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Dental health profile of a population with mental retardation in Israel.

The dental status and treatment needs of Israeli children and adults with mental retardation were studied in a random sample of 387 subjects. Four levels of mental retardation were represented: (1) educable (n = 70), (2) trainable (n = 92), (3) with mental retardation and behavioral problems (n = 106), and (4) with mental retardation and severe physical handicap (n = 119). The total age-adjusted DMF-T was 12.78 and differed significantly by behavioral group (p < 0.001); the M-T was 10.70 for the educable group compared with 5.52 for the group with mental retardation and severe physical handicap. Total treatment needs included a participant mean of 3.32 for restorations and 0.61 for extractions. Institutions having dental clinics had higher participant mean DMF-T, D-T, M-T, and F-T values (p < 0.05) compared with those institutions which had no clinics (16.04 vs. 9.74; 5.17 vs. 5.06; 9.45 vs. 4.16; 1.41 vs. 0.52). Age-adjusted CPITN scores significantly differed by behavioral group; the group with mental retardation and severe physical handicap had the highest CPITN 3 category mean score of 2.93 compared with x = 1.89 for the educable group; however, the educable group had the most sextants with no teeth (x = 2.48). Findings confirm high dental morbidity and significant oral health differences by behavior group, age, and dental clinic status. Advanced training is recommended for dental providers and the staffs of institutions to enhance oral health outcomes.

Adolescent↗

Psychometric properties of ADHD rating scales among children with mental retardation.

The validity of hyperactivity rating scales in children with mental retardation was evaluated. Forty-eight children with mental retardation were rated by parents, teachers and teaching assistants on rating scales measuring Attention Deficit/Hyperactivity Disorder (ADHD) as part of a related investigation. In addition, direct observations were conducted using the Abikoff Classroom Observation Code. The concurrent validity of each scale was examined. Scales completed by both teachers and teaching assistants were found to provide valid information for the assessment of ADHD in mentally retarded children. Results provided the best support for the ABC-C in the assessment of ADHD in mentally retarded children.

Attention Deficit Disorder with Hyperactivity↗

Wechsler Intelligence Tests: do we really have a criterion of mental retardation?

The definition of mental retardation offered by the American Association on Mental Deficiency refers to an IQ of approximately 70 or below. This is identical to the Wechsler criterion of a test performance two standard deviations (SDs) below the population mean. In fact, Wechsler tests have not supplied such a criterion, rather they have deviated from it by anything from .27 to a full SD. Having done without such a criterion for 40 years, we should consider exchanging it for one that is fixed in time and whose external validity is attested to by an accumulated body of evidence.

Child↗

[Fragile X syndrome. Clinical analysis of 300 Chilean patients with unspecific mental retardation].

BACKGROUND: Fragile X syndrome is the most important cause of sex linked mental retardation and the second of chromosomal origin, after Down syndrome. AIM: To apply the modified Hagerman score to patients with mental retardation and to relate clinical findings with cytogenetic and molecular diagnosis. PATIENTS AND METHODS: The modified Hagerman score was applied to 214 male and 86 female patients with mental retardation. The clinical variables in non fragile X and fragile X cases, determined by molecular and cytogenetic methods, were compared. RESULTS: The score in 210 non fragile X males was 10.5 + 3.7 (range 3-23), compared to 21.4 + 2.1 (range 19 to 23) in the four fragile X patients. All fragile X patients had mental retardation, attention deficits, hyperactivity disorders, hand biting and poor visual contact. Hand biting, flapping and perserving speech were observed in a significantly higher number of fragile X males. Only one of 86 females had fragile X syndrome: Her most relevant findings were a long face and high forehead, an attention deficit, hyperactivity and poor visual contact. No clinical differences with other mentally retarded females were found. CONCLUSIONS: Approximately 5% of institutionalized males with mental retardation have a fragile X syndrome.

Adolescent↗

Mental health services for persons with mental retardation: role of the advanced practice psychiatric nurse.

The term dual diagnosis is frequently used by mental health professionals to refer to people who have mental illness complicated by substance abuse. A less commonly recognized population are those individuals who have the dual diagnosis of mental retardation and mental illness. In this paper the author discusses (1) the mental health needs of persons with mental retardation and (2) the current state of services for them. There is a significant lack of appropriately trained professionals to help address the needs of this population. Traditionally, nurses have primarily been identified as providers of physical care. Advanced practice psychiatric nurses, however, are an underutilized group of professionals who have the capacity to assume a leadership role in clinical care, service coordination, and advocacy for individuals and families affected by mental illness and mental retardation.

Case Management↗

Labeling and discrimination of facial expressions by aggressive and nonaggressive men with mental retardation.

Researchers have demonstrated that people with mental retardation have deficits in processing facial expressions of emotion. The ability of 18 aggressive and 21 nonaggressive men with borderline to moderate mental retardation to label and discriminate facial expressions was investigated. Although results did not support the hypothesis that aggressive participants would have greater difficulty with emotion labeling and discrimination compared to their nonaggressive peers, they do suggest that aggressive individuals may have a negative emotional bias for facial expressions that are ambiguous to them. Results further suggest that adults with mental retardation are not able to label the six basic facial expressions with equivalent accuracy and provide evidence for the salience of the mouth when labeling and discriminating facial expressions.

Adult↗

[Mental retardation].

OBJECTIVE: This paper describes recent advances in the neurobiology of mental retardation, emphasizing new diagnostic resources provided by cytogenetics, molecular testing, and neuroimaging. SOURCES OF DATA: MEDLINE (January 2000 through October 2003), using the following key words: mental retardation, developmental disability, child, and adolescent. Search of the Pediatrics and New England Journal of Medicine websites using the key word mental retardation. The Online Mendelian Inheritance in Man (OMIM) database was searched for information on clinical genetics. SUMMARY OF THE FINDINGS: In October 2003, the number of genetic syndromes associated with mental retardation reached 1,149. Considering the genetic or environmental and congenital or acquired causes of mental retardation, current diagnostic investigation is able to detect the etiology in 50 to 70% of cases. CONCLUSION: Diagnostic evaluation should follow a stepwise approach in order to make rational use of the expensive tools of cytogenetics, molecular biology, and neuroimaging.

Adolescent↗

Research on mental retardation in Norway 1970-1980: a review.

The present paper reviews most of the research on mental retardation in Norway, published in 1970-1980. An important part of this work is represented by Hole's reports of experimental phenylketonuria (PKU) in the rat and studies on peptides and protein-associated peptide complexes in mental disturbances, including experimental studies of effects on brain function and behavior of a peptide fraction (factor 3 b 2). Among studies on diseases with mental retardation, are determinations of arylsulphatase A in cultured amniotic fluid cells and in amniotic fluid as measures of prenatal diagnosis of metachromatic leukodystrophy, reports on screening disorders of tyrosine metabolism and the occurrence of positive dye test in blind children and patients with physical and mental handicaps. Further, studies on the effects of antiepileptics on immunoglobulins are reported. In the fields of social medicine, psychology and psychiatry, only few studies have been published in international journals. Brief reports on Norwegian articles comprise studies on work and disability, social and psychological handicaps in special school pupils, psychosis in mental retardation, and some efforts to design psychological treatment programs for the mentally retarded. The majority of research on mental retardation or with relevance to this field in Norway, has either been supported by the Norwegian Council for Research on Mental Retardation, or the Norwegian Research Council for Science and the Humanities. Based on annual reports of these councils, and correspondence to centers involved in such research, we have collected reports published in the period 1970 to 1980. We have also received unpublished reports, but have chosen not to include most of them in this review, because of the difficulties for the readers to obtain these reports. The institutional and noninstitutional services for the mentally retarded in Norway have no formal connection with university or other research institutes. Nevertheless, most of the research papers have been submitted from such institutes. Most of the reports traced are in the fields of basic neurobiology, medicine, and psychology, and will be dealt with under these headings.

Animals↗

Degree of associative relatedness and sentence processing by adolescents with and without mental retardation.

Previous research has suggested that individuals with mental retardation are less likely than individuals without mental retardation to access and incorporate information about the relations between words of sentences in the representations of those sentences in memory (e.g., Merrill & Bilsky, 1990; Merrill & Jackson, in press). A cued recall study and a semantic verification study were conducted to determine whether the magnitude of this group difference could be made smaller by increasing the degree to which the words in the sentences were semantically related. In both experiments, individuals with mental retardation exhibited an ability to utilize contextual information to a greater extent when the words were related. In the highly related conditions, the differences between groups was virtually eliminated.

Adolescent↗

Mild mental retardation in children in a northern Swedish county.

A series of children with mild mental retardation (171 children, IQ 50-69) born in 1959-1970 in a northern Swedish county and registered by the Board for Provisions and Services to the Mentally Retarded were analysed retrospectively. The mean annual incidence of mild mental retardation in registered children alive at the age of one year decreased during the period 1959-1970 and for the whole period was 4.2 per 1000, compared with 3.9 per 1000 for severe mental retardation in the same period of time and area. Ten children once classified as mildly mentally retarded made considerable intellectual progress and after some time no longer needed special help from the authorities. There was a pronounced male excess in the total series (1.8:1) and in all the main aetiological groups. Only small differences were found in mean birth weights, gestational age and maternal age in the main aetiological groups. In the series as a whole, however, there was an increased frequency of children small for gestational age. Prenatal causes were considered relevant in 43 per cent, perinatal in 7 per cent, and postnatal in 5 per cent. In 2 per cent there was a psychosis. In 43 per cent the aetiology could not be traced. Genetic causes were found in 31 per cent; in 8 per cent there was a chromosomal aetiology, in a further 8 per cent a mutant gene disorder and in 15 per cent the aetiology were considered multifactorial, i.e. at least one first-degree relative also had mental retardation and in both cases the cause of the retardation was unknown. Thirty per cent of the children had associated CNS handicaps, epilepsy (16 per cent) being the most frequent.

Adolescent↗

Effects of verbal and physical prompts on external strategy use in children with and without mild mental retardation.

Effects of type of prompt on the use of external strategies were examined. Participants were 7-, 9-, 11-, and 17-year-old children without mental retardation and 11- and 17-year-old children with mental retardation (N = 272). They were given a task requiring memory for object placement and assigned to one of four conditions: no prompt, verbal prompt, physical prompt, verbal and physical prompt. In the physical and verbal prompt condition, 17-year-old children with mental retardation used strategies at the same rate as 17-year-old children without mental retardation. Eleven-year-old children with mental retardation used similar types of strategies as the 7-year-old children without mental retardation. Thus, strategy use of older children with mental retardation may be activated to the same level of children without mental retardation with prompts.

Adolescent↗

A biologic and genetic study of 40 cases of severe pure mental retardation.

The family history of 40 patients with severe "pure" mental retardation (MR) was studied to determine the incidence of mental retardation and dull-normal intelligence among relatives, probable etiologies and an empiric recurrence risk. Significant findings include: (1) an increased sex ratio (69% males) of propositi, (2) a significant proportion of patients with clinical manifestations besides MR, (3) virtually no consanguinity among parents, (4) a "positive" family history for over 1/2 of the propositi--about 37% of all children in the sibships were affected; about 21% of the full sibs were affected, (5) a higher number of offspring produced by dull persons and a lower number of offspring from retarded persons compared to two normal persons (6) a proportionately large number of affected children produced from matings involving one or two dull persons, (7) a tendency for dull to have additional dull children and mentally retarded parents to have further retarded children while normal parents with more than one affected child usually had further retarded children, (8) an incidence of affected parents of about 32%, and (9) an overall empiric recurrence risk of 14%. Several etiologies were discussed as possible causes of the condition(s) in this group: (unrecongized) environmental damage and/or maternal/fetal interaction; unrecognized chromosome abnormalities; the homozygous state of several different autosomal recessive gees: X-linked recessive mutations; autosomal dominat new mutations; and mutifactoral inheritance. It was concluded that the group was etiologically heterogeneous and although none of the probable etiologies could be excluded, it seemed reasonable to assume that autosomal recessive inheritance plays an important role in the etiology of severe "pure" mental retardation.

Adolescent↗

Anger, depression and self-concept in adults with mental retardation.

Self-report measures of anger, depression and self-concept that were designed for non-retarded children were adapted for adults with mental retardation. The measures were administered to 130 adults with mental retardation who lived in the community. Informants rated the subjects on shortened forms of the three emotional indices. Informant self-concept ratings were negatively correlated with ratings of anger and depression. On the self-report measures, subjects who reported low self-concept also reported high levels of depression. Mildly mentally retarded subjects were more likely to report anger than severe/moderately retarded subjects. Informant and self-report measures were significantly correlated for self-concept and depression, but not for anger. The results provide preliminary normative data on indices of emotional adjustment for community-based adults with mental retardation.

Activities of Daily Living↗

Screening for subtelomeric rearrangements in 210 patients with unexplained mental retardation using multiplex ligation dependent probe amplification (MLPA).

BACKGROUND: Subtelomeric rearrangements contribute to idiopathic mental retardation and human malformations, sometimes as distinct mental retardation syndromes. However, for most subtelomeric defects a characteristic clinical phenotype remains to be elucidated. OBJECTIVE: To screen for submicroscopic subtelomeric aberrations using multiplex ligation dependent probe amplification (MLPA). METHODS: 210 individuals with unexplained mental retardation were studied. A new set of subtelomeric probes, the SALSA P036 human telomere test kit, was used. RESULTS: A subtelomeric aberration was identified in 14 patients (6.7%) (10 deletions and four duplications). Five deletions were de novo; four were inherited from phenotypically normal parents, suggesting that these were polymorphisms. For one deletion, DNA samples of the parents were not available. Two de novo submicroscopic duplications were detected (dup 5qter, dup 12pter), while the other duplications (dup 18qter and dup 22qter) were inherited from phenotypically similarly affected parents. All clinically relevant aberrations (de novo or inherited from similarly affected parents) occurred in patients with a clinical score of >or=3 using an established checklist for subtelomeric rearrangements. Testing of patients with a clinical score of >or=3 increased the diagnostic yield twofold to 12.4%. Abnormalities with clinical relevance occurred in 6.3%, 5.1%, and 1.7% of mildly, moderately, and severely retarded patients, respectively, indicating that testing for subtelomeric aberrations among mildly retarded individuals is necessary. CONCLUSIONS: The value of MLPA is confirmed. Subtelomeric screening can be offered to all mentally retarded patients, although clinical preselection increases the percentage of chromosomal aberrations detected. Duplications may be a more common cause of mental retardation than has been appreciated.

Child↗

Pharmacotherapy of disorders in mental retardation.

This is a review of pharmacotherapy in children and adolescents with mental retardation from the perspective of DSM and ICD disorders. The existing research is reviewed in young people with mental retardation but, when data are lacking, we examined the literature from adults with mental retardation and from typically-developing children. The literature is discussed for each of the following disorders: ADHD, anxiety disorders, bipolar disorder, conduct disorder, depression, enuresis, schizophrenia, self injury, and tics and movement disorders. With the possible exception of ADHD, there is a woeful lack of empirical data on most of these disorders in young people with mental retardation. Clinicians will often be forced to extrapolate from data on adults having mental retardation and from typically-developing children. The best policy is probably to treat such patients cautiously, while gathering data on the effects of such therapy in the hopes of beginning a data base.

Adolescent↗

Detection of changes in naturalistic scenes: comparisons of individuals with and without mental retardation.

Abilities of individuals with and without mental retardation to search for and detect salient changes to naturalistic scenes were investigated using the flicker paradigm. Located in areas of central or marginal interest, changes involved an object's color, shape, or presence. Individuals with mental retardation required more time to detect changes of all types, and the magnitude of the group difference was more pronounced for marginal-interest changes. Supplemental eye-tracking data from 6 participants suggested that the basis of this effect was that individuals with mental retardation tended to maintain gaze in the region of central interest for longer periods of time prior to sampling the other areas of the scene. Implications for intelligence-related differences in visual attention are discussed.

Child↗

The neuropsychology of facial identity and facial expression in children with mental retardation.

We indirectly determined how children with mental retardation analyze facial identity and facial expression, and if these analyses of identity and expression were controlled by independent cognitive processes. In a reaction time study, 20 children with mild mental retardation were required to determine if simultaneously presented photographs of pairs of faces were pictures of the same person or of different people (identity matching), or to determine if the pairs of faces showed the same expressions or different expressions (expression matching). Faces of familiar and unfamiliar people were used as stimuli. For identity matching, reaction times were faster for familiar faces than for unfamiliar faces. For expression matching, there was no difference between familiar and unfamiliar faces. These results are consistent with neuropsychological findings from the general population indicating that analyses of facial expressions proceed independently from processes involved in establishing a person's identity. Our results suggest that the basic neuropsychological mechanisms that underlie cognitive processing of facial identity and facial expressions in children with mental retardation may be similar to those of people in the general population.

Child↗

Dissection of an inverted X(p21.3q27.1) chromosome associated with mental retardation.

In a 6 year old boy referred for mental retardation, fragile X syndrome was ruled out by cytogenetic and molecular analyses. Cytogenetic investigations revealed an inverted X chromosome (p21.3q27.1). A similar chromosomal rearrangement was detected in his mildly mentally retarded mother. Fluorescence in situ hybridization (FISH), using a panel of ordered YAC clones, allowed the identification of YACs spanning both the Xp21.3 and Xq27.1 breakpoints, where many non-specific mental retardation loci have been reported so far. Further investigations by FISH showed that the IL1RAPL1 gene at Xp21.3 was disrupted by the X chromosome inversion and therefore its inactivation may be related to the mental retardation observed in our patients.

Child↗