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Efficacy of a verbal training sequence on the sorting performance of moderately and severely mentally retarded adolescents.

Seven mentally retarded adolescents received either verbal or nonverbal training on a shape-sorting task within a multiple baseline design. Three clients received the nonverbal training sequence, which used contingent attention and praise for correct responses and a correction procedure for incorrect responses. The remaining four clients received the verbal training sequence, which was the same as the nonverbal sequence except that the clients were taught to verbalize (label) the relevant dimension of the objects they were sorting. Both training sequences resulted in the successful acquisition of the target performance, but only the verbal training sequence facilitated generalization of performance to a novel shape and to a new dimension. Following generalization testing, clients initially exposed to the nonverbal sequence were provided verbal training, which produced generalization in these previously unsuccessful clients.

Adult↗

A matched comparison of the developmental growth of institutionalized and deinstitutionalized mentally retarded clients.

Seventy mentally retarded clients previously residing in a large state institution were matched with 70 clients who remained at the same institution. Clients were matched by sex, level of retardation, years institutionalized, self-care skills, age, and IQ. Developmental growth, operationally defined as adaptive behavior change using a research version of the AAMD Adaptive Behavior Scale, was measured for all clients at Time 1 and Time 2. Demographic and environmental factors that could be associated with adaptive behavior growth were explored. Only the deinstitutionalized clients displayed significant growth. They increased in adaptive behavior and showed no significant change in maladaptive behavior.

Adult↗

Use of computer- and teacher-delivered prompts in discrimination training with individuals who have mental retardation.

Individuals with mental retardation received training on a series of 16 visual discrimination problems. Eleven subjects received computer-delivered instruction with a stimulus-fading procedure and 11 were trained with verbal and nonverbal prompts delivered by a human teacher. We found that (a) the teacher's prompts were effective with more subjects than was the computer-based fading procedure; (b) transfer to stimulus control to task stimuli was poor with the teacher's prompts but nearly perfect when fading was effective; and (c) subjects learned more discrimination problems with the computer-delivered fading procedure. These results suggest that the computer could be a useful tool to assist in discrimination training in special-education settings.

Adult↗

Endocrine disorders associated with mental retardation.

Endocrine disorders associated with mental retardation are described in relation to clinical characteristics, pathogenesis, diagnostic procedures, and treatment. Some endocrine disorders, particularly hypothyroidism, nephrogenic-diabetes insipidus, and hypoglycemic conditions, are frequently associated with mental retardation. Early diagnosis and prompt and proper management reduce mortality and the incidence of mental retardation associated with endocrine disorders.

Child↗

Exposure keratopathy in a mentally retarded adult population.

BACKGROUND: Mentally retarded patients are often found to have exposure corneal disease. No study could be found that documented the prevalence of this corneal disorder among these patients. METHODS: A retrospective study was conducted on a population of 210 mentally retarded adults to determine the prevalence of clinically significant exposure keratopathy. RESULTS: 17.6 percent of this population was found to have this disorder mostly due to incomplete blinking. CONCLUSIONS: Exposure keratopathy was found to be a significant ocular health problem among this population. Since many of these people cannot verbalize their discomfort it is recommended that the optometrist tailor his or her examination so this disorder is not overlooked and untreated.

Adult↗

Developmental assessment of cognitive style in mentally retarded psychotics.

Evaluated 55 mentally retarded psychotic adults with the Color-Form Preference Test, a developmentally based index of cognitive style, and other measures of intellectual, social, and psychiatric functioning. Results indicated that different grades of retardation were associated with distinct cognitive styles, which showed a systematic progression that corresponded to the normal developmental sequence. In contrast, no significant relationships were observed between cognitive style and psychotic manifestations. The findings suggested that fresh insights into mental retardation and psychosis are made possible by qualitative, developmental assessment. The implications for clinical application, theory formulation, and research were discussed.

Adolescent↗

Awareness of negative social conditions among mentally retarded, emotionally disturbed outpatients.

Many mentally retarded people experience negative social conditions for long periods in their lives. These include the stigmatizing effects of being labeled mentally retarded, rejection and ridicule, segregation, infantilization, social disruption, restricted opportunities, and victimization. Many mildly and some moderately retarded people are highly aware of these negative social realities and can articulate them in detail during psychotherapy interviews; the authors describe three such patients. There is a need for research on the possible effect of prolonged exposure to negative social conditions on the mental health of mentally retarded persons.

Adult↗

Reproductive capacity of the mentally retarded.

Ninety-seven mentally retarded females were studied to evaluate parameters of growth and development of secondary sex characteristics and the incidence of ovulation. These patients were selected on the basis of age and etiological classification. Patients were classified as Down's syndrome, brain damaged, or undifferentiated. The following parameters of growth and development were studied: anthropometric, bone age, and sexual maturation. Sexual maturation studies consisted of breast development, pubic hair, axillary hair, menarche, menstrual pattern, and ovulatory patterns. The following observations were made from this study: 1. Physical growth and secondary sexual development were retarded in all parameters. 2. The undifferentiated and brain damaged groups showed almost identical patterns of growth and development of secondary sexual characteristics. 3. The Down's syndrome group was the most retarded in physical growth and in secondary sexual development. 4. Skeletal age was a better guide than chronological age or height and body weight to the prediction of development of secondary sex characteristics and age at onset of menarche. 5. In 26 mentally retarded females who had achieved menarche the incidence of ovulation was approximately 60 percent.

Adolescent↗

Maladaptive behavior in children with Prader-Willi syndrome, Down syndrome, and nonspecific mental retardation.

Although some genetic, mental retardation syndromes have well-described behavioral features, comparative studies have not yet assessed the relative uniqueness of these so-called phenotypes. Maladaptive behavior of 43 children with Prader-Willi syndrome was compared to age- and gender-matched children with Down syndrome and with nonspecific mental retardation. The Prader-Willi group showed more frequent and severe internalizing, externalizing, and total problem behaviors on the Child Behavior Checklist. Some problems were elevated in all groups, and 12 behaviors were significantly elevated in Prader-Willi subjects relative to both comparison groups. Seven behaviors predicted membership into the Prader-Willi group with 91% accuracy. Implications were discussed for research on behavioral phenotypes in general and for dual diagnosis in particular.

Adolescent↗

Fluoxetine treatment of depression in mentally retarded adults.

Six depressed mentally retarded adults were treated with fluoxetine in an open trial. Five patients significantly improved, and one patient partially improved, after treatment with fluoxetine. Their mean Hamilton Depression Rating Scale score was significantly reduced after treatment. These results suggest that fluoxetine is a safe and effective treatment for depression in mental retardation. Some potential advantages of using fluoxetine in this clinical population are discussed. Additional controlled research studies are needed to confirm these findings.

Adult↗

Enhancing the creative problem-solving skills of educable mentally retarded students.

60 educable mentally retarded students from 3 schools in a large urban school system received 9 wk. of instruction from an adaptation of the Productive Thinking Program. Their performance on measures of creative thinking was compared with that of 60 students who received no additional instruction. The conclusions drawn were that (1) special attention to creative thinking training for educable mentally retarded students may be quite effective and results stable over time and (2) the measures of fluency, flexibility, and originality are the most useful in assessing creative thinking for this group.

Child↗

A two base pair deletion in the PQBP1 gene is associated with microphthalmia, microcephaly, and mental retardation.

X-linked mental retardation has been traditionally divided into syndromic (S-XLMR) and non-syndromic forms (NS-XLMR), although the borderlines between these phenotypes begin to vanish and mutations in a single gene, for example PQBP1, can cause S-XLMR as well as NS-XLMR. Here, we report two maternal cousins with an apparently X-linked phenotype of mental retardation (MR), microphthalmia, choroid coloboma, microcephaly, renal hypoplasia, and spastic paraplegia. By multipoint linkage analysis with markers spanning the entire X-chromosome we mapped the disease locus to a 28-Mb interval between Xp11.4 and Xq12, including the BCOR gene. A missense mutation in BCOR was described in a family with Lenz microphthalmia syndrome, a phenotype showing substantial overlapping features with that described in the two cousins. However, no mutation in the BCOR gene was found in both patients. Subsequent mutation analysis of PQBP1, located within the delineated linkage interval in Xp11.23, revealed a 2-bp deletion, c.461_462delAG, that cosegregated with the disease. Notably, the same mutation is associated with the Hamel cerebropalatocardiac syndrome, another form of S-XLMR. Haplotype analysis suggests a germline mosaicism of the 2-bp deletion in the maternal grandmother of both affected individuals. In summary, our findings demonstrate for the first time that mutations in PQBP1 are associated with an S-XLMR phenotype including microphthalmia, thereby further extending the clinical spectrum of phenotypes associated with PQBP1 mutations.

Abnormalities, Multiple↗

Impact of sociodemographic characteristics on the identification rates of minority students as having mental retardation.

Ethnic disproportionality among students with mental retardation and relationships between disproportionality and sociodemographic factors were investigated. Using national data, we examined the effects of gender, ethnicity, and sociodemographic factors on the proportion of students identified as having mental retardation. Results indicate a clear association among ethnicity, gender, and mental retardation. Sociodemographic variables were also strongly associated with the proportion of students identified. A logistic regression model that included sociodemographic predictors was significantly better than models with gender and race alone. Findings indicate that both individual student characteristics and district sociodemographic characteristics were important in determining the likelihood of identification of mental retardation and that the impact of the sociodemographic characteristics is different for each gender/ethnicity group. Recommendations for future research are provided.

Child↗

Assessment and treatment of clinical fears in mentally retarded children.

Three moderately mentally retarded females ranging from 8 to 10 years of age were treated for long-standing fears. These children had refused to talk with or be in the same general vicinity of adults other than their parents, a few close family members, and to a lesser degree, their teacher. To establish an accurate criterion for successful performance on dependent measures, participants were matched on age, sex, and level of mental retardation with children having "normal" amounts of fear. Dependent measures included approaching and talking to strange adults as well as child ratings of overall fear. Participant modeling was given by the mother who provided a sufficient amount of physical and verbal prompts to ensure that an acceptable greeting of adults specified by the mother were made. These prompts were gradually faded out as treatment progressed. Treatment, which was given in a multiple baseline format across subjects, proved effective and gains in the reduction of fears were maintained at the six month follow-up.

Behavior Therapy↗

Novel JARID1C/SMCX mutations in patients with X-linked mental retardation.

X-linked mental retardation (XLMR) is a heterogeneous disorder that affects approximately 2 in 1000 males. JARID1C/SMCX is relatively new among the known XLMR genes, and seven different mutations have been identified previously in this gene [Jensen LR et al., Am. J. Hum. Genet. 76:227-236, 2005]. Here, we report five novel JARID1C mutations in five XLMR families. The changes comprise one nonsense mutation (p.Arg332X) and four missense mutations (p.Asp87Gly; p.Phe642Leu; p.Arg750Trp; p.Tyr751Cys) affecting evolutionarily conserved amino acids. The degree of mental retardation in the affected males ranged from mild to severe, and some patients suffered from additional disorders such as epilepsy, short stature, or behavioral problems. This study brings the total number of reported JARID1C mutations to twelve. In contrast to other XLMR genes in which mutations were found only in single or very few families, JARID1C appears to be one of the more frequently mutated genes in this disorder.

Amino Acid Sequence↗

Effective decision-making/problem-solving training with mothers who have mental retardation.

Nine mothers with mental retardation received group training in decision-making using vignettes describing child-raising situations. A multiple baseline procedure was used to assess the effectiveness of training in how to identify and apply decision-making steps to trained and untrained vignettes. Significant changes in step identification and use were observed and maintained for a month for both types of vignettes. Although training included nothing to improve the appropriateness of decisions, there was significant improvement for the high-risk situations used in training. Results suggest that parents with mental retardation are able to learn and apply basic steps of decision-making.

Adult↗

Longitudinal comparisons of families who have adopted children with mental retardation.

Families with adopted mentally retarded children were contacted 3 years after original study to assess longer term adjustment. Data from 80% of the original sample indicated continued good family adjustment on a number of measures, including the Holroyd Questionnaire on Resources and Stress. Only 2 of 44 children were no longer living with their adoptive/foster parents for reasons related to adjustment difficulties. In contrast, 12 of the families had adopted or were fostering additional children, 11 of whom had handicapping conditions. These data indicate a positive prognosis for adoption as a desirable option for the rearing of children with mental retardation.

Adolescent↗

Cytogenetic abnormalities including the marker X chromosome in patients with severe mental retardation.

Cytogenetic investigations of mentally retarded patients at the care and rehabilitation centres of the Department of Health and Welfare and at special schools have been in progress since 1972. A total of 2533 cytogenetic investigations have been carried out. A chromosomal abnormality was found in 20,6% of individuals investigated. Next to Down syndrome the Martin-Bell syndrome with the marker X chromosome occurred most frequently. This syndrome is found in the White, Black, Indian and Coloured population groups. In 15 families that have been identified, 56 males and 35 females (carriers) have the marker X chromosome. The importance and role of diagnostic/genetic investigations in mentally retarded individuals is outlined.

Chromosome Aberrations↗