Search

In many Middle Eastern countries, including Lebanon, there is a stigma attached to families who have an intellectually impaired child. These families complain of isolation and lack of community resources that could help them cope with their circumstances to optimize the child's abilities. Health professionals and researchers should be cognizant of factors related to the process of stress adaptation to help families cope with their circumstances. The aim of this cross-sectional study was to identify factors that play a role in mothers' adaptation to the care of their intellectually impaired children. The results, based on a sample of 127 mothers from Lebanon, reveal that a high percentage of mothers had depressive symptoms. Multiple regression analysis demonstrates that by order of importance, the factors that determine maternal depression are family strain, parental stress, and family income. The conclusions about nursing implications from a cultural perspective are discussed and recommendations proposed.

Background: Compared with that in mental illness, cultural variation in popular conceptualisations of intellectual disability has been rarely addressed., Methods: A survey of the relevant literature was conducted., Results and Conclusion: Preliminous conclusions are that local conceptualisation does not lead to invariant social response, but that intellectual disability is generally distinguished from mental illness.

Rett syndrome (RTT) is an X-linked progressive encephalopathy. Mutations in the MECP2 (methyl-CpG-binding protein) gene have been found to cause RTT. In the past few years, the role of MECP2 mutations in patients with mental disorders other than RTT has been studied, finding that mutations in MECP2 also contribute to non-syndromic entities. More recently, it has been demonstrated that RTT shares clinical features with those of Angelman syndrome, another neurodevelopmental disorder. These observations must be confirmed in a large series, to better understand the criteria needed for justifying a molecular test. Consequently, we have searched for MECP2 mutations in 294 patients (43 Angelman and Prader-Willi like included) with mental retardation (MR) of unknown aetiology. We found six polymorphisms (three novel, three previously reported) in 10 patients, one novel unclassified silent change (p.V222V) in a man, and one causative mutation in a girl with MR. Once this case was clinically reviewed, the girl presented symptoms of atypical RTT. The mutation (p.Y141C) lies within the methyl-binding domain, and has only been reported once in another atypical RTT. Our results show that the MECP2 mutations account for a low frequency (1/416 chromosomes = 0.24%) among mentally retarded individuals, which imply that it is necessary to perform an exhaustive clinical examination of patients before determining whether analysis of MECP2 is required or not.

Our aim in this study was to validate a stress-process model for Hispanic caregivers of adults with mental retardation that incorporates family functioning. The model postulates that maladaptive adult behaviors are related to poorer family relationships and higher levels of family burden, which in turn is related to caregiver psychological distress and self-reported health. The 153 Hispanic caregivers were interviewed in their homes with structured interviews and self-report measures. We analyzed cross-sectional data using structural equation modeling. The hypothesized model provided an excellent fit to the data. Also, family relationships mediated the relationship between maladaptive adult behaviors and family burden, and higher levels of family burden were related to greater caregiver distress. Acculturation was negatively related to caregiver distress.

Mental retardation is a common form of cognitive impairment among children. The underlying causes of mental retardation are extremely heterogeneous, and include significant genetic factors. The coexistence of neuropathology and cognitive deficits supports the view that mental retardation is a disorder of brain development and plasticity. Rab3A, a member of the Rab small G protein family, is a key molecule in modulating basal neurotransmission and contributes to synaptic plasticity. The RAB3A gene is located on chromosome 19p13.11, near a region shown by a linkage study to be involved in the etiology of mental retardation. Because of both its function and chromosomal location, RAB3A is a potential candidate susceptibility gene for mental retardation. To investigate the possible genetic contribution of the RAB3A gene, we performed a case-control association study focused on the Han population of northwestern China using four common SNPs in the gene (rs7259012, rs17683539, rs2271882, and rs874628). Pairwise linkage disequilibrium analysis showed that the four SNPs were in linkage disequilibrium. However, there were no significant differences of either allele or genotype frequencies at any of the SNPs nor any significant differences in haplotype distributions between cases and controls. In conclusion, we have found no evidence for RAB3A conferring susceptibility on mental retardation in the Han Chinese population.

The impact of caring for a child with a developmental disability on the physical and mental health of Latina and Black American women was examined. We used the National Health Interview Survey to compare the health of older mothers who were co-residing with a child who had a developmental disability to the health of same age mothers without caregiving responsibilities. Findings show that for both groups, older adult caregivers were more likely to report having limitations from arthritis than their noncaregiving counterparts. Caregiving was associated with more depressive symptoms for Latinas, but this relationship was not found for Black American women. Findings suggest that physical and mental health of caregivers need more attention in research and practice.

Background: This study examined whether behaviour problems and adaptive behaviour of low functioning young adults, and well-being of their families, varied by diagnostic syndrome [intellectual disability (ID) only, cerebral palsy, Down syndrome, autism], as well as by cultural group., Methods: Behaviour disorders in young adults with moderate to severe ID were assessed from information provided by 282 caregivers during in-home interviews. The sample consisted of 150 Anglo participants, and 132 Latino, primarily Spanish-speaking, participants drawn from Southern California., Results: Behaviour disorders and maternal well-being showed the same pattern across disability syndromes. Autism was associated with the highest scores in multiple behaviour problem areas as well as maternal reports of lower well-being. Down syndrome was associated with the lowest behaviour problem scores and the highest maternal well-being. When behaviour problems were controlled for, diagnostic groups accounted for no additional variance in maternal stress or depression. The pattern of behaviour problems and well-being did not differ by sample (Anglo vs. Latino), although level on well-being measures did. Latina mothers reported significantly higher depression symptoms and lower morale, but also higher positive impact from their child than did Anglo mothers., Conclusions: Caregivers of young adults with autism report more maladaptive behaviour problems and lower personal well-being, or stress, relative to other diagnostic groups, regardless of cultural group. However, cultural differences exist in caregiver reports of depression, morale, and positive perceptions. Implications for service provision aimed at families of children with challenging behaviour problems are discussed in the context of culture.

This study explores the self-concepts of Hong Kong Chinese with intellectual disabilities. Face-to-face and individual interviews were conducted in Cantonese, using the Chinese version of the Adult Source of Self-Esteem Inventory (ASSEI) together with three open-ended questions to explore the participants' self-conceptions in different life domains. An opportunity sample of 135 young adults with intellectual disabilities was interviewed. The findings showed that the family self, the social self and achievement in school and work were the self-concept attributes most important to the participants. The participants of this study had a higher total self-concept than that of a comparison group of people without disabilities when the participants used the in-group social comparison to maintain positive self-perception. The importance of partnership with family, self-concept enhancement strategies and quality employment service are discussed in order to facilitate people with intellectual disabilities to develop more positive self-concepts and thus achieve better community integration.

Background: Interest in the concept of quality of life (QOL) as a category guiding the development and provision of services for individuals with intellectual disabilities (ID) in Poland is increasingly being observed. Its presence in rehabilitation and care is strongly associated with the assessment of importance and use of QOL domains and indicators by Polish professionals., Method: The present study examined the differences related to selected demographic variables in the assessment of importance and use of eight QOL domains made by professionals in Poland working with persons with ID., Results: The evaluation of the importance and use of eight QOL domains and their indicators is related significantly to professionals' level of education, place of work, and total years of work., Conclusions: The perception of professionals cannot be overlooked when considering the development and provision of QOL-related services and supports.

Background: The concept of quality of life (QOL) is explained on the basis of traditional Chinese culture and contemporary social and cultural trends., Method: The importance of internationally recognized QOL domains and indicators was determined for three respondent groups: consumers, parents of the consumers, and teachers and rehabilitation professionals working with the clients., Results: Principal component analysis confirmed seven factors influencing QOL for people with intellectual disabilities (ID) in China. These seven components are conceptually equivalent to the eight domains found in the international QOL literature., Conclusion: The concept of QOL has become one of the most sensitive issues in present Chinese society and peoples' lives, and an important theoretical framework for the education and rehabilitation for people with ID in several ways: curriculum development, service delivery models, education and rehabilitation practices, and employment models.

Background: The Quality of Life Questionnaire (QOL-Q) is used widely to evaluate the quality of life of persons with intellectual disability (ID). Its validity for use with Spanish-speaking cultures has been demonstrated for individuals with visual disabilities, but not for those with physical or intellectual disabilities. Such was the purpose of the present study., Method: Two samples were administered the QOL-Q under standardized procedures. The first sample was composed of 209 Mexican participants with physical disabilities; the second was composed of 424 Spanish participants with ID. The hypothesis tested was: the applicability (i.e. etic properties) of the measure across countries and respondents would be demonstrated if reliability data and if factor composition were similar to the original measure. Cronbach's alpha was used to test reliability and exploratory factor analyses were used to test validity (i.e. factor structure)., Results: Data indicated that the reliability and factor structure was similar to that reported in the questionnaire's standardization manual and consistent with that reported in a number of Anglo-Saxon countries., Conclusion: The present study offers additional support for the valid use of the QOL-Q with Spanish-speaking populations.

We identified homozygous truncating mutations in HOXA1 in three genetically isolated human populations. The resulting phenotype includes horizontal gaze abnormalities, deafness, facial weakness, hypoventilation, vascular malformations of the internal carotid arteries and cardiac outflow tract, mental retardation and autism spectrum disorder. This is the first report to our knowledge of viable homozygous truncating mutations in any human HOX gene and of a mendelian disorder resulting from mutations in a human HOX gene critical for development of the central nervous system.

Background: The health and well-being of Indigenous people is a significant global problem, and Aboriginal Australians suffer from a considerably higher burden of disease and lower life expectancy than the non-Indigenous population. Intellectual disability (ID) can further compromise health, but there is little information that documents the prevalence of ID among indigenous populations. This study provides information on ID among the Aboriginal population of Western Australia., Methods: The Disability Services Commission (DSC) of Western Australia has maintained a statewide database of people with ID since 1953. Data on people of Aboriginal descent were extracted from the DSC database and linked to two other state-based databases, the Hospital Morbidity Data System and the Deaths Registry, with additional linkage to the National Death Index. The linked data were used to assess the prevalence, survival patterns and causes of death in Aboriginal people with ID., Results: Although comprising 3.5% of the population, Aboriginal Australians represented 7.4% of all people registered for ID services. The level of ID was assessed as borderline or mild in 40.7% of cases, moderate in 19.9%, severe or profound in 12.1%, but had not been specified in 27.2% cases. Median survival was 55.1 years for men and 64.0 years for women, with a mean age at death (n = 102) of 19.6 years. The leading causes of death were respiratory diseases, diseases of the circulatory system, and accidents., Conclusions: The study presents unique population summary data for ID in the Aboriginal community of Western Australia. To provide appropriate prevention and intervention strategies, there is an urgent need for more detailed information on the prevalence and patterns of ID.

The concept of quality of life is increasingly being used internationally in the field of intellectual disabilities. We surveyed three respondent groups representing five geographical groupings on the importance and use of the 24 core quality of life indicators most commonly reported in the international quality of life literature. Results suggest (a) similar profiles on importance and use across respondent and geographical groups, but differences in the frequency per response category; (b) significant differences in mean quality of life importance and use scores for both respondent and geographic groupings; and (c) factors on importance and use generally grouped into eight core quality of life domains. Results are discussed in reference to the etic (universal) and emic (culture-bound) properties of the quality of life concept.

The etiology of intellectual disability was studied both in incident (n = 9,432) and prevalent (n = 9,351) populations in a one-year birth cohort born in Northern Finland in 1985-1986. Data from multiple sources were used to follow the children until the age of 11.5 years. Of the incident cases (n=119) with intellectual disabilities, 66.4% had etiologically biomedical associative factor. Paranatal factors were relatively fewer and prenatal more common compared with earlier studies. We found nearly double the prevalence of genetic factors leading to intellectual disabilities compared with a contemporary study from Norway. The differences between the populations, despite random variation, some dissimilarities between etiological categorization and diagnostic accuracy, are in most part due to true differences between the study populations and genetic pool.

The social determinants of intellectual disability (ID) are poorly understood, particularly in Australia. This study has investigated sociodemographic correlates of ID of unknown cause in Western Australian born children. Using record linkage to the Western Australian Maternal & Child Health Research Database, maternal sociodemographic characteristics of children with ID (of unknown cause) born between 1983 and 1992 (n = 2871) were compared with those of children without ID (n = 236,964). Socioeconomic indices for areas based on the census district of mother's residence were also included in the analysis. Aboriginal mothers (OR = 2.83 [CI: 2.52, 3.18]), teenagers (OR = 2.09 [CI: 1.82, 2.40]) and single mothers (OR = 2.18 [CI: 1.97, 2.42]) were all at increased risk of having a child with mild or moderate ID. Children of mothers in the most socioeconomically disadvantaged 10% had more than five times the risk of mild and moderate ID compared with those in the least disadvantaged 10% (OR = 5.61 [CI: 4.42, 7.12]). Fourth or later born children were also at increased risk (OR = 1.82 [CI: 1.63, 2.02]). The results of the study have implications both for further aetiological investigation as well as service provision for children with ID. Furthermore, many of the sociodemographic correlates identified in this study, particularly in the mild/moderate category of ID, are potentially modifiable, opening up opportunities for primary prevention.

The aim of this study was to investigate the leisure and recreational activities of a cohort of 34 students attending a full-time special school as reported by the students and their parents. Leisure activities which were mostly solitary and passive in nature were identified as those being most commonly engaged in. Barriers to leisure were also identified, with 'access to' and 'location of' the leisure facilities being barriers perceived by both students and parents. Data on aspects of leisure use, e.g. enjoyment, participation, assistance required, socialization and choice, were also collected.

Background: Significant deviation from normal weight (obesity and underweight) and lack of physical exercise have been identified as three of the most significant global behavioural risks to health., Methods: Body mass index (BMI) and levels of physical activity were measured in a sample of 1542 adults with intellectual disabilities (ID) receiving supported accommodation in nine geographical localities in Northern England. Comparative population data were extracted from the Health Survey for England 1998 and 2001., Results: Men and women with ID living in supported accommodation are at increased risk of being significantly underweight and physically inactive. Women with ID living in supported accommodation are at increased risk of obesity. Within the population of people with ID living in supported accommodation increased behavioural health risks are associated with gender, severity of ID, age and location., Conclusion: Significant deviation from normal weight and lack of physical exercise are significant behavioural risks to health among people with ID.

Intellectual disability (ID) is a life-long disability characterized by impaired cognitive and adaptive skills. Over the past few decades, a shift has occurred in the conceptualization and treatment of people with ID and research in health policy and health-care delivery has become increasingly global with a notable disparity between the developed and developing world. This review presents a literature overview of global health policy for ID with the intent to focus specifically on the policy and treatment within Israel. The methodology involved sites visits to care centers, discussions with stakeholders in health policy, and a literature review. We believe that Israel is in a unique position between a developed and developing culture. In particular, the distinct problems faced by the Arab and Bedouin community in terms of ID must be formally accounted for in Israel's future policies. Research from the developing world would be instructive to this end. The global approach in this presentation led to certain policy recommendations that take into account the uniqueness of Israel's position from a social, economic, religious, and demographic perspective. It is the hope that this paper will lead to an increased awareness of the challenges faced by persons with ID and their providers in all sectors of Israeli society and that the necessary policy recommendations will ultimately be adopted.

Objectives: The aims of the present study were to examine health characteristics and healthcare utilization in relation to people with intellectual disability (ID) having psychiatric disorders in Taiwan., Methods: A cross-sectional study was employed; study subjects were recruited from the National Disability Registration Database. Taiwan, stratified by administrative geographical area for the study. Statistical analysis of 1026 carers for people with ID was made to examine the health status and healthcare utilization of individuals with ID having psychiatric disorders., Results: Approximately 12.1% of people with ID had psychiatric disorders. These individuals were more likely to be poorer in health condition and consuming more medical services (in the outpatient, inpatient and emergency care areas), than those individuals without psychiatric disorders. These individuals with psychiatric disorders were also taking medicines regularly at a far greater percentage than did those without psychiatric disorders., Conclusions: Given the high prevalence of psychiatric disorders among individuals with ID, the healthcare system should take further steps to develop an appropriate health status monitoring system and community-based and easily accessible mental health services for them.

Cohen syndrome is a rare autosomal recessive disorder with a variable clinical picture mainly characterized by developmental delay, mental retardation, microcephaly, typical facial dysmorphism, progressive pigmentary retinopathy, severe myopia, and intermittent neutropenia. A Cohen syndrome locus was mapped to chromosome 8q22 in Finnish patients, and, recently, mutations in the gene COH1 were reported in patients with Cohen syndrome from Finland and other parts of northern and western Europe. Here, we describe clinical and molecular findings in 20 patients with Cohen syndrome from 12 families, originating from Brazil, Germany, Lebanon, Oman, Poland, and Turkey. All patients were homozygous or compound heterozygous for mutations in COH1. We identified a total of 17 novel mutations, mostly resulting in premature termination codons. The clinical presentation was highly variable. Developmental delay of varying degree, early-onset myopia, joint laxity, and facial dysmorphism were the only features present in all patients; however, retinopathy at school age, microcephaly, and neutropenia are not requisite symptoms of Cohen syndrome. The identification of novel mutations in COH1 in an ethnically diverse group of patients demonstrates extensive allelic heterogeneity and explains the intriguing clinical variability in Cohen syndrome.

This study was conducted to compare aging phenomena of persons with intellectual and developmental disability (ID) aged 40 years and older living in community residence (N = 65) with those living with their families (N = 43) in Jerusalem, Israel. All 108 persons and care givers were interviewed to ascertain health problems, sensory impairment, activity of daily living (ADL), cognitive skills, and leisure activities. Health problem had already developed by age 40 years. The most frequent were visual (33%), hearing impairments (20%) and dental problems (30%). The community residence group displayed more medical problems, whereas individuals living at home had more dental problems. Health problems in persons with Down syndrome were significantly higher. ADL functioning for all participants was high, but persons with Down syndrome and cerebral palsy had more dependence. A decline in functioning in both residential groups was observed concerning leisure time, but scores for social life leisure activities were better for the community residential group. The data provided in this study can serve as information to develop geriatric services for persons with ID and provide a basis for comparison with peers in the general population in Israel. Dental service to persons with ID living at home should be improved.

The findings of a portion of a 2-year ethnographic study involving North Indian Hindu immigrants living in the mid-Western United States is discussed. These findings illuminate the ways in which participants think and talk about mental retardation, how this linguistic information was obtained, and the cultural context within which participants have come to hold their beliefs. Implications for special education practice are explored as are some strategies that could be helpful when working with Indian families. Suggestions for future research are included.

Aims: To identify any UK children with variant Creutzfeldt-Jakob disease (vCJD) and obtain information about the causes of progressive intellectual and neurological deterioration (PIND) and the geographical distribution of cases., Methods: The PIND Study uses the monthly surveillance card that is sent to all UK paediatricians by the British Paediatric Surveillance Unit. Case details are obtained from the reporting paediatricians by telephone interview, site visit, or self completion of a questionnaire. A paediatric neurology expert group then classifies the anonymised cases. The Communicable Disease Surveillance Centre (CDSC) provides mapping support., Results: After five years and five months of surveillance, 1400 children had been reported. In the UK the majority of PIND cases had a confirmed diagnosis (comprising 99 different conditions); 505 "no cases" and 97 "outstanding" cases were excluded. A total of 798 PIND cases were included as follows: 577 with a confirmed underlying diagnosis; six with definite or probable vCJD, 51 who had undiagnosed PIND but were not thought to have vCJD, and 164 cases who were still under investigation. In some districts there were unexpectedly high numbers of PIND cases with a heterogeneous mixture of underlying diagnoses. In the five districts with the largest numbers of resident cases the majority not only came from a particular ethnic group but also had high reported rates of consanguinity., Conclusions: In districts with large numbers of PIND cases there are major resource implications. These children and their families have complex problems and they need access to diagnostic facilities and appropriate service provision.

Background: This study examines the rate of psychiatric diagnosis as per ICD-10 and Reiss Screen for Maladaptive Behaviours (RSMB), and distribution of psychiatric diagnosis with regard to the severity of intellectual disability (ID). It also explores the degree of agreement between Reiss screen and clinical diagnosis (ICD-10) in relation to dual diagnosis., Methods: In this study Intelligence (IQ) and Social Quotient (SQ) were estimated on Indian adaptation of Binet's scale and Vineland Social Maturity Scales, respectively, in 60 consecutive persons with ID. Both ICD-10 and RSMB were used independently to determine the presence of psychiatric diagnosis., Results: Clinically, according to ICD-10, about 60% of the sample was found to have dual diagnosis as compared to 48% on RSMB. Agreement between ICD-10 and RSMB about psychiatric comorbidity was 82%. Commonest psychiatric diagnosis was unspecified psychosis followed by bipolar affective disorders., Conclusion: Persons with ID do suffer from various psychiatric disorders. RSMB is a useful tool for differentiating between psychiatric problems and maladaptive behaviours. Hence RSMB can be used in the Indian context.

The human opposite paired-containing (HOPA) gene is believed to be a co-activator of the thyroid hormone receptor and involved in thyroid hormone signal transduction. The gene consists of 45 exons and includes a dodecamer duplication in exon 43, which has been reported to be associated with mental retardation, autism, psychiatric disorders and hypothyroidism. We were interested to know if the 12-bp duplication variant of the HOPA gene is a risk factor for mental retardation and schizophrenia in the Chinese population. We investigated the prevalence of the 12-bp variant in a sample of Chinese mental retardation and schizophrenic patients from Taiwan by PCR-based genotyping. None of the mentally retarded and schizophrenic patients were found to have this dodecamer duplication variant. Our results indicate that the HOPA polymorphism might be very rare in our population and is unlikely to be a major risk factor for mental retardation and schizophrenia in the Chinese population.

Record linkage of multiple sources was used to ascertain children with intellectual disability born in Western Australia between 1983 and 1992. The prevalence was 14.3 per 1000, 10.6 per 1000 for children with mild or moderate and 1.4 per 1000 for those with a severe level of intellectual disability. Prevalence was greater in males (prevalence ratio 1.6) and in children of Aboriginal mothers (prevalence ratio 2.3). Although prevalence of intellectual disability has been found to vary according to study location, method of ascertainment and criteria used, these estimates are similar to estimates from several recent studies.

Introduction: In the County of Copenhagen the treatment of mentally retarded (MR) children is centralised in the Center for Handicapped (CFH). The aim of this study was to elucidate whether the frequency of MR is higher in children from ethnic minorities than in the background population and whether an increase could be ascribed to inbreeding., Material and Methods: The medical records of children with a non-Danish family name and where both parents were from countries other than Denmark were studied., Results: A significant increase in the frequency of MR were found in the children from ethnic minorities: 16.6% of the children seen at CFH had an ethnic background compared to 14% in the background population, making a surplus of 18.5% more than expected. When a family was inbred other family members had Mr in 43.0%, whereas more family members with MR were found in only 14.2% of the families with no inbreeding. In the consanguineous families we found possible aetiological diagnoses in 21.5% of the children compared to 49% in the non-consanguineous families. In the entire group of children from ethnic minorities, we found Down's syndrome in 3.2% compared to 10.7% in Danish children with MR., Discussion: There is a surplus of MR in children from ethnic minorities in the County of Copenhagen. The causes are not known, nor are aetiological factors for MR for a great part of the children. Consanguinity is likely to be a risk factor for MR, especially if it is already present in the family. This conforms with previously published data from other countries.

There are a number of problems and challenges in relating the science of epidemiology to mental retardation (MR). These relate to how MR is defined and classified and how these definitions may change over time. These as well as other differences in ascertainment sources and methods need to be considered when comparing MR prevalence over time and place. On the other hand, advances in technology also provide new and efficient methods of data collection both by data linkage and by use of web-based methods to study rare diseases. While prevalence studies have not been individually reviewed, we have examined the range of data including recent studies relating to how prevalence differs according to age, gender, social class and ethnicity. Some problems with available etiological classification systems have been identified. Recent etiological studies, most of which use different classification systems, have been reviewed and explanations have been postulated to account for differences in results. Individual risk factors for MR are considered whilst the option of considering a population as opposed to a high risk strategy to MR prevention is raised. This might well involve improving the social milieu surrounding the occurrence of individual risk factors. The impact of biotechnological advances such as antenatal and neonatal screening and assisted reproduction on MR are discussed. The issue of how inequalities in access to technology may impact on case identification and even have the potential to further widen inequalities is raised. The importance of extending the use of epidemiological tools to study the social, health and economic burden of MR is also emphasized. However, in order to apply to MR the "prevention-intervention-research" cycle, which surely underpins all epidemiology, it is vital to ensure that the methodological challenges we raise are adequately addressed., (Copyright 2002 Wiley-Liss, Inc.)

Social identity theory was applied in group therapy for adults with mild mental retardation. Social identity theory suggests that social group membership, also called collective identity, has an impact on self-esteem. Individuals will try to maintain self-esteem by viewing their social groups positively. This may not be possible for individuals who are members of a stigmatized group. However, it may be possible to enhance self-esteem by broadening one's awareness of collective identity. Furthermore, being able to positively view other individuals who are co-members of one's own stigmatized group can also have positive consequences for self-esteem. A clinical vignette demonstrates this process in group therapy. Results are discussed as being applicable to members of various stigmatized groups.

Nine cases of Kabuki syndrome have been identified in Auckland and surrounding regions in the North Island, New Zealand since 1995. All have the characteristic facial dysmorphism and many of the well-described associated anomalies. Some of the abnormalities were unusual including a case with severe congenital mitral stenosis, two cases of eventration of the diaphragm, idiopathic thrombocytopaenic purpura and vitiligo. One child had an Arnold Chiari type 1 malformation and another had epibulbar dermoids, neither of which has previously been reported in this syndrome. There was a wide diversity of ethnic origin, with the syndrome being described in patients from the Pacific Islands for the first time. The cases described emphasize the broad range of associated anomalies found in Kabuki syndrome and further illustrate its presence in all ethnic groups.

The Wechsler Intelligence Scale for Children--Third Edition and the Cognitive Assessment System were compared for a sample of 78 White and Black students in special education programs for children with mental retardation. Results showed that the WISC-III identified more children as having mental retardation than did the Cognitive Assessment System. More important, however, the WISC-III classified disproportionately more Blacks than Whites as having mental retardation as compared to the Cognitive Assessment System. Results imply that the problem of disproportionate representation of Black children in special education classes for children with mental retardation may be addressed if the Cognitive Assessment System were used instead of the WISC-III.

Objective: To date, Micro syndrome has been reported in only three children from one family. We describe an additional 14 children from 11 families., Design: Retrospective case series., Participants: Fourteen children from 11 families attending one of five British hospitals., Main Outcome Measures: The following features were documented: pre- and postoperative eye findings, electrophysiologic analysis, systemic abnormalities, development, neuroimaging, genealogy, geographic origin of family., Results: We expand and modify the description of ocular and electrophysiologic findings in Micro syndrome. The eye findings of microphakia, microphthalmos, characteristic lens opacity, and atonic pupils were the presenting feature in all infants and were the most reliable diagnostic signs in the immediate postnatal period. Cortical visual impairment, microcephaly, and developmental delay were not always detectable initially; they developed in all children by 6 months of age. Microgenitalia were a useful diagnostic clue in affected males only. Therefore, eye features were more consistently useful in determining diagnosis than dysmorphology or brain imaging. The families of all the children originate from the Muslim population of Northern Pakistan. Inheritance is likely to be autosomal recessive., Conclusions: Micro syndrome usually presents to the ophthalmologist, who may be able to make the diagnosis on the basis of characteristic eye findings combined with ethnic origin. Initially, the nature and severity of nonophthalmic features are not apparent. Early diagnosis of the underlying condition is important to guide management of the cataracts, glaucoma, and developmental delay. It is helpful for the family and medical staff to be aware of the low level of vision that develops despite optimal ophthalmic intervention. Genetic counseling extending into the wider family is particularly important in view of the high rate of consanguinity.

This article examines current psychometric and testing practices that appear to do a limited job of assessing the intelligence of American Indian individuals. For several reasons, contemporary approaches are found to be inadequate. Unfortunately, these practices are then employed in making educational decisions and placing these same children into Special Education programs. Alternative methods of testing, including the Gf-Gc Cross-Battery Approach, are discussed and evaluated in terms of usefulness in the evaluation of American Indian children and adolescents. The cross-battery method of testing was found to provide more in-depth procedures for bypassing both language and cultural differences among American Indian individuals.

The utility of the Dementia Rating Scale (DRS) when administered to individuals with mental retardation (MR) was examined. Our sample was comprised of individuals residing in an intermediate care facility in the southeastern United States, included individuals diagnosed with mild, moderate, or severe MR, and consisted of both Caucasians (50%) and individuals of African-American descent (50%). Descriptive statistics for the DRS Total Score and five subtests (e. g., Attention, Initiation/Perseveration, Construction, Conceptualization, and Memory) obtained from our sample of individuals with mild MR compared favorably with previously published values. The group with mild MR performed significantly better than the group with moderate MR on the Total Score and all subtests except Construction, and the group with moderate MR performed significantly better than individuals with severe MR on all measures. These results show that the DRS can provide information about the cognitive strengths and weaknesses of individuals with mental retardation, and they show that the DRS can be administered to a wide range of individuals diagnosed with MR.

The present case report describes the arranged marriage of a Bangladeshi woman with moderate intellectual disability. It explores some of the social and cultural factors influencing the decision to arrange her marriage, and the dilemmas this presents in terms of bridging cultural differences between professional and lay concerns.

In an effort to encourage dental practitioners to provide needed services for children with mental retardation as they grow older, a review is provided of the ongoing difficulties faced by families and their children--including attitudes, residence, sibling and older parent dilemmas, cost of care and the realities of sexuality.

Identification of the comorbidity of mental retardation and psychopathology in a multicultural setting raises manifold difficulties. The present study explored the sampling and identification issues implicated in estimating the prevalence of this dual diagnosis in a South African clinic sample. The relations between the prevalence of dual diagnosis and socioeconomic status, gender, and severity level of retardation were investigated. The detection rate of 4.36% was significantly lower than that of other studies. Prevalence was found to be greater in areas of high socioeconomic status, among males, and among less severely retarded individuals. Implications of these findings for cross-cultural studies and for allocation of service resources for patients with dual diagnosis are considered.

The Americans with Disabilities Act bars discrimination in public accommodations, housing, employment and health care for people with disabilities. Efforts to "mainstream" (in housing and education) many of the individuals with mental retardation have altered the setting for dental services and place demands for services by dental practitioners in the community. These developments should be considered within the jurisdiction of this new legislation. Demographic characteristics and dental needs of children with mental retardation are reviewed to provide a background for this discussion.

Researchers have found elevated risk for maladjustment associated with being an African American adolescent in an urban environment as well as being an individual with mental retardation. The culturally relevant factors of ethnic identification, intergenerational support, and church support were investigated in relation to high risk exposure on maladjustment in 147 urban African American adolescents enrolled in EMR special education classes. Maladjustment was measured with both self- and parent-report. Risk exposure was measured in the personal, social, and community domains. Results indicate that presence of cultural factors were associated with better adjustment generally. Furthermore, ethnic identification appeared to protect adolescents exposed to high-risk conditions against experiencing significantly elevated maladjustment. Implications of culture on intervention and prevention were discussed.

Clinic data from the regional hospital in Cape Town, South Africa, over 4 years for 538 children with a diagnosis of mental retardation were examined in order to establish whether differences in mental retardation referral patterns existed between low and high socioeconomic areas. Results indicate that mild mental retardation referrals were underrepresented in low socioeconomic areas and that paramedical agencies were the primary referral source in these areas. Differences in mild mental retardation referral patterns by area over time may stem from service difficulties or variations in referral thresholds. Cross-cultural implications of the findings were discussed with relevance to the development of culturally sensitive community-based intervention programs.

We compared the ability of Chinese adults with mental retardation and nonhandicapped Chinese children to recognize the six basic facial expressions of emotion. Each subject was told a story identifying an emotion, presented with an array of six photographs of basic facial expressions of emotion, and asked to point to the photograph that depicted the story's emotion. Results showed that the children were more accurate than the adults in recognizing facial expressions of emotion except happiness on which both groups achieved 100% accuracy. Surprise, fear, anger, and disgust were confused most often by both groups. Recognition proficiency was not significantly correlated with age in the children or with IQ in the adults. Our results partially replicated those reported in earlier studies with non-Chinese individuals with mental retardation and raised the possibility that there may be cultural influences on a person's ability to recognize facial expressions of emotion.

There is a paucity of research on the frequency, intensity, and content of fears experienced by Hispanic (or Latino) American youth with mental retardation. We assessed fears experienced by 48 Hispanic youth with mental retardation (ages 10 to 21 years) using the Fear Survey for Children With and Without Mental Retardation. Although differences were found in the content of fears across age and gender groups, the number and intensity of fears did not differ. Promising evidence of the validity of this survey instrument was attained by its significant correlations with the Revised Children's Manifest Anxiety Scale. Implications for future research were discussed.