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Institutional tour effects on attitudes related to mental retardation.

The effects of an institutional tour on college students' attitudes toward the mentally retarded child, mentally retarded adult, state school for mentally retarded persons, and ward attendant at a state school for mentally retarded persons were investigated. Four separate Semantic Differential Scales were administered to 179 subjects prior to being given the alternative of touring a state school for mentally retarded persons (72 subjects took the tour). Pretest responses were used to divide subjects into initially positive or negative attitude groups. Posttest scores for each concept were then analyzed by a two-way classification mixed-models analysis of variance for unequal Ns. Results indicated significant positive effects on attitudes toward mental retardation as a function of the institutional tour.

Age Factors↗

Isokinetic torque, average power, and flexion/extension ratios in nondisabled adults and adults with mental retardation.

For successful placement of individuals with mental retardation into community environments, a minimal amount of functional strength is required to promote self-independence. Since muscular strength and power are commonly deficient in these groups, the purpose of this study was to compare isokinetic elbow flexion and extension peak torque (Nm), average power (W), and corresponding flexion/extension ratios of adults males with Down syndrome, with mental retardation without Down syndrome, and sedentary adult males without mental retardation. Subjects with Down syndrome (N = 9), mental retardation without Down syndrome (N = 13), and without mental retardation (N = 13) were tested for elbow flexion and extension peak torque and average power at 60 and 90 degrees/sec on a Cybex 340 isokinetic dynamometer. Results indicated: 1) subjects without mental retardation displayed significantly greater ( p < 0.01) peak torque and average power elbow flexion and extension than subjects with Down syndrome and subjects with mental retardation without Down syndrome at speeds of 60 and 90 degrees/sec, and 2) there were no significant differences between groups in peak torque and average power elbow flexion/extension ratios at both angular velocities. The results of the data analysis support the premise that adults with mental retardation are in need of exercise programs to improve muscular strength.

Adult↗

Syntactic complexity in the writing of students with and without mental retardation.

Students with and without mental retardation (ns = 45 and 60, respectively) were compared on nine measures of syntactic complexity in writing at three grade levels. A multivariate analysis of variance revealed significant differences for group, but not for grade level or group by grade level. Students without mental retardation scored significantly better than those with mental retardation on all components of syntactic complexity except clause length. These results support other research in which students with mental retardation showed significant deficits in written language. Implications for remediation were discussed.

Adolescent↗

Interaction between family violence and mental retardation.

Although family violence and mental retardation are both prevalent in today's society; very little research has been conducted to investigate the relationship between them. Characteristics that make individuals with mental retardation more vulnerable to family violence are discussed in the areas of child, adult, and sexual abuse. Common psychological effects of this trauma are then explored followed by implications for practice. Because family violence and mental retardation are both societal as well as personal issues, intervention and prevention efforts must occur at both a direct service level and a community/macro service level. With such intervention and prevention efforts, persons with mental retardation will receive superior service when dealing with issues related to family violence.

Adolescent↗

Effects of target probability and memory demands on the vigilance of adults with and without mental retardation.

The vigilance of young adults with mild mental retardation and without mental retardation was compared in two experiments in which observers performed two memory demanding, cognitively based, 60-minute tests. In Experiment 1, target probability was 5% or 30% with a 1.5-second event rate. Detection by observers with mental retardation declined more rapidly than did that of observers without mental retardation in both target probability conditions; further, their response criterion became increasingly more conservative. In Experiment 2, target probability was 10% or 30% with an 8-second event rate. Observers with mental retardation detected fewer targets under both target probability conditions. Subjects' response criterion differed as a function of level of intelligence from the onset of the vigil and did not change with time on task. Intelligence-related differences in vigilance were explained in terms of subjects' information-processing abilities.

Adolescent↗

Inhibition of return and negative priming by persons with and without mental retardation.

Persons with and without mental retardation who were matched on CA took part in three tasks: an inhibition of return task, a location negative-priming task, and an identity negative-priming task. Having participants perform all three tasks allowed us to correlate performance among the tasks and assess the various relationships among performance measures on negative priming and inhibition of return. The participants with mental retardation did not exhibit negative priming of identity. However, they did exhibit negative priming of location and inhibition of return. The participants without mental retardation exhibited all three effects. A different pattern of correlations was observed for the participants with and those without mental retardation. Possible reasons for this difference are discussed.

Adolescent↗

Facilitation and inhibition effects in visual selective attention processes of individuals with and without mental retardation.

Adolescents with and without mental retardation participated in a visual selective attention task. They were required to identify one of two letters presented to them on the basis of color. We manipulated the relation between target and distractor letters on successive trials. A target letter could have been a target on the preceding trial, a distractor on the preceding trial, or not appear on the preceding trial. Subjects without mental retardation exhibited facilitation when the target was identical to the target on the preceding trial and inhibition when the target was a distractor on the preceding trial. Subjects with mental retardation also exhibited facilitation when the target was identical to the target on the preceding trial but not when it had been a distractor on the preceding trial. The inefficient suppression processes may result in performance deficits for individuals with mental retardation across a variety of tasks.

Adolescent↗

A statewide survey of CMHC programs for mentally retarded individuals.

Although community mental health centers appear to have a mandate to serve mentally retarded children, adults, and their families, there is little evidence that adequate treatment actually is being provided. In a survey of the CMHCs in the state of Washington in 1979, the authors found that only about 1,500 of the estimated 22,500 retarded individuals with a potential for emotional disorder were actually being treated in the centers. Moreover, very few of the centers kept records of the intellectual ability of clients, and only about half had a staff specialist in retardation. The barriers to increasing the scope and frequency of services are discussed, as is the potential of continuing education training to improve mental retardation services in the mental health system.

Attitude of Health Personnel↗

Behaviour problems of the mentally retarded.

The behaviour profiles of 176 mentally retarded individuals from two reception centres and nine group homes were assessed. The correlations between behaviour and age, sex, degree of mental retardation, etiology of mental retardation and medical diagnosis were assessed using the Revised Child Behaviour Profile. The severity of behaviour disturbance did not vary with age or medical diagnosis. The moderately retarded subjects presented with more severe behaviour problems, such as aggression, than the severely mentally retarded subjects. The variable most predictive of behavioural problems was etiology of the disorder. Individuals with Down's syndrome had significantly fewer behaviour disturbances and those with autism and pervasive developmental disorder had significantly more behaviour disturbances than other subjects. A psychiatric disorder was found in 10.2% of the sample. The implications of these findings are discussed with respect to public policy.

Activities of Daily Living↗

Mutations in ARHGEF6, encoding a guanine nucleotide exchange factor for Rho GTPases, in patients with X-linked mental retardation.

X-linked forms of mental retardation (XLMR) include a variety of different disorders and may account for up to 25% of all inherited cases of mental retardation. So far, seven X-chromosomal genes mutated in nonspecific mental retardation (MRX) have been identified: FMR2, GDI1, RPS6KA3, IL1RAPL, TM4SF2, OPHN1 and PAK3 (refs 2-9). The products of the latter two have been implicated in regulation of neural plasticity by controlling the activity of small GTPases of the Rho family. Here we report the identification of a new MRX gene, ARHGEF6 (also known as alphaPIX or Cool-2), encoding a protein with homology to guanine nucleotide exchange factors for Rho GTPases (Rho GEF). Molecular analysis of a reciprocal X/21 translocation in a male with mental retardation showed that this gene in Xq26 was disrupted by the rearrangement. Mutation screening of 119 patients with nonspecific mental retardation revealed a mutation in the first intron of ARHGEF6 (IVS1-11T-->C) in all affected males in a large Dutch family. The mutation resulted in preferential skipping of exon 2, predicting a protein lacking 28 amino acids. ARHGEF6 is the eighth MRX gene identified so far and the third such gene to encode a protein that interacts with Rho GTPases.

Base Sequence↗

Some problems in the genetics of X-linked mental retardation.

X-linked mental retardation has recently become one of the most interesting genetic anomalies. Studying this group of conditions has led to many insights into the mechanisms involved in normal and abnormal gene actions in humans. Since the early 1980s, the number of disease entities for which the responsible genes could be localized on the X chromosome has increased from year to year; at the Ninth International Workshop on Fragile-X-Syndrome and X-linked Mental Retardation, 199 such disease units were counted (Hamel, 1999). Conventionally, these units were subdivided into two groups: syndromal and non-syndromal types. The syndro- mal types are characterized by external features, neurological signs, and/or metabolic anomalies. The non-syndromal types do not show such specific features; here, the X-linked mode of inheritance is the only indicator. Due to the reduced reproduction of mentally severely retarded males, a relatively high fraction of new mutants among cases of a specific type must be expected. It cannot be the purpose of the present short article to review sufficiently well the entire field; this would require a complete book. Rather, it is our intention to point to some open problems and possible ways for their solution.

Fragile X Syndrome↗

A syndrome with multiple malformations, mental retardation, and ACTH deficiency.

We report on a patient with severe pre- and post-natal growth retardation, moderate mental retardation, microcephaly, unusual face with marked micrognathia and cleft palate, minor skeletal abnormalities, atrioseptal defect, hypospadias, hearing loss, and secondary adrenal insufficiency due to isolated ACTH deficiency diagnosed at 7 years of age. Family history was negative. Adrenal insufficiency is an uncommon feature in multiple malformation syndromes and may thus serve as a diagnostic handle for recognizing other possible patients with a similar syndrome.

Abnormalities, Multiple↗

Ear disease and hearing sensitivity in mentally retarded children.

The population screened for mental retardation consisted of 12,882 children, 8 years old. One hundred and fifty-one mentally retarded children were clinically studied. No borderline case was included. One hundred and one children were randomly chosen to form a control group. On clinical examination the children were 9-10 years old. Tympanograms were obtained from 206 children (81.8%). The tympanograms in respect to 30.9% of ears were classed as abnormal in the case of mentally retarded children, but in only 17.8% of non-retarded children. The difference is statistically significant (P less than 0.01). Pure tone audiometry was performed in 213 children. Thirty children were tested in open sound field, and 9 children remained untested. The examinations revealed 31 children with decreased hearing sensitivity (24 with conductive impairment, 7 with sensorineural hearing loss). Three children with profound sensorineural hearing loss and 21 with conductive impairment were among the mentally retarded. The threshold values were also examined using two-way analysis of variance. The groups were classified according to findings in psychological tests and according to the clinical findings in relation to the tympanic membrane. These two sets of findings constituted the two independent variables. These two factors had a highly significant (P less than 0.001) effect, but their interaction was not significant. Mentally retarded children clearly have middle ear disease much oftener than non-retarded children, and such ear disease is accompanied by decreased hearing sensitivity.(ABSTRACT TRUNCATED AT 250 WORDS)

Acoustic Impedance Tests↗

FXR1, an autosomal homolog of the fragile X mental retardation gene.

Fragile X mental retardation syndrome, the most common cause of hereditary mental retardation, is directly associated with the FMR1 gene at Xq27.3. FMR1 encodes an RNA binding protein and the syndrome results from lack of expression of FMR1 or expression of a mutant protein that is impaired in RNA binding. We found a novel gene, FXR1, that is highly homologous to FMR1 and located on chromosome 12 at 12q13. FXR1 encodes a protein which, like FMR1, contains two KH domains and is highly conserved in vertebrates. The 3' untranslated regions (3'UTRs) of the human and Xenopus laevis FXR1 mRNAs are strikingly conserved (approximately 90% identity), suggesting conservation of an important function. The KH domains of FXR1 and FMR1 are almost identical, and the two proteins have similar RNA binding properties in vitro. However, FXR1 and FMR1 have very different carboxy-termini. FXR1 and FMR1 are expressed in many tissues, and both proteins, which are cytoplasmic, can be expressed in the same cells. Interestingly, cells from a fragile X patient that do not have any detectable FMR1 express normal levels of FXR1. These findings demonstrate that FMR1 and FXR1 are members of a gene family and suggest a biological role for FXR1 that is related to that of FMR1.

Amino Acid Sequence↗

General slowing of information-processing by persons with mental retardation.

On measures of speeded performance, persons with mental retardation typically respond more slowly than do those without mental retardation. A review of 45 published studies yielded 518 pairs of response times (RTs) in which each pair consisted of a mean RT for a group of persons with mental retardation for an experimental condition and the corresponding mean RT for a group of persons without mental retardation. The primary result was that RTs of individuals with mental retardation increased linearly as a function of RTs for persons without mental retardation in corresponding conditions. These results are consistent with the view that differences in processing speed between persons with and without mental retardation reflect some general (i.e., nontask specific) component of cognitive processing. Possible candidates for the general component were discussed.

Adolescent↗

Non-specific X linked mental retardation.

Non-specific X linked mental retardation (MRX) is mental retardation in persons of normal physical appearance who have no recognisable features apart from a characteristic pedigree. Review of published reports shows that there is clinical variability in the degree of mental retardation within families and genetic heterogeneity, based on gene localisation, between families. We propose a classification based on genetic localisation and a set of minimal clinical features that should be recorded in the hope of identifying possible specific phenotypes.

Genetic Linkage↗