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PubMed1989

A cytogenetic study of nonpolymalformed patients with mental retardation of clinically undefined etiology: application of a high resolution banding technique.

We performed a cytogenetic study on 140 nonpolymalformed patients with mental retardation of clinically undefined origin, using a high resolution banding technique, to determine how much chromosome abnormalities contribute to the etiology of this condition. A total of 15 patients (10.7%) were found to have autosomal or sex chromosomal abnormalities. Autosomal abnormalities included partial monosomy (5 cases), reciprocal translocation (one case), 13/14 robertsonian translocation (3 cases), unbalanced translocation (one case), inverted duplication of 15q (one case) and mosaic trisomy 21 (one case). Sex chromosomal abnormalities comprised structural rearrangement of the short arm of the X chromosome (one case) and 47, XXY in a pure or mosaic form (two cases). It should be noted that four out of the 5 cases of partial monosomy had subtle interstitial deletions, which might have been unidentified by the conventional G-banding method alone. In one case of the robertsonian translocation 46,XY,t(13;14)/45,XY,t(13;14), a small deletion was thought to have occurred in the cells with a chromosome number of 45. Comparison of clinical features of the 15 chromosomally abnormal patients with those of patients with normal karyotypes did not show any clinical parameter indicative of chromosome imbalance. These results suggest that a subtle chromosomal deletion is specific to mental retardation associated with few malformations. We believe that diagnostic evaluation of mentally retarded patients, even if nonmalformed, should include chromosome analysis using a high resolution banding technique.

Cells, Cultured↗
PubMed1988

The effects of two physical fitness programs designed for mentally retarded adults.

The purpose of this study was to design a systematic program of fitness training that enhances physical fitness of mentally retarded adults in sheltered workshops. The first phase of the study was a six-month program conducted by physical education graduate students while the second phase was four months in duration and led by workshop employees. Instructors followed a manual containing 48 lesson plans that were specifically prepared for mentally retarded participants. The physical fitness programs were evaluated using the Canadian Standardized Test of Fitness. MANOVA results revealed that both the six-month and four-month programs increased the physical fitness of the mentally retarded workers, although cardiovascular endurance improved only as a function of the four-month program.

Adult↗
PubMed1997

Hospital vs. outreach treatment of patients with mental retardation and psychiatric disorders: a controlled study.

The aim of this study was to investigate the effectiveness of specialized hospital treatment vs. outreach treatment of patients with mental retardation and serious mental illness. A total of 50 patients were randomly assigned to either the hospital treatment (n = 25) or the outreach treatment group (n = 25). The outcome measures included psychiatric symptoms, family burden, costs and hospital admissions. At most observation points (up to 28 weeks) and at all endpoints the two groups were equivalent with regard to psychiatric symptoms. The burden on carers did not increase significantly during the outreach treatment. Treatment costs were lower for the outreach treatment. Of the 25 patients who received outreach treatment, four had to be admitted to the specialized hospital. Aggressive behaviour, social competence and number of previous psychiatric hospitalizations were found to be predictors of treatment outcome. It is concluded that outreach treatment represents an effective and efficient alternative to hospital treatment for patients with mental retardation and psychiatric disorders.

Adolescent↗
PubMed1990

[Hepatitis B at an open institution for the mentally retarded. Immunogenic effect of a recombinant anti-hepatitis-B vaccine].

The diffusion of hepatitis B virus was investigated in an open institution for mentally retarded patients, where 24 patients with Down's syndrome (DS) and 94 with other types of mental retardation (OMR) were being cared for. The immunogenic response to a recombinant vaccine intramuscularly injected to the deltoid muscle at a dosage of 20 mcg in the months 0, 1 and 6 was also evaluated. Seropositivity for some hepatitis B serum marker was found in 19.4% of patients (12.5% of those with DS and 21.2% of those with OMR). HBsAg was found in 8.3% of patients with DS and in 4.2% of those with OMR. The seroconversion to antiHBs induced by the vaccine 8 months after the first dose was found in 72.3% of DS and in 100% of OMR. The poor immunogenic response in DS was attributed to age, as in patients younger than 20 years it was 91.6% while in those over that age it was only 33.3%. It was concluded that in open institutions for mentally retarded patients there is a risk of hepatitis B infection, also shown in closed institutions, and that recombinant hepatitis B vaccine is highly immunogenic in this population, although it has been found that in patients with DS the age has a very marked negative influence on the response to the vaccine.

Adolescent↗
PubMed2004

Familial hyperinsulinaemia associated with epilepsy and mental retardation--a syndrome of familial insulin resistance.

BACKGROUND: Variants of type A insulin resistance, characterized by female hyperandrogenism and 'acromegaloid' features, have been ascribed to genetic defects of the insulin receptor or post-receptor pathways via autosomal dominant or recessive inheritance patterns. Whilst a variety of congenital syndromes of insulin resistance are identified by their characteristic clinical phenotypes, an association with epilepsy and mental retardation has not previously been reported. CASE REPORT: We describe three female siblings (aged 12, 19 and 21) with fasting hyperinsulinaemia (116-443 pmol/l; normal range < 80 pmol/l) and mental retardation. Two siblings also have epilepsy. The eldest has features of severe insulin resistance with dyslipidaemia, acanthosis nigricans, 'acromegaloid features' and diabetes requiring high dose insulin therapy in combination with a glitazone. Their mother has fasting hyperinsulinaemia (113 pmol/l), mental retardation and epilepsy. None had clinical or biochemical features of hyperandrogenism or evidence of pigmentory retinopathy, deafness or renal insufficiency. Autoantibody screens were negative. Interestingly, there is no evidence of mental retardation or epilepsy among males in the family. CONCLUSION: This family suggests the presence of a yet-undefined syndrome of familial insulin resistance affecting female kindred. Further studies are being undertaken to clarify the genetic defects and mode of inheritance.

Adult↗
PubMed1987

Benefit-cost analysis of supported competitive employment for persons with mental retardation.

A review of the literature on unemployment rates among adults with mental retardation is presented and the societal impact of the number of adults with mental retardation who remain unemployed is discussed. The supported competitive employment model is presented as illustrative of a habilitation program allowing greater monetary returns to society than traditional adult service programs. A benefit-cost analysis of our supported competitive employment program, occurring during the period from 1978 to 1986, is presented from two perspectives: that of the consumer (i.e., the adult with severe disabilities) and that of the taxpayer. Results of the benefit-cost analysis indicate that supported competitive employment is a financially prosperous venture from both perspectives. That is, from the consumers' perspective, for every $1.00 relinquished in taxes, supplemental security income (SSI), and forgone workshop earnings, $1.97 was received in increased income; the net benefit per year was $3,894 consumer. From the taxpayers' perspective, for every $1.00 expended for the funding of supported competitive employment programs and in lost tax revenues realized by the provision of targeted jobs tax credits, $1.87 was accumulated in benefits; the net yearly benefit to the taxpayer was $4,063 per consumer. The authors conclude that supported competitive employment is a financially profitable venture for both consumers and taxpayers.

Adult↗
PubMed2004

Visual experience regulates transient expression and dendritic localization of fragile X mental retardation protein.

Fragile X syndrome is the most common form of inherited mental retardation and is caused by the loss of function of the Fragile X mental retardation protein (FMRP). FMRP is an RNA binding protein thought to play a key role in protein synthesis-dependent synaptic plasticity. The regulation of FMRP expression itself is also likely to be an important control point in this process. Here we used dark-reared/light-exposed rats to determine the role of experience in regulating FMRP levels in the visual cortex. We find that FMRP levels increase in the cell bodies and dendrites of visual cortical neurons after as little as 15 min of light exposure. Remarkably, FMRP expression in these neurons returns to baseline levels by 30 min of light exposure. These changes were post-transcriptional because the FMR1 mRNA levels remained constant over this time period. A transient increase in FMRP levels was also observed in synaptic fractions prepared from visual cortices of light-exposed animals. In contrast, alpha-calcium/calmodulin-dependent kinase II expression showed a sustained upregulation under these conditions. Finally, the increase in FMRP expression was inhibited by blockade of NMDA receptors. This tight temporal-spatial regulation suggests that FMRP plays a dynamic role in a distinct epoch of experience-dependent synaptic plasticity.

Animals↗
PubMed1975

Epidemiology of severe mental retardation in children: community studies.

Twenty-seven community studies of severe mental retardation, defined as IQ less than 50, were reviewed. The prevalence rate of this condition was about 4 per 1000 in older children; the rate was somewhat higher in males but did not vary by social class. About one-half of severely retarded children had significant associated handicaps. The cause of most cases of severe mental retardation is not known, but Down's syndrome accounted for one-sixth to one-third of cases and a small percentage were due to other chromosomal abnormalities, metabolic diseases, or infection.

Abnormalities, Multiple↗
PubMed1979

Speech skill levels and prevalence of maladaptive behaviors in autistic and mentally retarded children: a statistical study.

Forty-seven autistic and 128 mentally retarded children, ages 6 to 14, from a special school were assessed in terms of nine maladaptive behaviors and speech skill levels. The results indicated that the group of the mentally retarded children with withdrawal had significantly lower speech skill levels than the group of those without withdrawal, and the group of the autistic children with self-injury had significantly lower speech skill levels than the group of those without self-injury.

Adolescent↗
PubMed2003

Depression, anxiety, and relevant cognitions in persons with mental retardation.

We assessed depression, anxiety, and relevant cognitions in persons with mental retardation by administering modified versions of the Reynolds Child Depression Scale, the Beck Anxiety Inventory, the Automatic Thoughts Questionnaire, and the Cognitions Checklist to 46 persons with borderline to moderate mental retardation. Consistent with research with other groups, self-reports of depression and anxiety were highly correlated (r = .74) in these individuals, and cognitions were strong predictors of negative affect. Subscales measuring cognitions related to depression and anxiety were also highly related, limiting the "cognitive-specificity" hypothesis. Hierarchical multiple regression analyses offered mixed support for cognitive-specificity. We discuss the implications of these findings for the cognitive and affective assessment of persons with intellectual limitations.

Adult↗
PubMed2002

Early risk factors for mental retardation: role of maternal age and maternal education.

The predictive value of maternal age and education in relation to rates of administratively defined mental retardation in a 3-year birth cohort (N = 267,277) was studied. Low maternal education placed individuals at increased risk for both educable mentally handicapped (EMH) and trainable mentally handicapped (TMH) placements. Older maternal age was associated with increased risk of mental retardation, but for individuals with EMH, this age effect was only seen in the lowest education group. In terms of population-level risk, it was younger mothers with 12 years of education or less whose births were associated with the greatest proportion of mental retardation. From a public policy viewpoint, children born to mothers with low levels of education are an important group to target for prevention/early intervention efforts.

Adult↗
PubMed2003

Identification of two AGTR2 mutations in male patients with non-syndromic mental retardation.

Mutations in the coding region of the angiotensin II type 2 receptor gene (AGTR2) were recently identified to cause X-linked recessive mental retardation. We report a mutation screening of the AGTR2 gene in 57 Finnish male patients with non-syndromic mental retardation. We identified two mutations, a 62G-->T transversion, which leads to a substitution of glycine for valine (G21V) and a 157A-->T transversion, which causes a substitution of isoleucine for phenylalanine (I53F). The patients with AGTR2 sequence variants had severe/profound mental retardation, epileptic seizures, restlessness, hyperactivity, and disturbed development of speech.

Adult↗
PubMed1993

Localization of a gene responsible for nonspecific mental retardation (MRX9) to the pericentromeric region of the X chromosome.

Nonspecific X-linked mental retardation (MRX) includes several distinct entities with mental retardation but without additional distinguishing features. The MRX family reported here has been classified previously as MRX9. In this study, we performed linkage analysis of MRX9 with a panel of 43 polymorphic DNA markers dispersed over chromosome X. Two-point linkage analysis revealed lod scores of 3.52 and 3.82 at 0% recombination for OATL1 and MAOA, both located in Xp11.2-p11.4. Lod scores for linkage with PGK1P1, DXS106, and DXS132, all located in Xq11-q13, were 3.83, 3.82, and 3.52, respectively, all at 0% recombination. Multipoint linkage analysis showed two peaks with MAOA and DXS132/DXS106, respectively. Analysis of recombinational events indicated a position of the MRX9 gene between DXS164 and DXS453. These findings are compatible with a location of the MRX9 gene in the pericentromeric region of the X chromosome at Xp21-q13.

Adult↗
PubMed1998

Evaluation of a sexual abuse prevention program for adults with mental retardation.

Programs to teach sexual abuse prevention skills to persons with mental retardation have rarely been evaluated empirically, and typical evaluations are limited to assessment of the participants' knowledge rather than their performance of specific skills. In the present study, 6 adult women with mental retardation were trained in sexual abuse prevention, and performance was assessed using four separate measures: pretests and posttests of knowledge, verbal report, role play, and naturalistic probes. All women learned the skills but failed to exhibit them to criterion during the probes. We discuss the implications for further training and assessment of sexual abuse prevention skills.

Adult↗
PubMed1991

Developmentally based personality assessment of adults with mental retardation.

Difficulties in assessing emotional and personality disturbances in adults with mental retardation were discussed and an evaluation method overcoming some of these problems presented. Overshadowing, inappropriate equation of emotional symptoms with cognitive limitations, and failure to adjust for the differing functioning levels among persons with mental retardation when judging pathology were cited as reasons to include a specific developmental factor in assessing this group. Projective tests were cited in the literature as being effective techniques, and an apperceptive technique originally intended for use with children was presented as a means of adding the needed developmental factor.

Adolescent↗
PubMed1980

Event-related potentials evoked by sensory stimulation in normal mentally retarded and autistic children.

Evoked potentials (EPs) and slow potentials (SPs) were recorded during two sessions of sound (S) and light (L) conditioning (habituation: S alone; conditioning: coupling of SL; extinction: S alone after SL series). 125 children from 0 to 15 were examined: 82 exhibited signs of autistic behaviour and/or mental retardation; 43 were normally adapted. Two studies were performed. The first was an analysis of relationships: 65 clinical characters were noted for each child with behaviour scales and psychometric tests, 88 electrophysiological data were measured on averaged tracings. The second compared with t and chi 2 square methods the electrophysiological data of 3 groups clinically defined for age and typical syndrome. Results of the two studies supplemented each other. From the clinical point of view 3 major groups appeared: (1) autism, (2) mental retardation, (3) normal adaptation. From an electrophysiological point of view 2 major groups could be defined: (1) few conditioned EPs with small amplitude, generalized conditioned SPs, small unconditioned EPs, generalized unconditioned SPs, conditioning 'to time'; (2) many conditioned EPs, many conditioned rhythmic potentials, absence of generalized SPs, many localized vertex negative SPs (CNVs), large unconditioned EPs, no conditioning to time. Some differences were observed in generalized SPs, small and positive in mentally retarded children, negative or positive in autistic children. EP and SP data clearly help to differentiate pathological groups from a normal group but are insufficient to distinguish the autistic from the mentally retarded children.

Adolescent↗
PubMed1985

Psychotropic and anticonvulsant drug use by mentally retarded adults across community residential and vocational placements.

The prevalence of psychotropic and anticonvulsant drug use across a comprehensive continuum of community programs for mentally retarded adults was examined. Psychotropic and/or anticonvulsant drugs were administered to 48% of the sample. The hypnotic, antidepressant, antimanic, and anxiolytic medications were used by 6%-12%, the anticonvulsants by 48%, and the neuroleptics by 66% of those who were prescribed psychotropic drugs. Mildly mentally retarded persons used more neuroleptic, manic, and antidepressant medication. Severely and profoundly mentally retarded individuals used more anxiolytics and hypnotic drugs. Psychotropic and anticonvulsant drug intake varied little by sex. However, an institutional history was associated with higher levels of drug use. Seventy percent of those with an institutional history were prescribed drugs; in comparison, only 27% of those without such a history used medication. The percentage of individuals who used psychotropic and anticonvulsant drugs increased dramatically as both the vocational and residential placements became more restrictive. Possible toxic behavioral effects of psychotropic and anticonvulsant medication upon habilitative efforts were discussed. Future research issues were suggested.

Adolescent↗
PubMed1993

A scale of attitudes toward the application of eugenics to the treatment of people with mental retardation.

As part of a larger study of the attitudes of diverse samples towards the application of eugenics to the treatment of people with mental retardation, a 32-item summated rating scale was developed as a contemporary, brief, easy to administer and score, and psychometrically sound instrument. Data were collected and analysed that indicated satisfactory item characteristics and reliability, and initial support for the content and construct validities of the scale. Analyses of social desirability data revealed that scale scores were not influenced by the subjects' desire to adhere to socially desirable expectations. The scale should be useful for the investigation of questions concerning the formation, structure and correlates of attitudes toward the application of eugenics to the treatment of people with mental retardation, and the relationship of these attitudes to contemporary mental retardation policies and practices.

Adult↗