Treatment issues in genetic mental retardation syndromes.

Because of genetic advances and radical shifts in service delivery, psychologists in community mental health and social service agencies increasingly serve clients with mental retardation syndromes. Persons with specific genetic mental retardation syndromes often differ in their behavioral strengths and weaknesses and in their predispositions to psychiatric disorders. Although genetic breakthroughs are sparking more research on these so-called behavioral phenotypes, researchers have yet to systematically translate phenotypic data into guidelines for therapy and intervention. Using fragile X syndrome, Williams syndrome, and Prader-Willi syndrome as examples, this article shows how the behavioral phenotypes of mental retardation syndromes can inform clinical practice. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Problem behaviors associated with deletion Prader-Willi, Smith-Magenis, and cri du chat syndromes

Many studies have failed to determine a systematic dose-response relationship across different cognitive tasks between caffeine and EEG power spectra. However, a nonlinear approach to EEG analysis, which reconstructs a multi-dimensional state space from each electrode recording, can be used to compute the number of active degrees of freedom in the signal (the correlation dimension, D2), and can be interpreted as a measure of signal complexity. This study attempted to determine a consistent dose-response relationship between caffeine and EEG D2, across six oral caffeine doses (100-600 mg), with each subject acting as their own control, to create a probabilistic bias against finding any consistent linear or nonlinear dose-response relationship across different cognitive tasks. The experiment (n = 10) was conducted with three within-subjects explanatory variables, 2 (experimental, placebo) x 8 (caffeine level) x 4 (type of cognitive task performed), with EEG D2 as the response variable, measured from Fz, F3, F4 and Cz. A significant three-way interaction was found [F(21,245.3) = 3.65, P = 0.001]. Regression analyses revealed a linear trend for the response variable across trials for the placebo condition (average R2 = 0.54), whereas linear+quadratic trends explained an average 30% of the variance for the experimental condition, compared to 0.01% for the linear fit, indicating a robust quadratic dose-response relationship between caffeine and EEG D2. Three conditions had positive quadratic co-efficients, and one condition had a negative quadratic co-efficient. These results are discussed in terms of the implications for brain dynamics, and with respect to recent criticisms of the computation of D2 from EEG.     

Growth differences associated with compulsive and stereotyped behavior disorders in adults with mental retardation

Oxidized-low density lipoprotein (ox-LDL) exhibits various atherogenic properties, such as the formation of foam cells, the recruitment of macrophages into arteries, the promotion of cell growth, the formation of thrombi, and the development of vasoconstriction. Antioxidants inhibit atherosclerosis in cholesterol-fed animals by preventing the recruitment of macrophages into the arteries suggesting that oxidation is important for such recruitment of macrophages, and that it is responsible for early development of atherosclerosis. Calcification and an increase in extracellular matrix (ECM) are frequently observed in advanced atherosclerosis. These processes are active and regulated, not passive and degenerative, and do not appear to be controlled by ox-LDL. The control of calcifications and ECM increase will provide new directions in the treatment of atherosclerosis especially in the advanced stages. Future treatment should include strategies to inhibit the calcification and the increase in ECM.     

Clomipramine treatment for stereotype and related repetitive movement disorders associated with mental retardation

The efficacy of the serotonin uptake inhibitor clomipramine in the treatment of stereotyped and related repetitive behavior disorders was tested in individuals with severe and profound mental retardation. A double-blind, placebo-controlled crossover trial of clomipramine was associated with significant reductions in the frequency and intensity of stereotyped behavior and teacher ratings of stereotypy, hyperactivity, and irritability as well as increased adaptive engagement and decreased staff intervention for nontargeted behavior problems. Adverse effects occurred in 3 of the 10 subjects. Of the 7 subjects who tolerated the drug, 6 exhibited a clinically significant improvement in one or more repetitive behaviors. Our results provide support for the hypothesis that clomipramine is effective in treating stereotyped and related behaviors associated with mental retardation.     

Diagnostic testing for Prader-Willi and Angelman syndromes: response

Between 1992 and 1997, every year, approximately 30,000 examinations of the liver and bile ducts were done. Addition of colour Doppler imaging improves safety of procedures and allowed to perform ultrasound guided biopsy in 73 patients. On the basis of our experience we state that colour Doppler sonography guided percutaneous fine needle biopsy of the liver is useful, sufficient and safety diagnostic method of abscesses, cysts and malignant tumours of the liver.     

Persons with emotional disturbance and mental retardation.

In response to discussion by S. Reiss et al (see record 1982-30849-001) about sources of information on the prevalence of emotional problems among retarded people, the present author describes a study of 27,385 Ss with developmental disabilities that looks at dual diagnoses (psychiatric impairment and developmental disability) in relation to age, intellectual level, and residential setting. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Psychology and mental retardation.

The history of mental retardation as a focus within our discipline reflects a long, tortuous, and mostly honorable involvement with conceptions of intelligence and handicap and with parables of ethical treatment. The articles in this special section sum up past progress, assess current conceptions and controversies, and challenge the science and practice of psychology in this demanding field of human concern. An essential message of all of the articles in this special section should be discernible as a common theme: The study of mental retardation is part and parcel of the mainstream of American psychology. Concerns about the nature of intelligence and its determinants, for example, are equally the concerns of those who deal with normal or even advanced children and adults. Findings about the impact of living conditions on the functioning of retarded individuals have implications for all of us. Psychologists and other scientists with diverse points of view will find much of relevance to their own work in this forum. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Prader-Willi and Angelman syndromes. Disorders of genomic imprinting

OBJECTIVE: To develop estimates of state Medicaid expenditures attributable to smoking for fiscal year 1993. METHODS: The smoking-attributable fractions (SAFs) of state Medicaid expenditures were estimated using a national model that describes the relationship between smoking and medical expenditures, controlling for a variety of sociodemographic, economic, and behavioral factors. RESULTS: In fiscal year 1993, the SAF for all states (all types of expenditures) was 14.4%, with a range from 8.6% in Washington DC to 19.2% in Nevada. On average, SAFs ranged from a low of 7.9% for home health services expenditures to 21.7% for hospital expenditures. An estimated total of $12.9 billion of fiscal year 1993 Medicaid expenditures was attributable to smoking. The relative error of this estimate was 40.3%. CONCLUSIONS: Cigarette smoking accounts for a substantial portion of annual state Medicaid expenditures, with considerable variation among states. The range in expenditures among the states is due to differences in smoking prevalence, health status, other socioeconomic variables used in the model, and the level and scope of the Medicaid program.     

Statement on mental illness and mental retardation.

The statement made on behalf of the American Psychological Association is in support of Senate Bills 755 and 756 developed by Congress in response to President Kennedy's message on mental illness and mental retardation. "The psychologist is vitually concerned with the problems of mental disorders and of mental retardation. He is especially interested in research, and in the provision of services to people through the organized agencies of society, such as the public schools, community mental health centers, hospitals, institutions for the retarded, and rehabilitation agencies of many kinds." (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Psychology and mental retardation.

Presents the view that mental retardation is primarily a psychoeducational and social problem. In addition to traditional services, psychology can serve the mentally retarded in other indirect areas, e.g., new direct-care personnel training, institution management, manpower utilization in the retarded population, and help in changing the public image of the mentally retarded. It is concluded that psychology should increase its participation in education, training, and research in mental retardation, and that programs should be developed to demonstrate that psychological techniques and learning theories can be applied appropriately to the problems of the retarded person. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Bilateral periventricular nodular heterotopia with mental retardation and syndactyly in boys: a new X-linked mental retardation syndrome

Bilateral periventricular nodular heterotopia (BPNH) is a recently recognized malformation of neuronal migration, and perhaps proliferation, in which nodular masses of gray matter line the walls of the lateral ventricles. Most affected individuals have epilepsy and normal intelligence with no other congenital anomalies. A striking skew of the sex ratio has been observed because 31 of 38 probands have been female, and one gene associated with BPNH was recently mapped to chromosome Xq28. We report three unrelated boys with a new multiple congenital anomaly-mental retardation syndrome that consists of BPNH, cerebellar hypoplasia, severe mental retardation, epilepsy, and syndactyly. Variable abnormalities included focal or regional cortical dysplasia, cataracts, and hypospadius. We hypothesize that this syndrome involves the same Xq28 locus as isolated BPNH, and we review the expanding number of syndromes associated with BPNH.     

Genomic imprinting and its role in Prader-Willi and Angelman syndromes

Published and our own data, included in the CHRODYS database, on the dependence of phenotypic abnormalities in mono-, di-, and trisomics at human chromosome 15 on its parental origin are reviewed. The concept is confirmed that Prader-Willi and Angelman syndromes result from the combined effect of gene or chromosome mutations impairing the expression of syndrome-specific genes and from genomic imprinting, i.e., repression of corresponding genes received from one of the parents.     

Implicit and explicit memory in individuals with mental retardation

Students with and without mental retardation from three age groups were compared on implicit and explicit memory tasks. Consistent with previous research on intelligence-related differences in controlled and automatic processes, students without mental retardation performed better than those with mental retardation on the explicit memory task, but there was no difference between groups on the implicit memory task. For both groups implicit and explicit memory increased from age 6 to 8 to age 10 to 12, but did not significantly increase to age 15 to 17. Because implicit memory appears to be a relative strength for students with mental retardation, we suggest further exploration into broader types of implicit processes that may be useful in training situations.     

Community-based behavioral training approaches for people with mental retardation and mental illness.

Reviews challenges associated with behavioral training approaches for individuals with mental retardation and mental illness in the community. Family and non-family facilitated training are considered. Professional practice issues are reviewed, and justification for multifactor behavioral assessment is offered. Future research directions are discussed. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Cognitive controls and mental retardation.

Devised a color-digit interference task and 2 sorting tasks as variants of the Stroop Color-Word Test and R. Gardner, et al. (see record 1961-02266-001) sorting tasks, respectively. These tasks proved applicable to a mentally retarded sample (n = 39) and provided reliable measures of 2 cognitive control dimensions (constricted-flexible control and equivalence range). As predicted, the main test scores were significantly more variable in retarded Ss than in normals (n = 32). Analyses of performance on the color-digit interference task indicated that retarded Ss were significantly slower than the normals on all parts of the task, particularly under conditions of distraction (whether inherent in the task or externally imposed). Low IQ retarded Ss were significantly slower than high IQ retarded Ss on all parts of the task. The extreme cognitive control test scores of the retarded Ss, the nature of the attentional deficit among these Ss, and the relevance of a cognitive control approach for the training of retarded persons are discussed. (16 ref.) (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Self-invented addition strategies by children with mental retardation

Children with mental retardation often seem incapable of self-initiated learning. A training experiment was designed to determine whether such children could spontaneously invent more efficient addition strategies for calculating simple sums; apply these strategies to larger, unpracticed combinations; and retain these strategies after 5 months. An experimental group and a control group were shown a basic concrete counting procedure. Over 6 months, the experimental group was given regular opportunities to practice computing sums. Many of them invented calculational short cuts. On immediate and delayed posttests, they used significantly more sophisticated strategies than did control participants. Results suggest that children with mental retardation can invent, transfer, and retain strategies for learning tasks.