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Use of advance directives: facilitating health care decisions by adults with mental retardation and their families.

As increasing numbers of adults with mental retardation survive into adulthood and old age, they may face decisions about their health care and end-of-life treatment. Advance directives may serve as a tool for communicating one's preferences about future medical treatment. Information about the types, extent of use, and ethical context of advanced directives is provided and four critical issues regarding use of advanced directives by adults with mental retardation and their families are examined: assessment of decision-making capacity, standards for surrogate decision-making, family involvement in advanced directives planning, and constraints on the use of advanced directives. Implications for professionals are discussed, including ways to facilitate advance care planning.

Adult↗

Serum neurotrophin concentrations in autism and mental retardation: a pilot study.

To evaluate the availability of the serum neurotrophins for the diagnosis of the patients with neurodevelopmental disorder, we measured the serum concentration of brain-derived neurotrophic factor (BDNF) and neurotrophin-4 (NT-4) in the patients diagnosed with autism (n=18) and mental retardation (n=20), or healthy controls (n=16), using enzyme-linked immunosorbent assay. There tended to be a higher concentration of serum BDNF found in the autistic group ( P <0.05 by analysis of variance (ANOVA)) and the mental retardation group ( P <0.001 by ANOVA) compared to the control group. Serum NT-4 concentration tended to be increased in the mental retardation group (P <0.05 by ANOVA). We conclude that measuring the serum concentration of two neurotrophins, BDNF and NT-4, might be helpful to diagnose or classify disorders such as autism or mental retardation.

Adolescent↗

Referral reasons for psychological services for adults with mental retardation.

This study examined 391 adult referrals made to a mental health clinic serving individuals with mental retardation, in order to explore the relationship between referral source and referral reason. Each referral was categorized into 1 of 8 referral sources (e.g., vocational placements, day treatment programs) and into 1 of more of 10 behavior categories (e.g., depression, externalizing behavior problems). Externalizing behaviors were the most common overall referral reason. However, referral reason did vary as a function of referral source. Also, sex and mental retardation level were associated with referral reason. Implications for assessment practices and future research are considered.

Adult↗

Hemoglobin H disease and mental retardation: a new syndrome or a remarkable coincidence?

Each of three families of northern European origin contains a mentally retarded son with hemoglobin H (Hb H) disease. One parent is a carrier of mild alpha-thalassemia and the other is normal, suggesting that this form of Hb H disease results from the interaction between an inherited defect of alpha-chain production and one member of the pair in chromosome 16 and a new mutation on the other. Restriction-enzyme analysis indicated that the new mutation was not the same in the other three patients, and demonstrated at least two hitherto undescribed lesions involving the alpha-globin gene cluster. Unless the association between the Hb H disease and mental retardation is fortuitous, the new mutations may also be related to the development changes in these children. Since the mutations only came to light because there was concurrent inheritance of an additional alpha-thalassemia determinant, this type of mutation of chromosome 16 may have been overlooked in other mentally retarded patients.

Adult↗

The A140V mutation in the MECP2 gene is not a common etiological factor among Brazilian mentally retarded males.

In mammals, methyl-CpG binding proteins play a significant role in the control of gene expression through their association with chromatin-remodeling complexes. Mutations in the gene coding for methyl-CpG-binding protein 2 (MECP2) cause Rett syndrome and have also been reported in a number of X-linked mental retardation diseases. In this study, DNA samples from 363 male individuals with syndromic and non-syndromic mental retardation and other psychiatric diseases were screened for A140V (419C>T) mutation in the MECP2 gene, considered the most frequent MECP2 mutation in males. No 419C>T was found suggesting that the A140V mutation in the MECP2 gene is not a common cause of mental retardation in males. Recently, a new and abundant isoform of MECP2 was described, which has an alternative N-terminus, translated from exon 1, that was previously thought to be non-coding and has been excluded from many mutational screening, as well, the 5' and 3' UTR regions. We consider essential proceeding further screening in the whole extension of the MECP2 gene using clinically well-documented and larger sized sample to assure the overall contribution of MECP2 to mental retardation.

Adolescent↗

Social ecology of the workplace: contextual variables affecting social interactions of employees with and without mental retardation.

The social interaction patterns of employees with and without mental retardation across several different competitive employment sites were observed directly; narrative recording procedures were used. The data were analyzed to determine whether there were differences between the two groups of employees and the people involved in the interactions and the time period of the interaction and whether the interaction involved a social or task-related topic. The results were discussed in relation to their implications for integrating workers with mental retardation into settings with nonhandicapped workers.

Adult↗

Similarities in negative priming between persons with and those without mental retardation of equal chronological age.

Adolescents with and those without mental retardation participated in a negative priming procedure in two experiments. They identified letters to stimulus displays presented in pairs. Negative priming was observed as the slowing of response times when the distractor in the first display (prime) became the target in the second display (probe). In the standard procedure, all displays include one target and one distractor. In our modification, prime displays occasionally included a distractor without a target. Although adolescents with mental retardation typically do not exhibit negative priming when responding on the basis of stimulus identity, they did so with single letter primes in these experiments.

Adolescent↗

[Mental retardation and epilepsy].

504 clients, all living in institutions for mentally retarded, were examined with regard to level of functioning. 123 clients had epilepsy and 381 did not. The epileptic patients had more motor and communication disturbances and needed more help with everyday activities. Epilepsy was important, but explained fewer of the observed differences than did the degree of the motility problems. Among the clients with epilepsy the frequency of seizure was important. More frequent seizure was associated with lower levels of achievement. Therefore mentally retarded persons with epilepsy should be thoroughly examined/optimally treated by a neurologist/paediatrician and assured a medical follow up prior to possible transfer to a lower level of care.

Adolescent↗

[Medical and psychosocial evaluation of a mentally retarded adult population].

BACKGROUND: This study is aimed at describing the psychological, psychopathological, medical and social aspects of a mentally retarded population being provided with care by AFANAS-Jerez affording the possibility of assessing this type of problem from an epidemiological standpoint. METHODS: A descriptive case series study is conducted to characterize a mentally retarded (MR) population. An assessment was made of the psychological, medical and social aspects. The degree of intelligence was analyzed using the Weschler Adult Intelligence Scale. The adaptive behavior, frequency, typology and seriousness of the behavior problems were assessed by means of the Service Planning and Individual Scheduling Inventory. A psychopathological screening examination was conducted. This study is rounded out with a social and medical evaluation by means of structured interviews. The information from the different areas was encoded and was analyzed using the EPIINFO v6.0 and C.I.A. programs. RESULTS: The average age of the mentally retarded population studied was 32.2 (S.D. 9.2 years). Seventy-three percent (73%) males as compared to 27% females. Behavior problems were found to exist among 45.1% of the subjects. We have set up a statistical relationship between the existence/non-existence of said problems and the degree of MR, such that the greater the loss of intellectual capacity, the greater the probability of having behavior problems. One of the psychopathological symptoms worthy of special mention is the anxiety found to exist among 60.2%. The medical examination reveals the large number of problems related to with MR, we thus finding 22.48% of the subjects to have epilepsy, nearly 20% Down's Syndrome, etc. Also worthy of special mention is the low educational level of the parents and the family conflicts resulting from the child's retardation. CONCLUSIONS: Special mention must be made of how MR is a highly complex whole entailing a large number of related medical, psychological, psychiatric and social disorders, this being a situation brought to fore in this study, many of the disorders revealed to exist being related to the degree of MR. We are of the opinion that an epidemiological approach to the study of MR may be of use for a better comprehension of this problem.

Adult↗

Theories of ability and the pursuit of challenge among adolescents with mild mental retardation.

Dweck (1991) distinguishes two different ways children can view their abilities. Children who have an 'incremental theory' of their ability believe that it is a changeable, increasable and controllable quantity. Those who have an 'entity theory' believe their ability represents a fixed, unchangeable trait. Children with an 'incremental theory' tend to display adaptive achievement behaviours such as pursuing challenging activities, whereas children with an 'entity theory' tend to avoid challenges. The present study examined the usefulness of this distinction in understanding the behaviour and affect of children with mental retardation in an achievement situation. Results from an attributional questionnaire showed that children with mental retardation were significantly less likely to possess an incremental theory of their abilities than children without retardation. However, experimental results showed that when the context highlighted an incremental theory of ability, children with mental retardation showed the same positive motivational response as children without retardation (i.e. they chose high levels of challenge and reported greater interest-enjoyment). One unexpected finding emerged: children with mental retardation showed a tendency to choose lower challenge levels after receiving verbally administered success feedback relative to neutral feedback.

Child↗

Family views on sterilization for their mentally retarded children.

Eighty-eight parents of adolescents or adults with mental retardation answered written and/or telephone questionnaires concerning sterilization and reproductive health choices for their children with mental retardation. The 36 parents whose children were still living at home were more concerned about the capacity for sexual activity in their disabled child than were the 52 parents whose children were living outside the home. Otherwise, the two groups of parents were very similar in their attitudes. The reasons for considering sterilization were parental fear of sexual abuse, unwanted pregnancy and failure of birth control methods. Almost all the parents in this study wanted to participate in decisions concerning the sterilization of their disabled child.

Activities of Daily Living↗

On being called "mentally retarded": do developmental and professional perspectives limit labeling effects?

When children are labeled "mentally retarded," adults sometimes respond to their failures in ways that stimulate learned helplessness. The generalizability of such labeling effects was assessed in this study. Participants were four groups of adults that varied in professional experience with retarded children: within each group, some subjects subscribed to the developmental position (that retarded and nonretarded children have similar reasoning and learning capacities when matched for mental age) and some to the difference position (that retarded children lag behind their nonretarded peers in such capacities even when matched for mental age) of mental retardation. All subjects made judgments about the failure of a "retarded" and an unlabeled child. Strong labeling effects were found among proponents of the difference position but not among proponents of the developmental position, as well as among those groups with relatively little professional experience. Results suggest that beliefs consistent with the developmental position and experience with retarded children both attenuate the potentially helplessness-inducing effects of the mentally retarded label.

Attitude↗

Professionals' perceptions of psychotropic medication in residential facilities for individuals with mental retardation.

Professional staff in four state facilities for individuals with mental retardation were surveyed to determine their perceptions, knowledge and opinions regarding the use of psychotropic medication. A large majority of the 377 respondents indicated that the physicians in their facilities were primarily responsible for medication-related decisions. Under ideal conditions, however, all professional staff and parents were seen as having a greater influence in the decision-making process. Aggression, delusions and hallucinations, self-injury, other psychiatric disorders, and anxiety were rated as disorders most likely to result in medication therapy. Behaviour modification was viewed as a suitable alternative to drug treatment for acting out and aggression. The professionals indicated that behavioural observation was the most influential assessment technique in current usage, followed by global impressions and informal diaries. Over 80% of the respondents perceived their preservice and inservice training on issues related to the use of psychotropic medication to treat behaviour problems as inadequate, with 96% of them desiring continuing education. These findings were compared to data from similar studies of populations with other disabilities, and suggestions for modifications in the current decision-making processes related to the use of psychotropic medication in institutionalized individuals with mental retardation are discussed.

Adolescent↗

Mental retardation in childhood: clinical and diagnostic profile in 100 children.

Mental retardation (MR) is present in 2-3% of individuals in the general population, either as an isolated finding, or as part of an underlying disorder. It is the reason for a substantial part of referrals of patients and families to the pediatric genetic counseling units. MR may be genetically determined, or due to environmental (including perinatal) influences. Despite the thorough examination, physicians often are unable to define the etiology, in at least 30-50% of cases. This report presents the experience with 100 consecutive children with mental retardation, admitted to the Section of clinical genetics of the University pediatric hospital in Sofia over one year period. According to the routinely used classification, the patients' disease states have been subgrouped into genetic (35), multifactorial (3), environmental (3), and of unknown etiology (59). The research protocol included careful dysmorphologic, neurologic and developmental assessment, as well as cytogenetic, biochemical and molecular genetic testing. The rate of the diagnostic yield of the whole group was 41%. The discussion includes clinical examples and literature reports.

Abnormalities, Multiple↗

Prevalence of mental retardation and developmental disabilities: estimates from the 1994/1995 national health interview survey disability supplements.

In 1994 and 1995, the National Health Interview Survey included a Disability Supplement (NHIS-D) to collect extensive information about disabilities among individuals sampled as part of annual census-based household interview surveys. Here we describe the development and application of operational definitions of mental retardation and developmental disabilities to items in the NHIS-D to estimate prevalence. In our analyses, we estimate the prevalence of mental retardation in the noninstitutionalized population of the United States to be 7.8 people per thousand (.78%); of developmental disabilities, 11.3 people per thousand (1.13%); and the combined prevalence of mental retardation and/or developmental disabilities to be 14.9 per thousand (1.49%). Differences in prevalence estimates for mental retardation and developmental disabilities and among people of various ages are explored.

Journal Article↗

Prevalence of mental retardation and developmental disabilities: estimates from the 1994/1995 National Health Interview Survey Disability Supplements.

In 1994 and 1995, the National Health Interview Survey included a Disability Supplement (NHIS-D) to collect extensive information about disabilities among individuals sampled as part of annual census-based household interview surveys. Here we describe the development and application of operational definitions of mental retardation and developmental disabilities to items in the NHIS-D to estimate prevalence. In our analyses, we estimate the prevalence of mental retardation in the noninstitutionalized population of the United States to be 7.8 people per thousand (.78%); of developmental disabilities, 11.3 people per thousand (1.13%); and the combined prevalence of mental retardation and/or developmental disabilities to be 14.9 per thousand (1.49%). Differences in prevalence estimates for mental retardation and developmental disabilities and among people of various ages are explored.

Adolescent↗

Hereditary paroxysmal ataxia with mental retardation: a clinicopathological study in relation to episodic ataxia type 2.

A case of hereditary acetazolamide-responsive paroxysmal ataxia with mild mental retardation in an autopsied Japanese man is described. His ataxic attacks had occurred for approximately 65 years since the age of 6. One of his daughters had severe mental retardation and epilepsy, and the other had paroxysmal ataxic attacks and mild mental retardation. Analysis of the subject's CACNA1A gene and that in his daughter revealed neither mutations nor CAG expansion. Neuropathologically, cortical degeneration consisting of the marked loss of Purkinje and granule cells was found exclusively in the cerebellar vermis. This was consistent with findings at autopsy for cases reported as spinocerebellar ataxia 6. In addition, there were minor anomalies, such as hypoplastic cerebellum and brainstem, heterotopic Purkinje cells, and cortical microdysgenesis of the temporal lobe. It is considered that the cerebellar cortical degeneration and the minor malformations found in the brain are closely related to one another, rather than having occurred independently.

Adult↗

Muscle power, locomotor performance and flexibility in aging mentally-retarded adults with and without Down's syndrome.

Longer life expectancy is resulting in increasing numbers of elderly adults with mental retardation (MR). The objective of the study was to compare lower limb isokinetic muscle power, locomotor performance and flexibility of aged adult mentally-retarded individuals with and without Down's syndrome (DS). Nine subjects with MR and DS (mean age 61), and sixteen subjects with MR and without DS (mean age 63), performed leg power testing on a Biodex dynamometer. Parameters measured were dynamic torque, dynamic torque % body weight, and average power % body weight. Functional performance tests including "Timed Get-Up and Go" and flexibility were also analyzed and compared. Results indicate that in knee extension and flexion isokinetic power the MR group without DS showed significantly higher scores than the MR group with DS. The functional performance of elderly adults with MR and DS was significantly impaired compared with MR adults without DS, although no differences were observed between the two groups in the flexibility tests. It was concluded that muscle leg power, and gross motor performance of elderly mentally-retarded individuals without Down's syndrome is better than in those with Down's syndrome.

Journal Article↗