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PubMed1985

Hepatitis A and B in non-institutionalized mentally retarded patients.

The high incidence of hepatitis A and B in institutionalized patients with Down's syndrome is not fully understood. Under poor hygienic conditions immunological alterations might predispose to these infections. To minimize environmental influences, 125 patients with Down's syndrome (mean age 11.9 years) living at home with their families were studied for the occurrence of serological markers of Hepatitis A and B. 106 outpatients with mental retardation of other genesis (mean age 12.4 years), and 114 consecutive voluntary blood donors (mean age 18.0 years) from the same area served as controls. Evidence of previous hepatitis A virus infection was found in 5.6% of Down's patients, in 9.4% of other mentally retarded patients, and in 16.7% of healthy controls. Evidence of previous or ongoing hepatitis B virus infection was a common finding in both groups of mental retardation (Down's syndrome 20.0%, other mentally retarded patients 11.3%) in sharp contrast to healthy blood donors (0.9%, p less than 0.05). Patients with Down's syndrome, however, revealed a much higher incidence of HBs-antigenemia as compared with other mentally retarded patients (12.8% vs. 2.8%, p less than 0.01). All HBs antigen-positive cases had normal transaminase levels and no overt clinical signs of liver disease, suggesting an asymptomatic carrier state. These data indicate that hepatitis A is not a special risk for mentally retarded outpatients, while hepatitis B virus infection is hyperendemic even in not-institutionalized patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1993

Anthropometric and craniofacial patterns in mentally retarded males with emphasis on the fragile X syndrome.

Anthropometric and craniofacial profile patterns indicating the percent difference from the overall mean were developed on 34 physical parameters with 31 white, mentally retarded males (23 adults and 8 children) with the fra(X) syndrome matched for age with 31 white, mentally retarded males without a known cause of their retardation. The fra(X) syndrome males consistently showed larger dimensions for all anthropometric variables, with significant differences for height, sitting height, arm span, hand length, middle finger length, hand breadth, foot length, foot breadth, and testicular volume. A craniofacial pattern did emerge between the two groups of mentally retarded males, but with overlap of several variables. Significant differences were noted for head circumference, head breadth, lower face height, bizygomatic diameter, inner canthal distance, ear length and ear width, with the fra(X) syndrome males having larger head dimensions (head circumference, head breadth, head length, face height and lower face height), but smaller measurements for minimal frontal diameter, bizygomatic diameter, bigonial diameter, and inner canthal distance. Several significant correlations were found with the variables for both mentally retarded males with and without the fra(X) syndrome. In a combined anthropometric and craniofacial profile of 19 variables comparing 26 white fra(X) syndrome males (13 with high expression (> 30%) and 13 with low expression (< 30%), but matched for age), a relatively flat profile was observed with no significant differences for any of the variables. Generally, fra(X) syndrome males with increased fragile X chromosome expression have larger amplifications of the CGG trinucleotide repeat of the FMR-1 gene. No physical differences were detectable in our study between fra(X) males with high expression and apparently larger amplifications of the CGG trinucleotide repeats compared with those patients with low expression. Our research illustrates the use of anthropometry in identifying differences between mentally retarded males with or without the fra(X) syndrome and offers a comprehensive approach for screening males for the fra(X) syndrome and selecting those individuals for cytogenetic and/or molecular genetic testing.

Adolescent↗
PubMed1997

High-resolution mapping and transcript identification at the progressive epilepsy with mental retardation locus on chromosome 8p.

Progressive epilepsy with mental retardation (EPMR) is an autosomal recessive central nervous system disorder characterized by childhood onset epilepsy and subsequent mental retardation. The locus for EPMR has been mapped to human chromosome 8p23. We recently reported the construction of a YAC contig across the 4 centimorgan minimum genetic region that harbors the disease locus. We now report further delineation of the critical region to <700 kb. Our mapping strategy relied on the identification of nine novel microsatellite markers and the construction of a complete BAC contig across the critical region. Several partial gene sequences have been identified from the region and are being analyzed as candidate genes for EPMR.

Base Sequence↗
PubMed2006

Mutational screening of ARX gene in Brazilian males with mental retardation of unknown etiology.

ARX gene mutations have been known as important causes of developmental and neurological disorders and are responsible for a large spectrum of abnormal phenotypes, includeing syndromic as well as nonsyndromic forms of mental retardation. We have screened the entire coding and flanking intronic sequences of ARX gene in 143 mentally impaired males in order to investigate the contribution of ARX mutations to mental retardation in the population of Rio de Janeiro, Brazil. Three sequence variants were identified: one patient had the most recurrent mutation already observed in ARX gene, the c.428_451dup(24 bp), two patients presented the c.1347C>T (p.G449G) in exon 4, and one patient had the intronic variant c.1074-3T>C. Although two of these alterations were considered polymorphisms, the known pathogenic variant c.428_451dup(24 bp) was found at a high rate (4.8%) among X-linked mental retardation (XLMR) families. Our results, the first in Latin America, reinforce the idea that ARX mutations are relevant to mental retardation and are indicative that molecular screening of exon 2 should be considered in males with mental retardation of unknown etiology, associated or not with neurological manifestations, especially in familial cases.

Brazil↗
PubMed1998

[Health and health services for the mentally retarded before and after the reform].

Reform of the health services in Norway for the mentally retarded led to a complete closure of all institutions for mentally retarded persons by the end of 1996. All 109 of the mentally retarded persons in the county of Sør-Trøndelag who were previously institutionalized were observed throughout this process, in 1987 and 1995. Both physical handicaps and psychiatric/behavioural problems had worsened. The carers' knowledge of their clients' physical health was inadequate in more than half (55%) of the cases. Medical treatment needed improving in 36% of the cases, while it was clearly deficient in 9%. There had been a reduction in the use of general medical services, as well as in the specialised services of psychologists, psychiatrists and neurologists. The organisation of services for this group of clients is in need of improvement.

Adult↗
PubMedDate not supplied

[Some aspects of measurement by Wechsler Intelligence Scale in the context of mental retardation diagnosis in adults].

The paper deals with the problem of diagnosing mental retardation in adults, with a focus on the numerical indicator of intellectual level (intelligence quotient). According to the present standards, the process of diagnosing mental retardation demands a comprehensive approach: an evaluation of both the intellectual level and social competence, and should be carried out by a team of experts (medical consultants, a psychologist, an educationist, etc.). The paper shows that, in the process of diagnosing, excessive attention should not be paid to the indicator of intellectual level, expressed by the intelligence quotient. According to the theory of psychological testing, this indicator is saddled with some error. Its use as the main criterion in diagnosing can lead to drawing false conclusions. The paper presents the Wechsler Adults Intelligence Scale WAIS-R(PL), which is the most common tool used by Polish psychologists in diagnosing the intellectual level. It also indicates the limitations and strong points of this test in the process of diagnosing mental retardation. The article is mainly addressed to medical doctors, as it contains information relating to the problem of measurement by tests, which is scantily dealt with during the medical studies.

Adult↗
PubMed1994

Detection of metabolic disorders among selectively screened people with idiopathic mental retardation.

Fifth-eight people receiving residential or other services for idiopathic mental retardation were evaluated for evidence of metabolic disease. Five (8%) demonstrated persistent urinary organic acid abnormalities on at least three occasions, which pointed towards specific genetic metabolic defects. Instigation of specific treatment programs may have improved the quality of life for one of these participants. Appropriate genetic counseling was provided but could have been instigated much earlier had these investigations been performed as part of a routine workup for idiopathic mental retardation. This pilot study suggests a need for evaluation of other similar populations with idiopathic mental retardation.

Adolescent↗
PubMed1991

The predictive value of microcephaly during the first year of life for mental retardation at seven years.

Of 41 term infants surviving to seven years of age with head circumferences (HC) - 3 SD, 21 were mentally retarded (IQ less than or equal to 70) at seven years. HC - 2 SD was associated with mental retardation in 11 per cent of children. Socio-economic status did not affect mental outcome of children with HC - 3 SD, but higher socio-economic level was associated with better outcome for those with HC - 2 SD or who were normocephalic. Mental retardation in children with HC - 3 SD was more common in children with additional pathology. Of 25 children with no other pathology, one-quarter were mentally retarded at seven years; this was more common in children of normal birthweight. Disproportionate head-to-height ratio did not significantly affect outcome, although short stature was associated with less favourable outcome for those with HC - 2 SD or who were normocephalic.

Brain Damage, Chronic↗
PubMed1997

Screening and diagnosis for the fragile X syndrome among the mentally retarded: an epidemiological and psychological survey. Collaborative Fragile X Study Group.

The fragile X syndrome is an X-linked mental retardation disorder caused by an expanded CGG repeat in the first exon of the fragile X mental retardation (FMR1) gene. Its frequency, X-linked inheritance, and consequences for relatives all prompt for diagnosis of this disorder on a large scale in all affected individuals. A screening for the fragile X syndrome has been conducted in a representative sample of 3,352 individuals in schools and institutes for the mentally retarded in the southwestern Netherlands, by use of a brief physical examination and the DNA test. The attitudes and reactions of (non)consenting parents/guardians were studied by (pre- and posttest) questionnaires. A total of 2,189 individuals (65%) were eligible for testing, since they had no valid diagnosis, cerebral palsy, or a previous test for the FMR1 gene mutation. Seventy percent (1,531/2,189) of the parents/guardians consented to testing. Besides 32 previously diagnosed fragile X patients, 11 new patients (9 males and 2 females) were diagnosed. Scoring of physical features was effective in preselection, especially for males (sensitivity .91 and specificity .92). Major motives to participate in the screening were the wish to obtain a diagnosis (82%), the hereditary implications (80%), and the support of research into mental retardation (81%). Thirty-four percent of the parents/guardians will seek additional diagnostic workup after exclusion of the fragile X syndrome. The prevalence of the fragile X syndrome was estimated at 1/ 6,045 for males (95% confidence interval 1/9,981-1/ 3,851). On the basis of the actual number of diagnosed cases in the Netherlands, it is estimated that >50% of the fragile X cases are undiagnosed at present.

Adolescent↗
PubMed2001

Risperidone for controlling aggressive behavior in a mentally retarded child: a case report.

Risperidone is an atypical antipsychotic agent with dopamine and serotonin antagonistic effects. It is an effective treatment for reducing aggressive behavior in adults with mental retardation. The use of risperidone in a severely mental retarded child with aggression is described. Risperidone was able to reduce the aggressive behavior in this patient. No serious side effect was found. This case illustrated that risperidone is effective and well tolerated in treating aggressive behavior in children with severe mental retardation. However, the anti-aggressive effect of risperidone in mentally retarded children remains to be seen in a larger sample-size study.

Aggression↗
PubMed1990

Cytogenetic survey for autistic fragile X carriers in a mental retardation center.

A cytogenetic survey of 67 individuals previously identified as having mental retardation and autistic behaviors revealed 1 person (1.5%) with the fragile X chromosome (fra[X]) and 3 (4.5%) with autosome abnormalities. This low prevalence of fra(X) indicates that most persons with fra(X) in this mental retardation center did not have autistic behaviors severe enough to be identified as a secondary psychiatric diagnosis. The presence of other chromosomal abnormalities is consistent with the known causal heterogeneity of autism in mental retardation populations.

Adult↗
PubMed1994

Critical flicker frequency of mentally retarded and normal persons.

Critical flicker frequency (CFF) of 40 men, 20 mentally retarded whose mean age was 22.0 yr. and 20 normal whose mean age was 21.5 yr., was measured under binocular viewing using the Lafayette Visual Perception Control with a display unit. Subjects had been previously tested for visual acuity and color blindness. Analysis showed a significant difference in CFF between mentally retarded persons and normal individuals, the former having lower CFF than the latter. This finding suggests lower perceptual sensitivity of the mentally retarded persons. Further research with provision for EEG recordings is suggested.

Adult↗
PubMed2003

Equally unequal: gender discrimination in the workplace among adults with mental retardation.

Gender discrimination in the work place has been widely reported. Women are usually discriminated against both with respect to level of occupation and salary. The current study explored the correlation between gender and employment among adults with mental retardation, specifically, whether gender discrimination in the work place is as prominent among people with mental retardation as in the general population. Level of occupation and salary earned were studied in 227 adults with mild and moderate mental retardation residing in institutions, hostels, and sheltered homes in Israel. The findings suggest a correlation between gender and employment similar to that in the general population. Women were found to be employed mainly in sheltered workshops and lower levels of occupation, and to earn significantly less than the men. However, closer examination of each work place revealed that within each level of occupation there were no significant gender differences in salary. The finding suggests that while women with mental retardation earn lower salaries than men, this is mainly the result of their lower level of occupation. Rehabilitation efforts should therefore be directed toward ensuring higher levels of occupation as well as community employment among women with mental retardation.

Adult↗
PubMed2006

Rehabilitation of patients with conductive hearing loss and moderate mental retardation by means of a bone-anchored hearing aid.

OBJECTIVE: To evaluate whether the bone-anchored hearing aid (BAHA) can be applied successfully to patients with conductive hearing loss and moderate mental retardation. STUDY DESIGN: Retrospective clinical evaluation. SETTING: Tertiary referral center. PATIENTS: Twenty-two patients with congenital moderate mental retardation and conductive or mixed hearing loss were selected to receive a BAHA at the University Medical Centre Nijmegen, the Netherlands. Four of them were fitted despite a limited air-bone gap. INTERVENTION: Rehabilitative BAHA application. MAIN OUTCOME MEASURES: Implantation results, skin reactions, and audiological data were evaluated during a mean follow-up of 36 months. RESULTS: All the patients were still using the BAHA 7 days a week and for more than 8 hours a day after a follow-up period between 5 and 96 months. Two implants (9%) were lost due to insufficient integration but were reimplanted successfully. With the BAHA, mean free-field thresholds showed a clear mean improvement of 9 dB compared with the previous hearing aid. Considerable improvements in daily activities were seen in at least five patients. CONCLUSION: Moderate mental retardation should no longer be considered as a contraindication for BAHA application. Although implant loss was low, extra attention may be required from the personal care providers to maintain the percutaneous implant. The BAHA was well-accepted by the patients with moderate mental retardation and was being used for most of the day. Implementation of the BAHA as hearing aid treatment in patients with moderate mental retardation proved to be sufficiently effective and may have strongly positive effects on activities at school or at work.

Adolescent↗
PubMed1999

Patterns of respite use by aging mothers of adults with mental retardation.

Use of respite services by 275 aging families of adults with mental retardation was examined over a 4.5-year period. Although more than twice as many families used respite in 1993 compared to 1988, over half never used respite during this period. Most families using respite received in-home services, in moderate amounts, and with high levels of satisfaction. In an analysis of predictors of respite use, the only significant predictor in 1988 was poorer functional abilities of the adult with mental retardation. By 1993, respite use was predicted by three characteristics: poorer functional abilities, better health in the adult with mental retardation, and greater maternal caregiving burden. These findings reflect variability in aging families' needs for support and changing respite policies.

Adult↗
PubMed2003

Psychotherapy with persons who have mental retardation: an evaluation of effectiveness.

Studies of psychotherapy with persons who have mental retardation were examined. Studies conducted during a 30-year period were rated by an expert consensus panel and classified with regard to the nature of the research and outcome and effectiveness domains. We conducted a meta-analysis on a small number of the studies and found a wide range of research designs, types of interventions, and participants. The evaluation also pointed to a moderate degree of change in outcome measures and moderate effectiveness in terms of benefit to clients with mental retardation. We conclude that psychotherapeutic interventions should be considered as part of overall treatment plans for persons with mental retardation.

Adolescent↗
PubMedDate not supplied

Census enumeration of the mentally ill and the mentally retarded in the nineteenth century.

Enumerations of the mentally ill and mentally retarded were included in the six U.S. censuses conducted between 1840 and 1890. Inclusion of these categories reflected the new concern for the mentally ill and mentally retarded that was emerging at that time as part of a new social consciousness. Dr. Gorwitz analyzes these census results, considers the limitations of the data, and discusses the factors that led to discontinuation of the enumerations of the two groups after the 1890 census.

Persons with Disabilities↗
PubMed1990

[Prognosis in the mentally retarded child under the present conditions of institutional care].

The environment of mentally retarded children suffers from fluctuations of staff in institutes for social care. The reasons are subjective and in the organization of work. As compared with other countries, there are differences in the categories of workers of these institutions as well as in their numbers and training. The concept of care of mentally retarded children has not been fully elaborated in this country so far. This has adverse repercussions on the working results in social care institutes and on the health status of mentally retarded children.

Child↗