children with intractable partial epilepsy and aggressive behavior by using 2-deoxy-2-[18F]fluoro-D-glucose (FDG) positron emission tomography (PET). METHODS: Six children (mean age, 9.9 years) with intractable partial epilepsy and aggressive behavior underwent detailed psychodevelopmental assessment and FDG-PET scanning. The objective technique of statistical parametric mapping (SPM) was applied to define focal abnormalities of glucose metabolism, and compared those with those of a group of normal adult subjects (n = 17) as well as age-matched children with epilepsy with similar seizure characteristics but without aggression (n = 7). The findings were analyzed further by using a region-of-interest (ROI) approach. RESULTS: The aggressive children all showed developmental delay, and four of them also manifested autistic symptoms. SPM analysis demonstrated extensive glucose hypometabolism in the aggressive group bilaterally in the temporal and prefrontal cortex compared with that in normal adult controls. A focal area of medial prefrontal glucose hypometabolism was defined in the aggressive children as compared with the nonaggressive pediatric group with SPM, whereas ROI comparison of these groups confirmed prefrontal hypometabolism and also showed glucose hypometabolism of the temporal neocortex in the aggressive children. Severity of aggression correlated inversely with glucose metabolism of the left temporal as well as bilateral medial prefrontal cortex. CONCLUSIONS: Bilateral prefrontal and temporal neocortical brain glucose hypometabolism in children with epilepsy and aggressive behavior may indicate a widespread dysfunction of cortical regions, which normally exert an inhibitory effect on subcortical aggressive impulses. PET studies may be used to elucidate the neurobiologic basis of aggressive behavior in children.

Aggression↗

Repetitive self-injurious behavior: a neuropsychiatric perspective and review of pharmacologic treatments.

The phenomenology, pathophysiology, and psychopharmacology of repetitive self-injurious behavior (rSIB) are reviewed. Although numerous neurotransmitter systems are thought to be involved in the initiation and maintenance of rSIB, the majority of clinical studies attend to the role of serotonin or endogenous opioids. This focus has emerged from a conceptualization of rSIB as a problem of impulse control (primarily mediated by serotonin) and/or as a maladaptive pain-related behavior (ultimately mediated by opioids). A developmental perspective of rSIB is emphasized, highlighting the biased prevalence of rSIB among patients with mental retardation and severe personality disorders and the significance of critical developmental events leading to pathology in "pedagogical" neural circuits. A novel typology is offered in an effort to better match interventions with rSIB subtypes. Achievement of this ultimate goal however, must await further research.

Adult↗

Early clinical signs in the Rett disorder.

This review of the period from birth until the end of regression in classic Rett syndrome (RS) is based on personal experience of more than 600 cases over 12 years including video material on 42 cases showing behaviour before regression. A period of undoubted developmental progress followed by loss of skill is apt to persuade the physician that a fresh toxic or infective insult has afflicted a normal child but close scrutiny of classic Rett syndrome cases indicates that the cognitive and motor problems of the disorder are detectable from birth, that a developmental ceiling limits progress and that the timing and nature of the regression event and subsequent behaviour of the child indicate inherently defective central receptive processing with a highly specific profile. A model illustrates how the characteristic disturbances may express incompetent higher control. The robust mid infancy level skills of the Rett child and woman deserve careful analysis and offer potential for therapy. Metabolic and immune sequelae may occur as the developmental defect becomes manifest and such cascade events demand careful evaluation and offer further opportunities for intervention.

Adolescent↗

A population-based study of measles, mumps, and rubella vaccination and autism.

BACKGROUND: It has been suggested that vaccination against measles, mumps, and rubella (MMR) is a cause of autism. METHODS: We conducted a retrospective cohort study of all children born in Denmark from January 1991 through December 1998. The cohort was selected on the basis of data from the Danish Civil Registration System, which assigns a unique identification number to every live-born infant and new resident in Denmark. MMR-vaccination status was obtained from the Danish National Board of Health. Information on the children's autism status was obtained from the Danish Psychiatric Central Register, which contains information on all diagnoses received by patients in psychiatric hospitals and outpatient clinics in Denmark. We obtained information on potential confounders from the Danish Medical Birth Registry, the National Hospital Registry, and Statistics Denmark. RESULTS: Of the 537,303 children in the cohort (representing 2,129,864 person-years), 440,655 (82.0 percent) had received the MMR vaccine. We identified 316 children with a diagnosis of autistic disorder and 422 with a diagnosis of other autistic-spectrum disorders. After adjustment for potential confounders, the relative risk of autistic disorder in the group of vaccinated children, as compared with the unvaccinated group, was 0.92 (95 percent confidence interval, 0.68 to 1.24), and the relative risk of another autistic-spectrum disorder was 0.83 (95 percent confidence interval, 0.65 to 1.07). There was no association between the age at the time of vaccination, the time since vaccination, or the date of vaccination and the development of autistic disorder. CONCLUSIONS: This study provides strong evidence against the hypothesis that MMR vaccination causes autism.

Autistic Disorder↗

Myoclonus during prolonged treatment with sertraline in an adolescent patient.

A 15-year-old female adolescent with depression developed myoclonus after uninterrupted treatment with sertraline over 6 years. She was also receiving methylphenidate. Withdrawal of sertraline and continuation of methylphenidate did not result in any improvement. Treatment with valproic acid resulted in improvement of the movement disorder. This report suggests that myoclonus may be a side effect of sertraline in some adolescents. Further, we hypothesized that extended treatment over several years, young age, and a compromised central nervous system due to underlying disorders may be risk factors for the development of this side effect.

Adolescent↗

Are patients with social developmental disorders prosopagnosic? Perceptual heterogeneity in the Asperger and socio-emotional processing disorders.

It has been hypothesized that social developmental disorders (SDD) like autism, Asperger's disorder and the social-emotional processing disorder may be associated with prosopagnosic-like deficits in face recognition. We studied the ability to recognize famous faces in 24 adults with a variety of SDD diagnoses. We also measured their ability to discriminate changes in internal facial configuration, a perceptual function that is important in face recognition, and their imagery for famous faces, an index of their facial memory stores. We contrasted their performance with both healthy subjects and prosopagnosic patients. We also performed a cluster analysis of the SDD patients. One group of eight SDD subjects performed normally on all tests of face perception and recognition. The other 16 subjects were impaired in recognition, though most were better than prosopagnosic patients. One impaired SDD subgroup had poor perception of facial structure but relatively preserved imagery, resembling prosopagnosic patients with medial occipitotemporal lesions. Another subgroup had better perception than imagery, resembling one prosopagnosic with bilateral anterior temporal lesions. Overall, SDD subgroup membership by face recognition did not correlate with a particular SDD diagnosis or subjective ratings of social impairment. We conclude that the social disturbance in SDD does not invariably lead to impaired face recognition. Abnormal face recognition in some SDD subjects is related to impaired perception of facial structure in a manner suggestive of occipitotemporal dysfunction. Heterogeneity in the perceptual processing of faces may imply pathogenetic heterogeneity, with important implications for genetic and rehabilitative studies of SDD.

Adolescent↗

Epigenetics of autism spectrum disorders.

The autism spectrum disorders (ASD) comprise a complex group of behaviorally related disorders that are primarily genetic in origin. Involvement of epigenetic regulatory mechanisms in the pathogenesis of ASD has been suggested by the occurrence of ASD in patients with disorders arising from epigenetic mutations (fragile X syndrome) or that involve key epigenetic regulatory factors (Rett syndrome). Moreover, the most common recurrent cytogenetic abnormalities in ASD involve maternally derived duplications of the imprinted domain on chromosome 15q11-13. Thus, parent of origin effects on sharing and linkage to imprinted regions on chromosomes 15q and 7q suggest that these regions warrant specific examination from an epigenetic perspective, particularly because epigenetic modifications do not change the primary genomic sequence, allowing risk epialleles to evade detection using standard screening strategies. This review examines the potential role of epigenetic factors in the etiology of ASD.

Autistic Disorder↗

Behavior therapy with children and adolescents: a twenty-year overview.

A twenty-year overview of behavior therapy with children and adolescents is presented. The various techniques and their application to relevant major DSM-III-R categories are critically discussed. It is concluded that behavior therapy has made great progress and has proven applications in child and adolescent disorders but that its precise roles, comparative efficacy, and complementarity to other forms of psychotherapy and other treatments remain to be demonstrated. Much uncertainty stems from the relatively poor state of research in other forms of psychotherapy.

Adolescent↗

Asperger's syndrome and autism: neurocognitive aspects.

The objectives of this study were to see: (1) whether children with Asperger's Syndrome (AS) have similar neurocognitive deficits compared to nonretarded, or high-functioning autistic (HFA) children; and (2) whether the essential cognitive deficit among these children is in language or abstract problem solving. Subjects with AS, HFA, and a control group of socially impaired child psychiatric outpatient controls (OPC) were compared on a battery of neuropsychological tests. The results indicated that the AS and HFA groups differed little but that large differences from the OPC were observed on all tests. When the AS and HFA with FSIQ above 85 were compared to the OPC, outstanding deficits on motor coordination, language comprehension, and facial recognition were observed. Finally, some evidence is presented to suggest that the pattern of deficits of AS and HFA subjects varied by developmental level. The implications of these results for a neurological theory of autism are discussed.

Adolescent↗

ADHD and PDD.

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Attention Deficit Disorder with Hyperactivity↗