NAPDD patients exhibit altered electrophoretic mobility of cytosolic 5' nucleotidase.
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A normally developed and healthy 6-year-old girl suffered the onset of epilepsy with generalized tonic-clonic seizures and atypical absences. Initially the EEG showed epileptiform activity over the temporal and parietal regions, later there were episodes of bilateral synchronous spike-wave activity with a frequency of 1.5-2.5 Hz. After a few months, deterioration of cognitive and behavioural functions appeared and gradually increased with the development of a full-blown disintegrative psychosis that went on for several months. Sleep EEG recordings showed the characteristic abnormality described as continuous spikes and waves during slow sleep. Later there was a remarkable improvement of neuropsychiatric functions but a second outbreak of psychosis seems to have left the girl, who is now 9 years of age, with severe mental impairment.
This follow-up study reports data on 18 children fulfilling the ICD-10 criteria for childhood autism (n = 9) and Asperger syndrome (n = 9). In connection with the present study the original child psychiatric records were reassessed according to the ICD-10 criteria. The children were followed over a period of 30 years. The mean age at the time of study was 38 years. The results show that in adulthood the autistic patients had a poorer outcome than children with Asperger syndrome as regards education, employment, autonomy, marriage, reproduction and the need for continuing medical and institutional care. Particular attention is given to pharmacotherapy and the relationship between the childhood disorder and psychiatric morbidity in adult life.
The case of a 6-year-old boy who developed childhood disintegrative disorder (Heller syndrome) at the age of 4 years is presented, and specifics of the neurologic evaluation are detailed. A table is provided suggesting the complete neurologic work-up with the potential findings for children presenting with signs and symptoms of deterioration. A hypothesis for the aetiology of Heller syndrome proposes that predisposing genetic factors when combined with an environmental stress result in the deposition of amyloid and the disruption of synaptic transmission during the deterioration period. Speculation that the deterioration may be self-limited by activation of an immune response is based upon earlier findings that interleukin 1 has been shown to be involved in the breakdown of amyloid precursor protein in humans.
Though a sex difference in the incidence of autism has frequently been reported, few studies have considered sex differences in the severity of features associated with autism. The Autism Diagnostic Interview was used to assess the difference between a group of 21 females and 21 males with autism with equivalent chronological nonverbal IQ greater than 60. Males were rated to be more severely autistic than females on several measures of early social development, but not in any other areas. Results are discussed in relationship to hypotheses based on sex differences in other populations.
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The objectives of this paper are to review the literature on the diagnostic validity of schizotypal personality disorders (SPD) in childhood and to present the results of a chart review of 20 DMS-III-defined SPD children. The literature on this disorder is relatively limited, and although there is some agreement on clinical presentation, various authors have used different diagnostic labels. The DSM-III criteria for SPD appear to have acceptable content validity, but whether these are the best criteria for identifying a disorder with a particular etiology and natural history has not been investigated. The chart review on 20 SPD children presents information on early history, family background, neurodevelopmental impairments, and the degree of overlap with other, better-established diagnoses. On the basis of the chart review, suggestions for further research on the diagnostic validity of SPD are made.
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Published empirical studies were reviewed to establish family demographics of clinically referred children and adolescents. Data on parental marital status were collected from studies of referred and nonreferred children and adolescents in six empirical journals. Data on family socioeconomic status (SES) and child race or ethnicity were also collected. Based on 86 studies that presented parental marital status, 56.4% of clinically referred children and adolescents live with both their biological mother and biological father. Family SES data were difficult to summarize because of the different methods of presenting this information. When considering race and ethnicity, 86% of the children and adolescents in the clinical samples were Caucasian American. Of the studies that were originally reviewed, 80.4% did not include parental marital status and 36.7% did not include SES, race, or ethnicity of the participants. There were no significant differences between journals in the rates of inclusion of family demographic data. In addition to revealing family demographic information about children and adolescents who participate in clinical research, these data highlight the lack of consistency in the presentation of family demographic data in published research. Suggestions are provided to help researchers collect and present meaningful family demographic data in clinical and nonclinical research with children and adolescents.
The validity of a social subtyping scheme forwarded by Wing and her colleagues is examined in a sample of 53 autistic individuals. Clinical ratings of subtype (aloof, passive, active-but-odd), IQ estimates, and measures of adaptive functioning and level of autism were obtained. Subjects were individually administered the Autism Diagnostic Observation Schedule (ADOS) by the principle examiner, who was blind to subtype assignment. The general findings supported the validity of aloof and active-but-odd subtypes as predictors of behavior across language/communication, reciprocal social interaction, and stereotyped behavior/restricted interest domains and suggested that individuals assigned to these respective groups differed in a number of important ways (e.g., level of autism, IQ, adaptive behavior). Partial support for an intermediate, passive subtype was garnered. The clinical utility of social subtypes is discussed as an important implication of this work.
We examined the distribution of ages of onset of autism and related communication handicaps and assessed factors related to age of onset. Subjects were approximately 1,800 children seen at Division TEACCH (Treatment and Education of Autistic and related Communication handicapped CHildren) since 1970. Exact numbers of subjects varied with different analyses due to missing data. Data were gathered through direct assessment, interview, and questionnaire. Seventy-six percent of autistic children were identified by parents by 24 months of age, and 94% by 36 months. Families reporting early onset tended to seek help sooner and to be seen by TEACCH sooner. Early onset was most clearly related to severity as measured by IQ and ratings on the Childhood Autism Rating Scale (Schopler, Reichler, & Renner, 1986). The findings support the treatment of age of onset of autism by DSM-III-R (American Psychiatric Association, 1987).
A study was conducted to assess the effects of redesigning the physical environment (i.e., the classroom) on the occurrence of self-stimulation, on-task behavior, inappropriate behavior, and inactivity. Three developmentally disabled males, diagnosed as autistic-type, participated. Data were collected using a withdrawal design. Results showed a decrease of self-stimulation and inactivity and an increase of on-task behavior. Inappropriate behavior remained unchanged across experimental conditions.
BACKGROUND: The validity of clinical guidelines changes over time, because new evidence-based knowledge and experience develop. OBJECTIVE: Hence, the European clinical guidelines on hyperkinetic disorder from 1998 had to be evaluated and modified. METHOD: Discussions at the European Network for Hyperkinetic Disorders (EUNETHYDIS) and iterative critique of each clinical analysis. Guided by evidence-based information and based on evaluation (rather than metaanalysis) of the scientific evidence a group of child psychiatrists and psychologists from several European countries updated the guidelines of 1998. When reliable information is lacking the group gives a clinical consensus when it could be found among themselves. RESULTS: The group presents here a set of recommendations for the conceptualization and management of hyperkinetic disorder and attention deficit/hyperactivity disorder (ADHD). CONCLUSION: A general scheme for practice in Europe could be provided, on behalf of the European Society for Child and Adolescent Psychiatry (ESCAP).
VIQ-PIQ differences have been studied in children with autism and Asperger syndrome but have not been studied in a separate group of children with PDD-NO, although, PDD-NOS has a much higher prevalence rate than autism and deficits in communication and social interaction are severe. The Wechsler Intelligence Scale for Children-Revised (WISC-R) was administered to 100 children, aged 6-12 years, with PDD-NOS (n = 76), autism (n = 13), and Asperger syndrome (n = 11). PDD-NOS was diagnosed using explicit research criteria. No overall differences between VIQ and PIQ were found in PDD-NOS and autism. Peaks in the subtest scores on Information, Similarities, Picture Arrangement, and Mazes, and troughs in the subtest scores on Comprehension, Digit Span, and Coding were demonstrated in children with PDD-NOS. Their score on the Freedom from Distractibility factor was lower than the scores on the Verbal Comprehension factor and the Perceptual Organization factor. Children with PDD-NOS seemed to have a similar VIQ-PIQ profile as children with autism, and on the subtest level children with PDD-NOS showed some similarities to children with Asperger syndrome or autism. It was not possible to distinguish PDD-NOS from autism or Asperger syndrome by using IQ scores.
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The objective of this study was to refine the Children's Social Behavior Questionnaire (CSBQ), to reduce its length, and to verify its psychometric properties. The CSBQ is a questionnaire for parents or caregivers of children with PDD. The items describe a broad range of features that are typical of PDD, particularly in its milder forms. Based on conceptual judgment and factor analyses, the number of items was reduced from 96 to 49. Six subscales were constructed to allow a differentiated description of PDD problems. Estimates for internal, test-retest, and inter-rater reliability, and for convergent and divergent validity were good. Different clinical and control groups showed the hypothesized patterns in nature and degree of their problems.
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This paper studied whether abnormal P3 amplitudes in PDD are a corollary of abnormalities in ERP components related to selective attention in visual and auditory tasks. Furthermore, this study sought to clarify possible age differences in such abnormalities. Children with PDD showed smaller P3 amplitudes than controls, but no abnormalities in selective attention. Adolescents with PDD showed abnormal selective attention, as reflected by larger auditory Processing Negativity (PN) and visual N2b, but no P3 abnormalities. Dipole localizations revealed that the locations of PN generators in subjects with PDD differed from controls. It was concluded that the abnormalities in selective attention in adolescents with PDD have a normalizing effect on P3, and possibly act as a compensatory process.