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Responses and sustained interactions in children with mental retardation and autism.

Sustained interactions and responses to social bids made by children with autism and verbal-age-matched children with mental retardation were recorded in two naturalistic settings. Children with autism produced fewer positive responses and more "no responses" than children with mental retardation; both groups were more likely to make positive responses to adults and not to respond to other children. Furthermore, although the frequency of conversations was not different for the two groups, children with autism were significantly less likely to engage in sustained play compared to children with mental retardation. Results suggest that children with autism are able to master the more rote and need-oriented social skills, such as simple conversation, but may not develop other forms of social interactions, like play.

Adolescent↗

Generalized language learning by children with severe mental retardation: effects of peers' expressive modeling.

In this study, we investigated the conditions that contribute to observational learning of generalized language in children with severe mental retardation. Matrix-training strategies were used to teach 6 children with mental retardation to combine known words into two- or three-word utterances consistent with syntactic rules. Subsequently, the children learned two or more unknown words concurrently, inducing word-referent relations consistent with these word order rules. Generalized learning of responses not taught directly was shown to be under experimental control using a multiple baseline design across submatrices. Expressive modeling of only four or five responses was sufficient to promote recombinative generalization in the expressive and receptive modalities. Thus, 95% to 98% of subjects' learning was attributed to generalization processes. This study demonstrates how the efficiency of language training with children with mental retardation might be enhanced by coupling observational learning and matrix-training strategies.

Behavior Therapy↗

Differences in brain metabolites between patients with autism and mental retardation as detected by in vivo localized proton magnetic resonance spectroscopy.

We performed volume-selective proton magnetic resonance spectroscopy (1H-MRS) of the brain with a 1.5 T magnet in 28 patients with autism, and compared the results with those from 28 age-matched patients with unclassified mental retardation and 25 age-matched healthy children. Peaks for N-acetylaspartate, choline and creatine, but not lactate, were observed in each group on 1H-MRS. The N-acetylaspartate/choline ratio was lower in patients with mental retardation than in patients with autism and controls (P = .05, respectively). However, there were no differences in the N-acetylaspartate/ choline ratios between patients with autism and controls, and the N-acetylaspartate/creatine and choline/creatine ratios did not differ among the three groups. These results suggest that N-acetylaspartate is decreased in patients with mental retardation and that a disorder or dysfunction of neurons in the brain exists. There also appear to be differences in the brain lesions or dysfunctions found in patients with autism and mental retardation.

Aspartic Acid↗

The mental health professional, the mentally retarded, and sex.

Because of the departure of the mentally retarded from cloistered institutions and their placement in community living arrangements, community mental health professionals are being called on more than ever to help the retarded and their parents deal responsibly with issues of sexuality. The mental health professional who would counsel the retarded must first develop and appreciation of the unique medical and social issues that can affect the sexual expression of the retarded. These issues include sexual maturation, reproductive ability, contraception, sterilization, abortion, and sex education. The author, through a review of the literature, discuss these issues and offers guidance to counselors struggling with the difficult decisions concerning sexual expression among the mentally retarded.

Abortion, Induced↗

[Infection with hepatitis B and C viruses in the mentally retarded].

AIM: To study the prevalence of hepatitis C virus infection (HCV) and the associated risk factors in an institution for the mentally retarded, in addition to its relation with hepatitis B infection (HBV). METHODS: The presence of antibodies against the HCV (anti-HCV) and markers for the HBV was evaluated in 94 mentally retarded subjects admitted to a single institution. Information concerning the sex, age, length of admission, type and degree of mental retardation, history of sexual promiscuity, surgery and blood transfusions was collected in every individual. RESULTS: The prevalence of infection by HBV was 22.3%. Only one individual was detected as being positive for the HCV.

Adolescent↗

Assessment of depression in mentally retarded adults.

Depression and psychopathology were assessed in 110 adult patients (ages 18-71 years) classified as borderline, mildly, moderately, or severely mentally retarded. Patients completed modified versions of the Beck Depression Inventory, the Zung Self-Rating Depression Scale, the MMPI depression scale, the Thematic Apperception Test, and the Psychopathology Instrument for Mentally Retarded Adults. Clinicians and ward personnel rated the patients on the Hamilton Rating Scale for Depression and an informant version of the Psychopathology Instrument for Mentally Retarded Adults. The measures correlated significantly with each other and were consistently related to the diagnosis of depression.

Adolescent↗

Estimate of prevalence of mild mental retardation: methodological aspects.

This paper sets out from an epidemiological point of view problems of measuring the prevalence of mild mental retardation. These include the problems always met in prevalence studies, of the validity of the classification, the completeness of ascertainment and the estimation of the size and characteristics of the denominator population. They also include difficulties peculiar to the condition of mild mental retardation, including the choice of method of classification whether by IQ testing or administratively; the heterogeneous nature of the individuals so characterised; and the confounding effects of social and biological factors and the changes in the implications for the affected individual of the condition, depending on age, sex and environment. It is concluded that normally measurements of prevalence can only be carried out on school age children and that these will change with age, and that we probably know very little about prevalence or implications of mild mental retardation at other ages.

Adult↗

[Occurrence of hepatitis B and C among mentally retarded].

The objective of this study was to examine whether the prevalence of hepatitis C, like hepatitis B, is increased among the mentally retarded in Denmark. The prevalence of serological markers of hepatitis B and C was examined in an institution for the mentally retarded. A total of 126 out of 178 inhabitants (71%) with a median age of 49 years (range 23-78) participated. All subjects were anti-HCV-negative by third generation ELISA antibody test. A total of 45 (35.7%) subjects were anti-HBc-positive and 10 (7.9%) were HBsAg-positive. Among subjects with Down's syndrome (n = 20), 55% were anti-HBc-positive and 30% were HBsAg-positive as compared to 32% and 3.8% respectively among others. In conclusion, hepatitis C infection seems to be uncommon among mentally retarded persons in Denmark and the risk of acquiring infection not significantly increased as compared to that of the general population. The prevalence of serological markers for hepatitis B was high and comparable to previous studies in this population.

Adult↗

Increasing productivity rates in adult mentally retarded clients: the failure of self-monitoring.

A series of three studies investigating procedures to increase productivity rates of mentally retarded adults in a sheltered workshop are reported. In the first study, the use of a behavioral intervention program implemented in a small classroom consisting of prompting, social praise, and performance feedback was found to significantly increase the productivity rates of five mentally retarded adults. Study 2 showed that simply the change in environment from the large, open space workshop setting to the smaller classroom did not contribute significantly to improve production. Despite the gains evident in the first study, production rates were found to decrease to pretreatment levels once the intervention was terminated. Study 3 examined the use of self-monitoring to increase productivity and achieve generalization across settings. Results of that study found self-monitoring to be ineffective. Implications for future research are discussed in light of the failure of self-monitoring with the mentally retarded.

Adult↗

Prevalence of the fragile X syndrome in Yugoslav patients with non-specific mental retardation.

Mutations at two fragile sites, FRAXA and FRAXE, loci are caused by an expansion of a CGG/GCC trinucleotide repeat and are characterized by mental retardation. Here we report molecular screening survey of 97 unrelated individuals diagnosed with non-specific mental retardation (MR), which produced positive test for FRAXA in two boys and none positive for the FRAXE mutation. In addition, we studied allelic frequency distribution for the FRAXA locus in this group of mentally retarded patients, as well as in the 99 healthy subjects of Yugoslav population. The distribution of FMR1 CGG repeat size in both groups was similar: the most common allele contained 29 repeats (32.86% in the healthy population and 54.54% in MR population), followed by the allele with 28 CGG repeats (21.43% in the healthy and 12.2% in MR population). Premutation alleles with more than 45 repeats were not found in control nor in the MR group.

Adolescent↗

Understanding leisure services for individuals with mental retardation.

This paper was developed to promote understanding of the complex process of leisure service delivery for children and adults with mental retardation and encourage people to work together to enhance leisure opportunities in integrated settings. The meaning of several terms associated with leisure were clarified and limitations of past and existing leisure services presented. We discussed our belief that all individuals, including those with mental retardation, have the right to experience leisure. Suggestions were made for development of leisure services involving social integration with peers who do not have mental retardation, the facilitation of active participation, development of age-appropriate behaviors, provision of comprehensive leisure education services, encouragement of self-determined leisure participation, and systematic coordination and communication among practitioners and family members.

Humans↗

Effects of procedure on memory scanning of mildly mentally retarded adults.

The performance of mildly mentally retarded adults on recognition memory tasks was compared with that of nonretarded control subjects. Following Sternberg's (1969) procedure, material to be remembered was presented in a fixed-set procedure, during which subjects were tested repeatedly on the same well-learned set of material, and a varied-set procedure, during which they were only tested on a memory set once before having to learn a new set. Mean reaction times (RTs) in all groups increased linearly as the number of items in the fixed memorized set increased; but "no" RTs of retarded adults tested under the varied-set procedure did not show this pattern. There was a gradation of slopes for the linear regression functions of RT on memory set size in both procedures, from less steep for nonretarded adults to increasingly steep values for nonretarded children and retarded adults. These results suggest that retarded adults use different processing strategies in the two procedures and that rate of processing increases with increasing mental age.

Adolescent↗

Reconsidering the construct of mental retardation: implications of a model of social competence.

A historical overview of efforts to integrate social competence into a definition of mental retardation was provided. The existing AAMR definition was criticized for its incorporation of a muddled construct of adaptive behavior, which over-emphasizes psychopathology and underemphasizes social intelligence. A model of general competence was presented, and mental retardation was reconceptualized as a condition characterized by deficits in social, practical, and conceptual intelligence. Although such a conceptualization would be an improvement on the current definition, we argued that a definition of mental retardation based on social competence outcomes (i.e., need for support in playing critical social roles) would be preferable to a definition that is still tied too closely to psychometric criteria.

Adaptation, Psychological↗

Oligophrenin 1 mutations frequently cause X-linked mental retardation with cerebellar hypoplasia.

BACKGROUND: Mutations of oligophrenin 1, one of the first genes identified in nonspecific X-linked mental retardation (MRX), have been described in patients with moderate to severe cognitive impairment and predominant cerebellar hypoplasia, in the vermis. OBJECTIVE: To further delineate the phenotypic and mutational spectrum of the syndrome, by screening oligophrenin 1 in two cohorts of male patients with mental retardation (MR) with or without known posterior fossa anomalies. METHODS: Clinical examination, cognitive testing, MRI studies, and mutational analysis (denaturing gradient gel electrophoresis and direct sequencing) on blood lymphocytes were performed in 213 unrelated affected individuals: 196 patients classified as MRX and 17 patients with MR and previously detected cerebellar anomalies. RESULTS: Four novel oligophrenin 1 mutations were identified. In the MRX group, two nonsense mutations were detected. In the MR group, two mutations were found: a deletion of exons 16 to 17 and a splice site mutation. All patients shared characteristic clinical, radiologic, and distinctive features with a degree of intrafamilial variability in motor and cognitive deficits. CONCLUSIONS: Oligophrenin 1 mutations were found in 12% (2/17) of individuals with mental retardatin and known cerebellar anomalies and in 1% (2/196) of the X-linked mental retardation group.

Adolescent↗

The hazards of vinyl glove ingestion in the mentally retarded patient with pica: new implications for surgical management.

OBJECTIVE: To report experience with the treatment of complications of vinyl glove ingestion in mentally retarded patients with pica. DESIGN: A retrospective case series. SETTING: Two university-affiliated hospitals. PATIENTS: Five mentally retarded patients, 4 with a history of pica, who were admitted for the management of complications resulting from the ingestion of vinyl gloves. MAIN OUTCOME MEASURES: Type of complication, treatment and operative outcome. FINDINGS: The patients ranged in age from 26 to 46 years. One patient died while awaiting surgical consultation of massive gastrointestinal bleeding from a large gastric ulcer caused by a vinyl glove bezoar (VGB). Four VGBs were removed surgically. Endoscopic removal was difficult or impossible because the gloves had become hardened and matted. CONCLUSIONS: VGB should be considered in institutionalized mentally retarded people with a history of pica when they present with gastrointestinal symptoms. VGBs should be removed directly by laparotomy, gastrotomy or enterotomy. Endoscopic removal is not recommended.

Adult↗

A new syndrome of dwarfism, brachydactyly, nail dysplasia, and mental retardation in sibs.

We describe a new multiple congenital anomaly/mental retardation (MCA/MR) syndrome in chromosomally normal sibs. Both had microcephaly, a "coarse" face with synophrys, ear anomalies, type B brachydactyly, nail dysplasia, skeletal anomalies, dwarfism, and mental retardation. Their mother had nail dysplasia, mild mental retardation, and short stature. An uncle, a younger brother of the mother, died at 17 years of age and also had a "coarse" face, digital anomalies, dwarfism, and severe mental retardation. The malformation complex in this family apparently has not been described previously, and the manifestations of the patients do not correspond to those of any known malformation syndrome. The disorder may be attributable to the pleiotropic effect of an autosomal dominant or an X-linked semidominant gene.

Abnormalities, Multiple↗

Lost once, the Fragile X Mental Retardation protein is now back onto brain polyribosomes.

The Fragile X Mental Retardation protein (FMRP) is an RNA-binding protein and its absence leads to the Fragile X syndrome, the most common form of inherited mental retardation. Because it has been acknowledged for a long time that FMRP is associated with polyribosomal mRNPs in all non-neuronal cellular systems studied so far, it is thought that it regulates translation in neurons also; however, its exact function remains elusive. Recently, it has been reported that, contrary to non-neuronal cells, brain FMRP is not associated with the translation machinery, but is part of repressed small RNP complexes excluded from polyribosomes.(27) To elucidate this puzzling result, Stefani et al.(17) and Khandjian et al.(32) have optimized methods to analyze brain polyribosomes and now provide definitive evidence for the association of FMRP with brain polyribosomes. In addition, the data presented in these two reports clearly indicate that FMRP's function resides at the translation control level.

Animals↗

Persons with mental retardation in nursing homes in 1977 and 1985.

Findings from the 1977 and 1985 National Nursing Home Surveys with respect to residents with mental retardation were compared. The single most notable finding was that very little changed over the 8-year period. The size of the mentally retarded population changed little (remaining at an estimated 40,500), the characteristics of the population remained essentially the same, and the services received by residents with mental retardation in 1985 remained at essentially the same low levels as those found in 1977. Implications of the findings as they relate to the initiative begun with the passage of P.L. 100-203 in 1987 and the forthcoming regulations to guide its implementation were discussed.

Activities of Daily Living↗