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PubMed1992

K-ABC profiles in children with fragile X syndrome, Down syndrome, and nonspecific mental retardation.

Etiology-specific profiles of intellectual abilities were compared in three groups of males with mental retardation using the Kaufman Assessment Battery for Children (K-ABC). Subjects included 10 males with fragile X syndrome, 10 with Down syndrome, and 10 with nonspecific mental retardation who were equated on both mental and chronological age. Across all three groups, sequential processing was lower than simultaneous processing or achievement, and particular subtests (e.g., Gestalt Closure) were relative strengths. Although boys with Down syndrome showed less extreme patterns of domain strengths and weaknesses, they showed a significant strength in the Sequential Processing Hand Movements subtest. In contrast, the Hand Movements subtest was lowest of all K-ABC subtests for males with fragile X syndrome. Implications were discussed for more fine-tuned research and intervention efforts.

Achievement↗

PubMed1984

Issues in fertility control for mentally retarded female adolescents: II. Parental attitudes toward sterilization.

Knowledge of the issues involving sterilization for mentally retarded adolescents is limited. Data were obtained from 69 interviewed parents whose mentally retarded daughters were attending the Cincinnati Adolescent Clinic. (A companion paper focuses on the experience of these girls with sexual activity, sexual abuse, and contraceptive use.) Forty-six percent of the parents had considered sterilization for their daughters. Twenty-six percent were still seeking the operation. Interest in sterilization especially correlated with increased severity of retardation (P less than .005) and with difficulty teaching menstrual hygiene (P less than .005). Parents of mildly retarded females sought tubal ligation, and those with severely retarded daughters chose hysterectomy and stressed their concern about menstrual management. Eighty-five percent of the parents favored a state statute that would enable sterilization under certain conditions and limitations. In general, few parents supported either an extremely restrictive or permissive position about sterilization.

Adolescent↗

PubMed1994

Psychometric analysis and revision of the Mental Retardation Attitude Inventory.

Psychometric analyses of data from 230 individuals yielded a 29-item four-scale revision of Harth's (1974) original 50-item five-scale summated-rating inventory to measure attitudes toward individuals with mental retardation. Results showed (a) satisfactory item characteristics; (b) adequate reliability and homogeneity for the inventory; (c) support for the four-part multidimensional structure of the inventory; (d) adequate reliability, homogeneity, specificity, and independence of the four scales; and (d) initial support for the validities of the inventory and the four scales. Additional analyses revealed that overall inventory and scale scores were not biased by social desirability responding. The inventory should be useful for the investigation of questions concerning attitudes toward individuals with mental retardation and the relations of these attitudes to mental retardation policies and practices.

Attitude↗

PubMed1998

Understanding referential expressions in context: use of common ground by children and adolescents with mental retardation.

Listeners interpret utterances against the common ground, or network of presuppositions shared with the speaker. The first purpose of the study was to determine whether individuals with mental retardation use the major sources of common ground (i.e., physical copresence, linguistic copresence, and community membership) to resolve referential ambiguity. The second purpose was to determine whether they seek confirmation of their referent choices in accordance with the certainty of interpretation afforded by the common ground. The third purpose was to determine whether they signal noncomprehension when faced with ambiguity and common ground that is not informative. The final purpose was to evaluate the relationship between within-group variability in common ground use and measures of nonverbal cognition, receptive and expressive language, and social cognition. Participants were school-age individuals with mental retardation and typically developing children matched to them on nonverbal MA. Common ground use was examined in a role-playing task in which the participant responded to ambiguous utterances. Common ground was manipulated within participants. We determined whether referent selections were appropriate for the common ground, whether they were accompanied by confirmation requests, and whether noncomprehension was signaled. Both groups used all sources of common ground to resolve referential ambiguity at better than chance levels but were less successful in using community membership. Both groups also requested confirmation of their referent choices most often when the common ground was based on community membership. Both groups signaled noncomprehension when the common ground was not informative. Different aspects of common ground use were related to different predictors for the group with mental retardation.

Adolescent↗

PubMed1997

Signaling of noncomprehension by children and adolescents with mental retardation: effects of problem type and speaker identity.

Previous research has demonstrated considerable within-individual and within-group variability in the signaling of noncomprehension by persons with mental retardation. The first purpose of this study was to determine whether within-individual variability in such signaling was related to differences in the nature of the inadequate message and the identity of the speaker. The second purpose was to evaluate the relationship between within-group variability in noncomprehension signaling and measures of cognition, receptive and excessive language ability, speech intelligibility, and social cognition. Participants were school-age individuals with mild mental retardation and typically developing children matched to them on nonverbal MA. Noncomprehension signaling was examined in a direction-following task in which inadequate message type and speaker were manipulated. It was found that message type, but not speaker, influenced noncomprehension signaling, with no difference between the two groups. We also found that performance on a test of receptive language ability was the best predictor of noncomprehension signaling for persons with mental retardation.

Adolescent↗

PubMed1988

Community-based instruction with profoundly mentally retarded persons: client and public responsiveness.

Three experiments were conducted to evaluate the effects of a training program implemented in a community setting for teaching receptive language skills to profoundly mentally retarded persons. In Experiment 1, the program was implemented in a local department store and consisted of a least-to-most intrusive prompting paradigm and contingent consequences. The community-based training strategy was effective in teaching receptive identification of three objects to a profoundly mentally retarded adolescent. Additionally, generalized improvements occurred in other store locations, although cross-modal generalization in terms of changes in expressive skills did not occur. These results were replicated in Experiment 2 with two other clients in the same store, and in Experiment 3 with another client in an outdoor recreational area. Also, a questionnaire survey indicated that store employees in the first two experiments had very favorable reactions to the program. Results are discussed in regard to continued research with community-based training as a means of expanding educational opportunities for individuals who are profoundly mentally retarded.

Adolescent↗

PubMed1991

Mental retardation and psychiatric disorders.

Estimates of the prevalence of comorbidity of psychiatric disorders and mental retardation in community and clinical populations range from 14.3 to 67.3 percent. A wide variety of disorders have been reported in this population, including schizophrenia, depression, and, commonly, conduct disorder. The incidence of specific disorders appears to be related to the level of retardation and the concomitant presence of seizure disorder. Accurate assessment of psychiatric disorders in this population is difficult because mentally retarded patients have poor communication skills and because most diagnostic instruments were developed for persons of normal intellectual functioning. Treatment includes educational, behavioral, and pharmacological interventions, but guidelines for safe use of psychotherapeutic drugs are needed.

Comorbidity↗

PubMed1984

A syndrome with true anophthalmia, hand-foot defects and mental retardation.

A syndrome with true anophthalmia, facial anomalies, hand and foot defects and mental retardation, has been observed in three patients. This syndrome is different from the Lenz syndrome, which includes microphthalmia or anophthalmia with narrow shoulders, double thumbs, skeletal abnormalities such as clavicle thinning, dental, cardiovascular and urogenital malformations without mental retardation.

Abnormalities, Multiple↗

PubMed1997

Comparative mortality in mentally retarded patients in California, with and without Down's syndrome, 1986-1991.

BACKGROUND: The large database of the California Department of Developmental Services provides a data source for mortality rates in persons with mental retardation by age, sex, severity, cause and associated conditions. RESULTS: Data for 1986-1991 were used to determine age-related mortality rates in cases with and without Down's Syndrome, in three severity levels of mental retardation. Distribution data for the data-base population are also given. CONCLUSION: In both Down's and non-Down's patient groups excess mortality increased with severity of mental retardation at all ages. In both groups excess mortality tended to decrease with advancing age to age 35-39 years, and to increase at the older ages. The age increase in excess death rate at age 40 years and up was steeper in the patients with Down's Syndrome.

Adolescent↗

PubMedDate not supplied

[Mental retardation convulsions and cerebral atrophy; main neurological changes in Darier's disease].

Darier's disease is determined by an autosomal dominant gene and is clinically characterized by confluent hyperkarotic papules. Mental retardation may appear in up to 10-20% of patients. Convulsions, spinocerebellar tract degeneration, polyneuropathy, psychiatric disorders and cerebral atrophy occur with a low and as yet not precisely determined incidence. In the medical literature reviewed only two previous cases of cerebral atrophy associated with Darier's disease were found. This study describes six members of a family affected with an unusually high incidence of neurologic manifestations. The mother and her five sons showed skin lesions, which, on histological examination, revealed the characteristic pathological changes of Darier's disease. Two of the five siblings with convulsions and mental retardation underwent computed tomography of the brain and both patients revealed the classic findings in atrophy; moderate enlargement of the lateral ventricles and widening of the cortical sulci. Cerebral atrophy was correlated with mental retardation and convulsions.

Atrophy↗

PubMed1981

Kabuki make-up syndrome: a syndrome of mental retardation, unusual facies, large and protruding ears, and postnatal growth deficiency.

A previously unrecognized mental retardation malformation syndrome was observed in five unrelated Japanese children. Consistent clinical features included moderate-to-severe mental retardation, progressive dwarfism of postnatal onset, a peculiar facies characterized by long palpebral fissures, with eversion of the lateral third of the lower eyelids, arched eyebrows, broad and depressed nasal tip, large prominent earlobes, short fifth fingers, abnormal dermatoglyphics including absence of digital triradius c or d, and frequent otitis media in infancy. Inconsistent abnormalities included epicanthal folds, cleft or high-arched palate, widely spaced teeth, low occipital hair line, scoliosis, and dislocation of the hip joint. Neither familial occurrence nor parental consanguinity was noted. The etiology of the malformation syndrome remains unknown.

Abnormalities, Multiple↗

PubMed1999

Caring for adults with mental retardation: survey of family practice residency program directors.

In their core curriculum guidelines, the Society of Teachers of Family Medicine has recognized the importance of training family physicians in caring for persons with mental retardation. We mailed surveys to all family practice residency directors in the United States, questioning them about experiences and methods used to teach residents about health care needs of adults with mental retardation and the importance of this education. We found that 84% of programs provide residents with one or more experiences, and 60% instruct residents in this area. Most directors ranked this education as very important or important. There was no relationship between type or age of residency program and likelihood that residents were educated about mental retardation. The importance of this education is discussed.

Adult↗

PubMed2005

Subtelomeric study of 132 patients with mental retardation reveals 9 chromosomal anomalies and contributes to the delineation of submicroscopic deletions of 1pter, 2qter, 4pter, 5qter and 9qter.

BACKGROUND: Cryptic chromosome imbalances are increasingly acknowledged as a cause for mental retardation and learning disability. New phenotypes associated with specific rearrangements are also being recognized. Techniques for screening for subtelomeric rearrangements are commercially available, allowing the implementation in a diagnostic service laboratory. We report the diagnostic yield in a series of 132 subjects with mental retardation, and the associated clinical phenotypes. METHODS: We applied commercially available subtelomeric fluorescence in situ hybridization (FISH). All patients referred for subtelomeric screening in a 5-year period were reviewed and abnormal cases were further characterized clinically and if possible molecularly. RESULTS: We identified nine chromosomal rearrangements (two of which were in sisters) corresponding to a diagnostic yield of approx. 7%. All had dysmorphic features. Five had imbalances leading to recognizable phenotypes. CONCLUSION: Subtelomeric screening is a useful adjunct to conventional cytogenetic analyses, and should be considered in mentally retarded subjects with dysmorphic features and unknown cause.

Abnormalities, Multiple↗

PubMed1979

Staff members' attitudes toward the sexual behavior of mentally retarded residents.

Seventy-five staff members from four private residential facilities for mentally retarded adults were surveyed to elicit their attitudes toward the sexual behavior and sexual knowledge of residents in their care. Facility policies and programs applicable to residents' sexuality were also the subject of inquiry. Comparable attitudinal surveys and studies dealing with the sexuality of mentally retarded persons were reviewed in the context of the present findings.

Adolescent↗

PubMed1995

The law hath not been dead: protecting adults with mental retardation from sexual abuse and violation of their sexual freedom.

The extent to which three professional groups (law enforcement officers, licensing personnel, and sex educators/counselors) utilize legally relevant criteria when assessing the sexual abuse of an adult with mental retardation was examined. Subjects were randomly assigned to one of four experimental conditions that varied in terms of the ability of a woman with mental retardation to understand concepts involving the nature and consequences and right of volition in a sexual relationship. The results indicated significant effects for both treatment condition and group. Thus, more explicit, standardized criteria should be developed for professionals to utilize when assessing consent involving possible sexual abuse of adults with mental retardation.

Adult↗

PubMed1987

Relative importance of caries risk factors in Finnish mentally retarded children.

The relative importance of associations between caries occurrence and exposure to sugar-sweetened products, fluoride preventives, medication with drugs affecting saliva secretion and the standard of oral hygiene was assessed in 125 mentally retarded and 79 healthy 9-10-yr-old Finnish children. The evaluation of the relative importance of the associations was based on logistic regression analyses. For the mentally retarded children the most important determinant of caries risk was their poor standard of oral hygiene. Frequent use of sugar-sweetened snacks was found to be an important determinant of caries risk for both the mentally retarded and the healthy children.

Dental Caries↗

PubMed2001

Use of primed in situ labeling (PRINS) for the detection of telomeric deletions associated with mental retardation.

Rearrangements involving the telomeric regions of human chromosomes are often associated with mental retardation. These rearrangements, however, are difficult to detect using conventional cytogenetic techniques. We propose the use of primed in situ (PRINS) labeling as an alternative to fluorescence in situ hybridization because it is very fast, reproducible, and simple to perform. Sixty-five children with unexplained mental retardation were studied using PRINS technology; two of them were shown to have a telomeric deletion.

Base Sequence↗

PubMed1993

"Project lifestyle" and new forms of living for the mentally retarded in the local community.

Since 1890, representatives of the Ministries and Departments of Social Security in Norway, Sweden, Finland, Iceland and Denmark have been working to create a foundation and a framework for a common Scandinavian research and development project concerning the quality of life of the mentally retarded. In 1992, with financial support from The Nordic Council of Ministers, the exchange of information and the building up of a network was begun, involving those taking part in some 50 development projects in Scandinavian countries. The project has focussed on three themes central to efforts made on behalf of and with the cooperation of mentally retarded people in these countries: the mentally handicapped person in the local community, independence and the quality of life, and communication. The aim of the project is to establish a Scandinavian network of key persons in the field, to systematize and propagate promising developments and to cast light on differences and similarities between various Scandinavian models for research and development related to the mentally retarded.

Activities of Daily Living↗