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Career paths through mental retardation services: an epidemiological perspective.

Children born during a five year period, resident in a British city, and administratively defined as mentally retarded were followed up at age 22. Age specific prevalence rates ranged from 2.5/1000 at age 5 to 14.7/1000 at age 11. The various mental retardation services for children and adults are described and the frequencies with which different paths through services were followed are given. Almost three-quarters of those who had been at a school for educable, mentally retarded children received no services in the young adult period. The distributions of various associated disabilities among those receiving different services after leaving school are given. These illustrate the multiple problems presented by those who continue to require services in adulthood. The epidemiologic data presented provide a basis for estimating service needs.

Adolescent↗

[Specifics in the treatment of epilepsy in children with mental retardation].

Authors discuss some specifics in treating epilepsies in children with mental retardation that have to be considered. First is the problem of precise diagnosis, because of certain phenomena in mentally retarded children, and a possibility of the so called pseudoretardation that can be caused by epileptic seizures or inadequate medication. A specific problem is vulnerability of these patients regarding antiepileptic drugs, especially phenytoin and phenobarbiton. Usage of psychopharmacs should be minimized and drugs interaction has to be considered permanently. A general trend in treatment should go towards increase in usage of carbamazepine and valproic acid, preferring monotherapy.

Child↗

Comorbidity of mental retardation and affective disorders.

Affective disorders in all forms do occur in persons with mental retardation. The presence and degree of mental retardation modify manifestations of these disorders. Diagnosis is difficult because of the absence of classical manifestations and frequent occurrence of inter-episode behaviour disturbances. Response to treatment may not be as favourable as in their normal counterparts.

Antidepressive Agents↗

Simulated and community-based instruction involving persons with mild and moderate mental retardation.

In this study of simulated instruction and/or community training of four functional living tasks was compared across groups of adolescents and young adults with mild and moderate retardation. Forty individuals participated in the assessment and training activities of this study, including 20 participants with mild retardation and 20 individuals with moderate retardation. A mixed factorial design was used to evaluate differences associated with level of mental retardation, simulated versus community-based instruction, and assessments in school and community settings. Participants with mild retardation performed better than their counterparts with moderate retardation on the simulated tasks and in the community settings. Participants with mild retardation were more successful in generalizing from the simulated instructional experiences to the community settings than were their counterparts with moderate retardation. However, in many of the situations, community training was sufficiently powerful to eliminate any advantage associated with some of the participants having experienced prior simulated instruction. Results are discussed in relation to the need to more closely examine the design and delivery of functional living skills instruction involving individuals with different levels of mental retardation. Specifically, the effectiveness of instructional simulations for teaching functional living skills does not appear to be uniform across level of retardation and targeted tasks. The highest priority question no longer appears to be whether or not simulated versus community-based instruction is more efficacious. Future research might more productively focus on the quality of different instructional simulations in combination with community assessment and/or training opportunities for teaching functional living skills to persons who experience different levels of mental retardation.

Adult↗

Seriation, conservation, and theory of mind abilities in individuals with autism, individuals with mental retardation, and normally developing children.

Seriation, conservation, and theory of mind abilities were examined in individuals with autism (N = 16), mental retardation (N = 16), and in normally developing children (N = 16). Seriation tasks included seriation of tubes, blocks, and flat squares. Conservation tasks included conservation of area, number, substance, quantity, and weight. Theory of mind tasks involved predicting false belief and understanding value and fact beliefs. Participants with autism performed better than participants with mental retardation on seriation, while no differences emerged between these groups on conservation and false belief. Individuals with autism performed less well than individuals with mental retardation on the value and fact belief tasks; however, when verbal ability was held as a covariant, the difference was no longer significant. Normally developing children performed better than the other two groups on all tasks. These results suggest that autism does not involve a specific impairment in theory of mind and that theory of mind deficits are not unique to autism.

Adolescent↗

[Digital computerized absorptiometry in the diagnosis of osteoporosis in a group of the severely mentally retarded].

BACKGROUND: The loss of bone mass after a certain age is a physiological fact that, in certain circumstances, is capable of inducing osteoporosis. In the diagnostic strategies for this condition, densitometry is a well-accepted technique now widely used. On patients with severe mental retardation, a number of circumstances come into play and may foster a greater frequency of osteoporosis. GOAL AND SCOPE OF STUDY: The goal of the study is to identify the bone mineral density values in a group of severe mentally retarded individuals using a digital densitometry technique in order to compare these with the general population and investigate the possible influence on these values of certain clinical variables and therapeutic regimens. VOLUNTEERS: One hundred and ninety-two patients of both sexes and treated at an institution devoted to the study and care of severely mentally retarded individuals. ACTIONS TAKEN: In all cases, the bone mineral density value has been obtained by means of digital densitometry using a compact desktop system using dual X-ray absorption measurement technology The data obtained have been contrasted with sex, age, degree of mobility and anti-epilepsy or sedative medication. RESULTS: In the population under study, the bone mineral density is significantly lower than that of the general population: 25% present osteopenia and 22% osteoporosis. These results are associated with the fact that they move little and are regularly taking phenobarbital medication. The population with Down's syndrome is also seen to be a group at risk. CONCLUSIONS: Osteoporosis is frequent among severe mentally retarded patients. The greatest risk is associated with insufficient mobility, Down's syndrome and regular medication with phenobarbital. Digital densitometry is a simple procedure that may be useful to identify the true dimension of this problem and the efficacy of the various preventive or curative procedures put forward.

Absorptiometry, Photon↗

Microcephaly and digital anomalies: a newly recognized syndrome of recessively inherited mental retardation.

We describe 2 brothers with congenital microcephaly and moderately severe mental retardation. The presence of identical and symmetrical digital anomalies suggests that this constellation represents a clinically recognizable recessively inherited mental retardation syndrome. Recognition of the clinical diagnosis should provide accurate genetic counseling to parents of a single affected child.

Abnormalities, Multiple↗

Enhancing the recognition and production of facial expressions of emotion by children with mental retardation.

The ability to recognize and respond appropriately to facial expressions of emotion is essential for interpersonal interaction. Individuals with mental retardation have problems not only in recognizing but also in accurately producing facial expressions of emotion. In Experiment 1, directed rehearsal was used to teach six boys with mild and moderate mental retardation to increase their ability to recognize facial expressions of emotion. In addition, their ability to produce the six basic facial expressions of emotion was periodically assessed throughout the study. The results showed that the boys' accuracy in recognizing facial expressions of emotion increased rapidly with instruction and that their increased accuracy was maintained at 8- and 12-week assessments following the termination of instruction. However, their increased levels of recognition did not generalize to the production of these emotions. In Experiment 2, four boys who had participated in the first study were provided with directed rehearsal training in the production of the six basic facial expressions of emotion. Their ability to produce facial expressions of emotion increased with instruction and was maintained following the termination of instruction. In addition, independent raters judged that the boys' production of these emotions matched the emotions that they were required to produce, suggesting a socially valid behavior change. These studies showed that the ability of children with mental retardation to recognize and produce facial expressions of emotion can be enhanced through instruction.

Adolescent↗

Implicit memory in aging adults with mental retardation with and without Down syndrome.

Effects of age and IQ on implicit memory in adults with mild or moderate mental retardation with and without Down syndrome were examined. When the etiologically defined groups were equated on age (and IQ), an age-associated difference in implicit memory performance was not evident. When data were reanalyzed, including only participants with unspecified mental retardation from a broader age range, we found a significant but small age-related difference and a significant but small IQ-related effect on implicit memory. In summary, although implicit memory showed an age-associated difference and IQ-associated variation in adults with mental retardation, these effects were relatively small. Data support existing theories proposing the relative invariance of implicit processes across a range of individual differences in age and intelligence.

Adult↗

Role relations between children who are mentally retarded and their older siblings: observations in three in-home contexts.

Older nonhandicapped siblings and same-sex younger mentally retarded siblings (n = 16 sibling pairs) were observed in their homes during toy play, snack, and television-viewing, as were an equal number of comparison sibling pairs, matched for age, gender, and family characteristics. Role relationships between mentally retarded children and older siblings were asymmetrical, with older siblings assuming frequent teacher, manager, and helper roles. Older sisters, in particular, participated in frequent teaching. Amount of interaction between siblings was directly related to the contexts in which they were observed. Nonhandicapped siblings engaged each other more as playmates. Interactional correlates of language and adaptive competencies of the mentally retarded children are presented, as are correlations between the age of the siblings and the roles they assume with each other.

Adaptation, Psychological↗

Ethical challenges in the conduct of research involving persons with mental retardation.

Researchers face challenges in balancing the demands of sound research methods and ethical standards in studies involving persons with mental retardation. While the autonomy of individuals must be respected, researchers also have an ethical responsibility to protect vulnerable individuals from social, psychological, or physical risks of research participation. Some individuals may face significant barriers in the research decision-making process due to cognitive limitations, lack of information and support, and limited experience and opportunities for exercising choice. The historical context of biomedical and behavioral research involving persons with mental retardation and current ethical issues related to informed consent and surrogate decision-making are examined. Implications for researchers and professionals are discussed, including ways to support individuals with mental retardation and their families in research decision-making.

Adult↗

Self-directed strength training: its effect on leg strength in men with mental retardation.

OBJECTIVE: To compare isokinetic measures of peak torque (newton-meters) and total work (joules) in men with mental retardation on a test of knee extension after 12 weeks of trainer-directed training and 1 year of self-directed training. DESIGN: Repeated measures of analysis of variance using a planned comparison approach involving an independent 2 x 3 (group x test) design. SETTING: Subjects were tested at a university athletic training facility on three separate days. SUBJECTS: Volunteer sample of 12 subjects with mental retardation: 6 in strength training group, 6 in control group. INTERVENTION: Subjects performed an isokinetic knee extension test at a velocity of 60 degrees/sec on three separate days. Test 1 was the pretest, test 2 was conducted after 12 weeks of trainer-directed hydraulic resistance training, and test 3 was conducted after 1 year of self-directed hydraulic resistance training. RESULTS: For both isokinetic parameters measured, the strength-trained subjects demonstrated significantly higher scores after 12 weeks of training and after 1 year of training than the scores achieved on the pretest. No significant differences in peak torque or total work scores were found between test 2 and test 3 scores for the strength-trained group. No significant changes in isokinetic scores between test sessions were found for the control subjects. CONCLUSION: Men with mental retardation can maintain strength following a self-directed hydraulic resistance strength training program.

Adult↗

Cognitive battery for differential diagnosis of mental retardation vs. psychosis.

Because of their severe cognitive and social deficits, seriously impaired and regressed psychotics are often misdiagnosed as mentally retarded. This diagnostic confusion, which carries dire consequences for treatment, has prevailed due to the lack of objective tests directed at this problem. Procedures are needed to specifically measure and differentiate the hallmarks of the intellectual dysfunction in both conditions (i.e., cognitive abnormality [psychosis] and subnormality [mental retardation]). Such methods also must be adapted to the particular problems and limitations of these populations. We propose here the use of three long established tests and a new developmentally rooted Cognitive Diagnostic Battery, one that assesses conceptual, perceptual-motor, and social maturity. Empirical study supported the validity of this Battery for differential diagnosis between mentally and functionally retarded psychotics matched for IQ, 97% of the developmentally disabled group exhibiting deficits on all three tests of conceptual development vs. 27% in the functionally mentally retarded group.

Diagnosis, Differential↗

Fragile X mental retardation syndrome: from pathogenesis to diagnostic issues.

The Fragile X (FRAXA) syndrome is the most common cause of familial (monogenic) mental retardation and is widespread in human populations. This syndrome is characterised by an unusual mode of transmission for an X-linked disease. In affected families, one frequently finds clinically normal transmitting males, whose daughters - also clinically normal - have a high risk of having affected children. The risk of developing the disease (penetrance) thus appears to increase in successive generations of the same family through maternal transmission. As shown by molecular cloning of the fragile X locus, Fragile X mutations are unstable expansions of a CGG trinucleotide repeat, located in the first exon (non-protein-coding) of the FMR1 gene (for Fragile X Mental Retardation). Two main types of mutation are observed in affected families. A full mutation is found in patients with mental retardation and corresponds to large expansions of the repeat. Premutations are moderate expansions and are found in normal transmitting males and in the majority of clinically normal carrier females. About 15% of patients show a mosaic pattern consisting of both full mutations and premutations. Although analysis of the CGG expansion has led to the establishment of reliable tests for diagnosis and genetic counseling of Fragile X syndrome, care must be exercised to use these tools to answer the concerns of the families and avoid doing harm. In our opinion, testing in children should be restricted to those who show a developmental delay, cognitive deficits and/or abnormal behavior evocative of the syndrome. A carrier diagnosis in a girl who is clinically normal should probably only be performed at an age where she can understand the consequences for family planning and the options of prenatal diagnosis. When testing children with borderline cognitive deficits, a positive diagnosis should be used to improve educational strategies for the children - and not to stigmatise them.

Child↗

Positive and negative psychotropic effects of lamotrigine in patients with epilepsy and mental retardation.

PURPOSE: To describe significant positive or negative psychotropic effects of lamotrigine (LTG) observed in epilepsy patients with mental retardation (MR). METHODS: Seven mentally retarded epilepsy patients, [5 with Lennox-Gastaut syndrome (LGS)] who experienced significant behavioral improvements or worsening after addition of LTG to their medication regimen were studied. RESULTS: LTG produced behavioral improvements in 4 patients. Patient 1, a 14-year-old girl, had LTG added to valproate (VPA) and thioridazine, resulting in diminished lethargy, less hyperactivity, and more appropriate speech. In a 17-year-old boy (patient 2) LTG added to VPA, phenytoin (PHT), and gabapentin (GBP) lessened irritability and hyperactivity. In patient 3, a 41-year-old woman, LTG added to PHT, VPA, and carbamazepine (CBZ) diminished lethargy and enhanced her social interactions. In patient 4, a 27-year-old man, LTG monotherapy diminished irritability and hyperactivity. Adverse behavioral effects were noted in 3 patients. In patient 5, a 43-year-old man, LTG added to PHT, phenobarbital (PB), lorazepam, sertraline, and thioridazine produced irritability, hyperactivity, and poor cooperation. In patient 6, a 29-year-old woman, LTG added to VPA produced frequent screaming, temper tantrums, increased rocking movements, and hyperactivity. In patient 7, a 29-year-old man, LTG added to VPA and PHT resulted in severe exacerbation of baseline behaviors, including self-injurious activity, temper tantrums, and failure to obey simple instructions. CONCLUSIONS: In some patients with epilepsy and MR, LTG has significant positive or negative effects on behavior.

Adolescent↗

A population-based study on epilepsy in mentally retarded children.

PURPOSE: This study presents data on cumulative risk of seizures, cause, comorbidity, and remission of epilepsy among mentally retarded (MR) children followed until the age of 22 years. METHODS: A total of 151 MR children were identified at the age of 8 or 9 years by screening four birth cohorts of 12,882 children born from 1969 to 1972 in the Finnish province of Kuopio. Information about epilepsy was gathered longitudinally when children were 9 to 10, 17, and 22 years old. The guidelines for epidemiological studies on epilepsy proposed by the International League Against Epilepsy were followed. RESULTS: By the age of 10 years, 29 of the 151 MR children (19%) had epilepsy. The cumulative risk for epilepsy at 22 years was 21%. The probability of developing epilepsy was increased fivefold in severely MR children compared with mildly MR children, i.e., in 27 of the 77 severely MR children (35%) versus 5 of the 74 mildly MR children (7%). Postnatal causes of mental retardation or association with cerebral palsy increased the risk for epilepsy, especially in the mildly MR children. When these risk factors were not present, the mildly MR children exhibited only a 3% risk for epilepsy, whereas the respective risk was about 10-fold in severe mental retardation. The cumulative probability of epilepsy being in remission for 5 years by the age of 22 was 32%. CONCLUSIONS: The cumulative risk of epilepsy varies according to the severity and the cause of the retardation as well as the presence of additional disabilities. The cumulative probability of epilepsy remission tended to increase with age.

Adolescent↗

Low significance of MECP2 mutations as a cause of mental retardation in Brazilian males.

MeCP2 is a protein that selectively binds to methylated cytosines through its methyl-CpG-binding domain (MBD) and connects DNA methylation to transcriptional repression. Mutations in MECP2 gene, located in Xq28, have been reported as being the major cause of Rett syndrome and are also associated with some cases of X-linked mental retardation in both males and females. In this study, we present the first screening in the MECP2 gene in a Brazilian cohort of 239 unrelated males with idiopathic mental retardation. Eight sequence variations were observed in 10 patients: one novel putative pathogenic variant, two never described variants of unknown pathogenic value and five non-pathogenic variations. We conclude that in mentally retarded Brazilian males, non-pathogenic variants in the MECP2 gene are more common than actual pathogenic mutations, and therefore alterations in this gene have a weak relationship with mental retardation in males.

Adult↗

Assessing the utility of atypical antipsychotic medication in adults with mild mental retardation and comorbid psychiatric disorders.

OBJECTIVE: Research on psychiatric outcomes among individuals dually diagnosed with mild mental retardation and co-occurring mental illness who are treated with antipsychotic medication is markedly limited due to difficulties encountered in (1) making valid and reliable psychiatric diagnoses and (2) accurately rating and following psychiatric symptom change over time in this specialty population. METHOD: To address these issues, DSM-IV psychiatric diagnoses were made by an experienced dual-diagnosis clinician, and the Aberrant Behavior Checklist (ABC) and the Global Assessment of Functioning were used to assess behavioral and psychiatric features in a psychiatric partial hospital setting. Data were collected by chart review from 72 patients admitted consecutively from January 1998 to December 1999. Assessments were compared at admission and discharge in this retrospective study for 3 treatment groups that were defined by antipsychotic medication status at discharge: no antipsychotic (N = 15), atypical antipsychotic only (N = 41), and mixed atypical/typical antipsychotics or typical anti-psychotic only (N = 16). RESULTS: Improvement on the ABC social withdrawal subscale was greater for atypical anti-psychotic medication-treated, dually diagnosed patients than for those who received other treatment regimens. In addition, a dose-response relationship was observed for this subscale and atypical antipsychotic medication dose. CONCLUSION: For certain psychotic patients with mild mental retardation, the atypical antipsychotics may be an appropriate and effective treatment modality.

Adult↗