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

A sex-linked familial neurological disease consisting of cerebral palsy, mental retardation, choreoathetosis, and compulsive aggressive behavior is associated with a loss of an enzyme that participates in purine metabolism, namely, hypoxanthine-guanine phosphoribosyltransferase. The production of excessive uric acid in this disorder implies that the enzyme is involved in the normal regulation of purine biosynthesis. This is the first example of a relation between a specific enzyme defect and abnormal compulsive behavior. It is also the first enzyme defect in purine metabolism demonstrated in a neurological disease.
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PMID:Enzyme defect associated with a sex-linked human neurological disorder and excessive purine synthesis. 602 Feb 92

The authors evaluated the presence of assaultive behavior in 103 children, aged 6-12 years, seen in the psychiatric inpatient and outpatient services of a municipal hospital. No racial or ethnic differences were found. Boys were significantly more assaultive than girls and used fire setting and hitting with objects more often. Inpatients were significantly more assaultive than outpatients. Children with conduct disorders, specific developmental disorders, and mental retardation were more assaultive than those with neurotic disorders. Multiple regression analysis showed that the child's past aggressive behavior, absence of anxiety and depression, and parental assaultive behavior were the best predictors of assaultive behavior.
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PMID:Predictors of assaultiveness in latency age children. 618 81

A kindred has been studied in which mental retardation and marfanoid clinical features are present in several individuals. The pedigree is consistent with X-linked recessive inheritance. Four affected males aged 12-18 years and four obligate carriers have been identified. Clinical findings in the 4 affected males included a tall slender habitus (3) (the fourth was tall but muscular), a long-narrow face (3), large head (4), highly arched palate (4), small mandible (4), abnormal speech (4), hypernasal voice (3), joint hyperextensibility (3), borderline to large testes (3), pectus excavatum (2), atrial septal defect (1), and a double row of teeth (1). Mental retardation (4) ranged from mild to severe; abnormal behavior included hyperactive and aggressive behavior (2), autistic-like (1) and jovial behavior (1). One and possibly two, males had absence of the corpus callosum. Chromosome studies on all were normal; no marker X was observed. We believe this family probably represents a new form of X-linked mental retardation.
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PMID:A form of X-linked mental retardation with marfanoid habitus. 671 3

Two brothers presented from birth with features characteristic of Sotos syndrome (cerebral gigantism): overgrowth, craniofacial abnormalities, and mental retardation with hyperactive and aggressive behavior. X-ray examination of the hands revealed imbalanced and advanced skeletal age in one, whereas anterior fontanel bones were present in both brothers. Various hormone concentrations in plasma were all within normal limits, as were the results of a search for abnormal metabolites in plasma and urine. The occurrence of this usually sporadic syndrome in two sons of possible remotely consanguineous, healthy parents, suggests that in some cases Sotos syndrome may be inherited as an autosomal recessive trait. Thus our observation may support the suggestion of heterogeneity of Sotos syndrome. Until specific tests for the identification of various types are available, genetic counseling for this syndrome is difficult.
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PMID:Sotos syndrome in two brothers. 744 80

Aggressive behaviors of two men with mental retardation were investigated during sheltered workshop and community employment. The first year of community work showed a monthly average reduction in aggressive behaviors of 93%. Changing to a typical work setting with regular expectations led to this decrease.
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PMID:Decrease violence in a day program: community-based endeavors. 748 Apr 85

Operant conditioning-based behavioural interventions are commonly used for the behavioural problems of individuals with mental retardation. There is also growing evidence of the benefits of these interventions for treating some of the behavioural problems of individuals with acquired cognitive deficits resulting from brain trauma. However, the effects of behavioural interventions on behavioural problems occurring during acute neurorehabilitation, when orientation and memory are most impaired, have not been studied. In this empirical case study, operant conditioning-based procedures were applied with an 8-year-old girl recovering from brain trauma and related neurosurgery. Screaming, non-compliance and aggression, which were disrupting rehabilitation therapies and follow-up neuroimaging, were treated using differential positive reinforcement techniques. Beneficial behavioural intervention effects were demonstrated using single-subject experimental methods. Aberrant behaviour during physical and occupational therapies was reduced, and cooperation with a computerized tomography (CT) scan without sedation was accomplished using operant behavioural intervention. Results support the use of operant interventions early in recovery from brain trauma, and highlight the importance of interdisciplinary collaboration for the implementation and further study of early behavioural interventions.
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PMID:Behavioural training during acute brain trauma rehabilitation: an empirical case study. 758 54

We contrasted a sample of children and adolescents with affective disorders and mental retardation with a comparison group on behavioral symptoms, associated diagnoses, and psychopharmacologic treatment. Fifty consecutive patients with both impaired intellectual functioning and at least one affective disorder admitted to a psychiatric inpatient unit for children and adolescents with developmental disabilities and psychiatric disorders were matched to a group of 50 inpatients without depression. Behavioral symptoms such as suicidal ideation or gestures, crying, irritability, sleep problems, agitation, mood lability, and social withdrawal/isolation occurred significantly more often in the affective group than in the comparison group. Aggression, however, was the most frequent behavior concern for both groups, whereas disruption/destruction was identified significantly more often in the comparison group. Regarding Axis I diagnoses, the comparison group was more often identified with externalizing disorders (ADHD, ODD), though there was a high rate of comorbidity in the affective disorder group. The behavioral symptoms used to diagnosis normally developing children and adolescents appear to be applied in making affective disorders diagnoses in this sample of children and adolescents with mental retardation.
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PMID:Affective disorders in hospitalized children and adolescents with mental retardation: a retrospective study. 765 3

To investigate the relationship between psychiatric disorders and severe behavior problems in mental retardation, statewide client databases from developmental disabilities services in California (N = 89,419) and New York (N = 45,683) were analyzed and juxtaposed. The study focussed on nine major DSM-III-R psychiatric categories (or their equivalents), and severe forms of aggressive behavior, property destruction, self-injurious behavior, and stereotyped behavior in individuals 45 years old and younger with mental retardation of all levels of severity. In California, 3.9% had at least one psychiatric diagnosis; in New York, 5.4%. The rate of specific psychiatric diagnoses was variable across states, suggesting local preferences in diagnostic practices. Severe behavior problems occurred in 22.1% in California and in 41.4% in New York. This difference in rates can be attributed in part to different recording criteria for behavior problems. With regard to the association between psychiatric diagnoses and problem behaviors the results were consistent across databases: No compelling correlations were found. This means that neither aggression, self-injury, destruction, nor stereotypies determine whether a person receives a psychiatric diagnosis or not.
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PMID:The association between psychiatric diagnoses and severe behavior problems in mental retardation. 790 16

A cohort of 199 individuals with mental retardation referred for behavioral and psychiatric crisis intervention services was studied to determine attributes differentiating physically aggressive behavior from other behavioral problems. Individuals with aggressive and nonaggressive behavior had similar neurological histories and current medical status and similar levels of seizure disorders and CNS abnormalities. Aggressive individuals more often had psychiatric diagnoses of organic brain syndrome, but frequencies of this diagnosis in each group were small. Current aggression was predicted by gender, level of mental retardation, and history of previous institutional placement; the strongest predictor was history of aggression. These data suggest a complex equation to describe social inadequacy involving interactions between CNS functioning and developmental cognitive and social variables that are only partially defined at this time. Further work to characterize this interaction almost certainly must include a prospective longitudinal analysis of social and developmental functions early in life.
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PMID:Characteristics of community-based individuals with mental retardation and aggressive behavioral disorders. 805 99

The FG syndrome is an X-linked recessive mental retardation syndrome. Ten patients are reviewed with special emphasis on the natural history of the intellectual development, constipation, and the prognosis for growth and behaviour. Six out of 10 patients are still macrocephalic, and there is no evidence for a specific growth pattern with respect to height. The degree of mental retardation is is usually severe. The behaviour is characteristically friendly, sociable and over-talkative, with periodic aggression. Six patients have seizures. A characteristic progression seems to occur from congenital hypotonia with joint hyperlaxity at birth, to joint contractures with apparent spasticity and unsteady gait later in life. The constipation was a temporary problem in five cases. The cowlick and the fetal pads persist and are important, but not specific, for the diagnosis.
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PMID:A clinical follow-up of British patients with FG syndrome. 805 29


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