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Query: UMLS:C0025362 (mental retardation)
 15,878 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A large number of investigation techniques are used to establish the relationships between the clinical and biological data which are necessary for physiopathological analysis in the field of developmental disorders. It therefore seemed necessary to develop a quantified grouping system, based on developmental assessments, which could allow closer matching between clinical evaluations and biological numerical data. Two hundred and two subjects presenting developmental disorders (autistic disorder, pervasive developmental disorder not otherwise specified and mental retardation) were examined. For each child, a quantification of autistic behaviour, intellectual impairment, neurological signs and language and communication disorders was performed. A cluster analysis of these quantified data elicited four subgroups according to the scores obtained in these four different areas. We showed the value of this approach by applying it to one of the studies of monoamines routinely examined in childhood autism--dopamine and HVA, its main urinary derivative. Moreover, this method revealed a subgroup within the total population which was independent of nosographic classification and which had a particular clinical and biochemical profile. Other applications could follow, for example in the fields of neurophysiology, cerebral imaging, molecular biology and genetics.
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PMID:Quantified multidimensional assessment of autism and other pervasive developmental disorders. Application for bioclinical research. 779 50

Noncontingent reinforcement (NCR), a response-independent schedule for the delivery of reinforcement, has been found to be effective in reducing behavior when the reinforcer delivered is responsible for behavioral maintenance. In this study, dense and lean schedules of response-independent attention were compared to determine whether it is necessary to begin with a dense schedule before fading to a lean schedule, or whether treatment would be as effective using a lean schedule at the outset. The subjects were 5-year-old identical quadruplets diagnosed with mental retardation and pervasive developmental disorder who displayed destructive behavior that was maintained by social attention. NCR was selected partially because it is not very labor intensive and could be implemented by a single mother simultaneously with all 4 children. Using a combination multielement and multiple baseline design, it was found that (a) a dense schedule of response-independent reinforcement (i.e., fixed-time 10 s) resulted in immediate and dramatic reductions in destructive behavior with no evidence of an extinction burst, and (b) an equivalent reduction in destructive behavior was achieved with a lean schedule of response-independent reinforcement (fixed-time 5 min) only after a systematic fading procedure was implemented. The findings suggest that the effectiveness of NCR may be dependent on the use of a dense schedule initially, and that systematic fading can increase the effectiveness of a lean schedule.
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PMID:Schedule effects of noncontingent reinforcement on attention-maintained destructive behavior in identical quadruplets. 806 30

Assessed differences in sex ratio, severity of associated mental retardation, and various metrics of severity of autism in autistic, PDD-NOS, and developmentally disordered (non-PDD) cases. Males with autism were more frequent than females, particular at higher IQ levels. The three clinical groups differed, in expected ways, in the various measures of severity of autism with the PDD-NOS cases being intermediate between the strictly diagnosed autistic group and the non-PDD developmental disordered group. Sex differences were primarily confined to IQ; sex differences in other metrics of severity of autism were not prominent. Implications for future research are discussed.
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PMID:Sex differences in pervasive developmental disorders. 810 1

An estimate of the prevalence of autism in tuberous sclerosis (TSC) was made by interviewing the parents of 21 children between ages 3 and 11 ascertained during a previous population study of the condition in the West of Scotland. Five of the children (24%) were rated autistic and a further four (19%), all of whom were girls, had socially impaired behavior categorized as pervasive developmental disorder, without fulfilling all the DSM-III-R criteria for autism. One further boy had disruptive attention-seeking behavior that had excluded him from his normal school. The estimated prevalence from this study of autism in TSC is 1 in 4 children in general, and 1 in 2 of those with mental retardation. Tuberous sclerosis could be a significant cause of autism and pervasive developmental disorders, particularly in girls.
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PMID:A prevalence study of autism in tuberous sclerosis. 833 Oct 50

In this report we present data on cognitive development, language, behavior and social skills in 12 children and adolescents, nine girls and three boys, aged between 2.5 and 19 years, with Coffin-Siris syndrome (CS). 1. Mental retardation was mild in three patients and moderate in the nine others. 2. Speech onset was severely retarded with little interest in language. In the older group (seven patients aged 7 to 19 years), language comprehension was appropriate to the mental level. 3. Gross-motor functioning and autonomy, with the lowest score on "Task-orientation", were equal to the mental development. 4. Most frequently, aggressive disturbed behavior was observed, especially in the youngest children, while mixed disturbed behavior was observed in the oldest patients. Almost half of the CS patients (5/11) presented symptoms of pervasive developmental disorder, with 2/11 scoring in the pathological range. Obsessive interests, strong dependence on patterns and rituals and unusual fears were characteristic behavioral problems also when they got older.
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PMID:The Coffin-Siris syndrome: data on mental development, language, behavior and social skills in 12 children. 859 67

Elective mutism (EM) is not regarded as a separate diagnostic category in the ICD-9, but is included under the heading "313: Disturbance of emotions specific to childhood and adolescence." In the ICD-10 EM is acknowledged as a separate diagnosis defined as "a marked, emotionally determined lack of speech in certain situations in a child with a normal or near normal speech/language ability." The diagnosis excludes pervasive developmental disorder and specific developmental disorders of speech and language. Two patients referred for EM to a child and adolescent psychiatry outpatient clinic, showed specific developmental delays and assessment indicated slight mental retardation in one of them. The question arose regarding how extensively EM is reported in the literature as associated with developmental disorder/delay. A search was carried out in four data-bases where most references were listed under the keyword "mutism". This paper presents the two case studies, and the results of the literature inventory. It concludes that EM may be associated with developmental disorder/delay and suggests that this could be a predisposing factor for an emotionally determined lack of speech in certain situations.
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PMID:Elective mutism--associated with developmental disorder/delay. Two case studies. 944 3

The frequency and clinical presentation of autism in 28 probands with tuberous sclerosis complex (TSC) are reported and risk factors that may influence the development of autism in TSC are examined. Eight probands meet ICD-10 and DSM-IV criteria for autism, an additional 4 meet criteria for pervasive developmental disorder (PDD). Twelve TSC probands with autism/PDD are compared to 16 TSC probands without these conditions for factors which may underlie the association of autism and TSC. A specific seizure type, infantile spasms, as well as mental retardation, are increased in the TSC, autistic/PDD group. Furthermore, rates of social phobia and substance abuse are elevated among first-degree relatives of TSC probands with autism compared to first-degree relatives of TSC probands without autism. Implications of these findings in understanding the association of autism and TSC are discussed.
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PMID:Autism in tuberous sclerosis complex. 958 71

The purpose of this study was to determine the prevalence of the fragile X (FRAX) CGG trinucleotide expansion in a population of young girls (n = 45) diagnosed with pervasive developmental disorder (PDD). Their mean age was 43.7 months (range, 25 to 132 months). Diagnoses included autistic disorder (n = 20), PDD (n = 23), and Asperger's syndrome (n = 2). Molecular FRAX testing was performed on all patients by using the Southern gene blot technique. Genomic DNA was digested with both EcoRI and EagI, fractionated on agarose gel, and blotted and probed with the radiolabeled StB12.3 FMR-1 probe. None of the subjects were found to have an expansion of CGG in either the 2.8 kb or 5.2 kb fragments. A 95% CI, for the prevalence of the FRAX mutation in female subjects with PDD, has an upper bound of 2.9%. We conclude that the prevalence of FRAX positivity in girls with PDD is lower than previously reported. This raises the question of whether any association between FRAX and PDD in female subjects is specific to PDD or is related rather to the presence of mental retardation.
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PMID:Absence of the fragile X CGG trinucleotide repeat expansion in girls diagnosed with a pervasive developmental disorder. 973 17

In this study, we examined three maladaptive behaviors, self-injurious behavior (SIB), stereotypies, and aggression in adults with autism, pervasive developmental disorder, not otherwise specified (PDD-NOS), and mental retardation. We used a brief functional analysis rating scale. The Questions About Behavioral Functions (QABF), to examine the function of each behavior. Across the three groups, our results indicated that aggression was primarily maintained for attentional reasons and stereotypies for nonsocial reasons. No specific function(s) were found to maintain SIB. These results suggest that the function of a maladaptive behavior may be associated more with the particular maladaptive behavior displayed rather than inclusion in a certain diagnostic group. Implications of findings for assessment and treatment issues are discussed.
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PMID:An analysis of maladaptive behaviors in persons with autism, PDD-NOS, and mental retardation. 977 Feb 55

Pervasive developmental disorders without mental retardation is a new clinical category including high-functioning autism, Asperger syndrome and pervasive developmental disorder non otherwise specified. Its recognition is recent and still problematic in many regards. This article reviews the historical, theoretical and clinical relations between pervasive developmental disorders without mental retardation and bordering disorders. The consequences of an inadequate diagnosis on measures of assistance for these patients is also investigated. The authors conclude on the necessity in considering, independently of the diagnosis, the description of symptoms, the intellectual level and the adaptative level in order to take the most appropriate educational and psychosocial decisions regarding pervasive developmental disorders without mental retardation.
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PMID:[Diagnosis of pervasive developmental disorders without mental retardation and its impact on obtaining social and educational services in Quebec]. 977 56


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