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PubMed1992

Adiposity in children: is mental retardation a critical variable?

Several authors have suggested that obesity is more prevalent amongst children with mental retardation than non-mentally retarded children. However, studies on which this suggestion is based typically lack adequate control groups. The current research compared adiposity amongst mentally retarded versus non-mentally retarded children. Study 1 compared 110 mentally retarded children with 107 non-mentally retarded children (162 males, 61 females; age range 11 months-20 years). The independent (predictor) variables included IQ and mental retardation. Dependent (criteria) variables were BMI and age-corrected BMI. Study 2, using the National Health and Nutrition Examination Survey-II data bank compared 20 children with reported mental retardation with 4015 control children on three variables: BMI, age-corrected BMI, and subscapular to triceps skinfold ratio. Results of both studies found no significant difference in adiposity or body fat distribution between mentally retarded and non-mentally retarded children. Moreover, no significant difference emerged when either age or gender were controlled and no curvilinear or interaction effects were observed.

Adipose Tissue↗
PubMed1990

[The need of special services in the future care of the mentally retarded].

A proposal for deinstitutionalization of the mentally retarded in Norway passed the Norwegian Storting in May 1988. The Ministry of Health and Social Affairs has prepared guidelines for planning and implenting this reform. On 1 January 1991 the responsibility for providing the necessary services was transferred to the municipalities. Many problems have to be solved in order to ensure that the life of each mentally retarded person is qualitatively improved after normalization and integration into society. In July 1989 the Ministry of Health and Social Affairs issued a document which assumed that necessary specialists and special services for the mentally retarded already exist, and are being used. Our study has questioned this assumption as being incorrect or incomplete. We interviewed persons in charge of the system of health care in all 19 Norwegian counties. Our conclusions are as follows: The mentally retarded do receive specialist services in various areas of somatic medicine. Their needs as regards psychiatric care are not taken care of by psychiatric specialists in hospitals or at out-patient facilities. Data shows that the system of institutionalized health care for the mentally retarded provides unique services which are best described as systematically applied behaviour analyses. Such special competence is often lacking in the Norwegian health services otherwise. In order to ensure that the mentally retarded will receive the best possible care, this competence must be integrated into the future system of care. It is most importance to consider these problems without delay. This article also discusses organizing special training and treatment for the mentally retarded themselves and special training for the persons providing the care.

Health Planning Guidelines↗
PubMed1995

Variation in the influence of selected sociodemographic risk factors for mental retardation.

OBJECTIVES: This study explored the utility of subdividing mental retardation into groups based on the presence of other neurological conditions. METHODS: Data were abstracted from birth certificates as part of a case-control study of mental retardation among 10-year-old children. The study sample included 458 case children and 563 control children selected from public schools. Case children were subdivided on the basis of intelligence quotient (IQ) score and the presence of other neurological conditions. RESULTS: Other neurological conditions were more common with severe mental retardation than with mild mental retardation. Regardless of IQ level or the presence of other neurological conditions, boys were more likely than girls to have mental retardation. Older mothers were more likely than younger mothers to have a child with mental retardation accompanied by another neurological condition. High birth order, Black race, and low maternal education were associated with a higher prevalence of isolated mental retardation. CONCLUSIONS: These findings suggest that sociodemographic risk factors for mental retardation vary according to the presence of other neurological conditions and that subdivisions based on medical or physical criteria may be useful in epidemiologic studies of mental retardation.

Black or African American↗
PubMed1985

Embryonic testicular regression syndrome and severe mental retardation in sibs.

The embryonic testicular regression syndrome associated with severe mental retardation is reported in three 46,XY sibs each of whom has a 46,XY chromosome complement. A fourth sib, a sister, also is severely retarded mentally; her chromosome complement is 46,XX. The 46,XY individuals, who were raised as females, presented varying degrees of genital ambiguity, indicating that their gonadal activities had been arrested at different times during embryogenesis. No trace of gonadal tissue could be found in either patient. The coincidence of the embryonic testicular regression syndrome and severe mental retardation in the same sibship is discussed.

Adult↗
PubMed1989

A multidimensional model of mental retardation: impairment, subnormal behavior, role failures, and socially constructed retardation.

A multidimensional model of mental retardation was proposed in which impairment, subnormal behavior, and role failures were underpinned by medical, statistical, and social models of normal, respectively. Thus, individuals may be designated "retarded" in one or a combination of logically independent senses. A framework for research and theory was also provided. A new fourth dimension, socially constructed retardation or mental handicap, was defined and its etiological influences in medical, statistical, and social dimensions of retardation was considered, as were interactions of these dimensions. New strategies for operationalizing and alleviating mental retardation were identified.

Achievement↗
PubMed1983

Nonspecific mental retardation in British Columbia as ascertained through a registry.

Nonspecific mental retardation (i.e., mental retardation with no known etiology) at all levels of functioning was investigated in the population of British Columbia, with ascertainment through the British Columbia Health Surveillance Registry. The age-specific prevalence of nonspecific retardation for the 15- to 29-year age group, where ascertainment was best, was 4.4 per 1,000 population at year-end 1978. A minimum incidence of 5.2 per 1,000 male live births and 4.0 per 1,000 female live births was calculated for the 1952-1965 birth cohort. Data on the number of cases associated with either microcephalus, hydrocephalus, epilepsy, or cerebral palsy were presented. Seventy-three percent of the cases with mild mental retardation had no associated disability, whereas only 26.9 percent of profoundly retarded cases were without any of these conditions. Survival rates varied with respect to the level of mental retardation.

Adolescent↗
PubMed1990

Teaching self-help skills to autistic and mentally retarded children.

Three autistic, mentally retarded children, ranging in age from 4 to 11 years, and a six-year-old mentally retarded girl, were taught various adaptive behaviors using a multiple baseline design. Skills taught were shoe typing, toothbrushing, hair combing, putting on pants, shirt, and socks, and eating and drinking. Training included modeling, verbal instructions, prompting, and edible and social reinforcement. Treatment procedures involved the whole-task method of teaching self-help skills and consisted of three phases: (a) the trainer modeled and verbally described the target behavior; (b) the trainer physically and verbally guided the child through the entire sequence of task-analyzed steps; and, (c) the child was instructed to perform the behavior independently. The results of this study and their implications for future research are discussed.

Activities of Daily Living↗
PubMed1995

A population-based study on the causes of mild and severe mental retardation.

The causes of mental retardation (MR) were studied as part of a multidisciplinary epidemiological case-control study in 151 mentally retarded patients identified by screening four age cohorts (12,882 children) at 8-9 years of age in the province of Kuopio, Finland. The causes of MR in 77 severely retarded (SD < or = -3 SD) and 74 mildly retarded (-2 > SD > -3) children were divided into pre-, peri-, postnatal and unknown groups according to the probable time of onset. The causes were pre-, peri-, postnatal and unknown in 60%, 9%, 8% and 23%, and 22%, 1%, 3% and 74%, in the two populations, respectively. Genetic causes were found in 28% of all 151 cases; the three most common subgroups were trisomy 21, fragile X syndrome and aspartylglycosaminuria (13%, 4% and 2% respectively). The study design used provided reliable information on the causes of MR and also demonstrated those forms of genetic metabolic diseases typical of Finnish inheritance.

Acetylglucosamine↗
PubMed2006

[Genetic mental retardation. Presentation of the GIRMOGEN network].

INTRODUCTION: Mental retardation is the most frequent disability and is already quite apparent in infancy. The World Health Organisation (WHO) estimates that it affects approximately 3% of the population in industrialised countries. Among the aetiologies that cause mental retardation it would appear that 30% have a genetic origin, although in recent years the progress made in molecular genetics in relation to new mutations and new genes that can account for mental retardation advances at an incredible pace. It is for this reason that, three years ago, a group of clinicians and researchers, most of whom were working in Spain on fragile X syndrome (the most prevalent of the hereditary causes of mental retardation), decided to set up the GIRMOGEN (Genetic Mental Retardation Research Group). Most of us had noticed how many of the clinical cases that we dealt with went undiagnosed and that the exact prevalence of this disability in Spain was not known either. DEVELOPMENT: GIRMOGEN was funded by the Carlos III Health Institute and is made up of eight groups; a ninth group is responsible for coordinating the work. Its members are all involved in clinical studies or research into mental retardation with a genetic origin, and belong to 15 hospitals and to five universities from a total of 11 different autonomous communities in Spain. Some of its aims include gathering all the data on patients and families in a common database for epidemiological and prevalence studies; distributing genes to be studied in order to search for mutations; creating generally approved work protocols, and training professionals in this field. CONCLUSIONS: In this supplement, which is the result of a training course, we report all the findings we have obtained in these three years of work.

Biomedical Research↗
PubMed1997

[Fragile X syndrome is still unrecognized: efficacy of molecular diagnosis in mentally retarded probands].

BACKGROUND: The fragile X mental retardation syndrome is the most common cause of inherited mental retardation. Identification of the unstable mutation responsible for the disease has allowed the design of a fully reliable molecular test for the diagnosis of the disease and for genetic counselling (identification of clinically normal carriers and prenatal diagnosis). We started in July 1991 to search for the mutation in mentally retarded probands, with no known cause for their phenotype. We present the results of a 42-month experience. POPULATION AND METHODS: One thousand and one hundred fourty-nine probands were analysed. In case of a positive diagnosis, an extension of the molecular study to relatives was proposed. DNA samples were studied by Southern blot following EcoRI or EcoRI + EagI digestion. Clinical data were collected from referring clinicians. RESULTS: Seventy-three carriers of a full mutation were identified, belonging to 52 families. The mean age of the fragile X probands was 16 +/- 14 years, which is very surprising for a disease that causes significant manifestations by the age of 2 to 3 years. This indicates an insufficient knowledge about this disease in France. Most of the demands for the test were from clinical geneticists. This diagnosis is of major importance for genetic counselling, as illustrated by the following study of 108 women at risk in these families. CONCLUSIONS: The importance of an early diagnosis followed by an extended family study, for carrier screening and prevention of this severe disease, justifies molecular testing on any child with mental retardation or significant language delay of unknown cause, in the absence of clinical signs formally excluding a fragile X diagnosis.

Adolescent↗
PubMed2005

Loss of ZDHHC15 expression in a woman with a balanced translocation t(X;15)(q13.3;cen) and severe mental retardation.

X-linked mental retardation (XLMR) affects one in 600 males and is highly heterogeneous. We describe here a 29-year-old woman with severe nonsyndromic mental retardation and a balanced reciprocal translocation between chromosomes X and 15 [46,XX,t(X;15)(q13.3;cen)]. Methylation studies showed a 100% skewed X-inactivation in patient-derived lymphocytes indicating that the normal chromosome X is retained inactive. Physical mapping of the breakpoints localised the Xq13.3 breakpoint to within 3.9 kb of the first exon of the ZDHHC15 gene encoding a zinc-finger and a DHHC domain containing product. Expression analysis revealed that different transcript variants of the gene are expressed in brain. ZDHHC15-specific RT-PCR analysis on lymphocytes from the patient revealed an absence of ZDHHC15 transcript variants, detected in control samples. We suggest that the absence of the ZDHHC15 transcripts in this patient contributes to her phenotype, and that the gene is a strong candidate for nonsyndromic XLMR.

Adult↗
PubMed1994

[Considerations of psychopathology in mental retardation].

There is a high incidence of psychiatric disorders in mentally retarded subjects: one third to two thirds of mentally retarded subjects exhibit psychiatric disorders, a proportion which is much higher than that found in subjects with normal intelligence. The issue is to clarify the nature of the relationship between cognitive and psychiatric disorders (generally analyzed in a dichotomous approach). A way to analyze the phenomenon is to consider a psychopathological approach, which can define the underlying mechanisms responsible for this incidence. The aim of this paper is to analyze the explicatory value of deficient cognitive development, as the main factor determining a specific personality organization. Direct and indirect effects of cognitive impairment on the development of personality disorders are described: the first, in terms of how cognitive deficit (i.e. severity, homogeneity in several cognitive domains, pattern of development) disorganizes personality; the second, in terms of impact that cognitive deficit could have on the child's relationship with the external world, especially with the mother. In order to illustrate these viewpoint, the paper discusses the role of cognitive functions in the development of personality. Specifically, the way the normal child processes his perceptual and motor experiences is analyzed, that is pursuit of new causal links in his knowledge seeking activity of mastering the world. The child's primitive relationship with the world is then aimed at learning, exploring and searching for new causal links. In the light of these considerations, what the child with Mental Retardation experiences is discussed. A series of psychopathological mechanisms in Mental Retardation are postulated. The organization of the Mentally Retarded child's internal world is described, as reflected in Rorschach protocols, which outline a chaotic and primitive internal world, but with a specificity of its own. Finally, the paper discusses the hampering effect that cognitive impairment has on the quality of the relationship with the caregiver. This effect can be seen in terms of the child's interactive capacity and, at the same time, in terms of the emotional impact on the caregiver that derives from interacting with a mentally retarded child. From the above considerations a global approach to the psychopathology of cognitive and affective aspects of Mental Retardation seems warranted. Both aspects acquire a specific significance when seen in light of a specific personality organization. Defining the characteristic of this specific organization seems to be the key to a more comprehensive approach to psychiatric disorders of Mental Retardation.

Cognition Disorders↗
PubMed1987

Traditional views and new perspectives on the genetics of mild mental retardation.

Normal intelligence and mild mental retardation are generally assumed to be determined by additive polygenes. This old and basic theory is challenged by findings from modern population studies. The discrepancies concern frequency of mild mental retardation, sex ratio, and additional somatic handicaps in populations with mild mental retardation.

Humans↗
PubMed1985

The prevalence of psychiatric morbidity in mentally retarded adults.

302 mentally retarded adults, sampled by epidemiological criteria, were examined with regard to handicaps, behaviour, skills and psychopathology by use of the MRC HBS-schedule and a list of psychiatric items. Based on research criteria, a computerized psychiatric diagnosis was made on a hierarchial scale. A psychiatric disorder was diagnosed in 85 (27.1%), which is a smaller prevalence rate than found in other studies. Next to behaviour disorder (10.9%), psychosis of uncertain type (5%) was the most common disorder. Dementia and early childhood autism were found equally often (3.6% each). Neurosis was seldom (2%), while schizophrenia (1.3%) and affective disorder (1.7%) occurred at about the same rates as found in similar investigations. No cases of alcohol or drug abuse were found.

Adolescent↗
PubMed1991

[Sterilization of mentally retarded women].

The sterilization of mentally retarded women raises gynecologic, psychiatric and legal questions. Gynecologic considerations include the experience of the reliability of reversible contraceptive methods with mentally retarded patients and questions concerning the type of operation. From the psychiatric point of view the prognoses of mentally retarded patients and the expected psychic coping with the operation are essential aspects in the indication for sterilization. Last but not least, the Swiss legal framework for sterilization must be considered. This aspect is illustrated on the basis of the results of a catamnestic analysis of 21 patients operated on between 1980 and 1987.

Adolescent↗
PubMedDate not supplied

Say-do-report training to change chronic behaviors in mentally retarded subjects.

Two mentally retarded subjects with language deficits participated. A say-do-report correspondence training was implemented to break the functions of present conditions given by a long history of contingencies maintaining chronic inadequate patterns of behavior. The say-do-report procedure was implemented following a multiple baseline design across two behaviors in one subject and in an AB design for the other. During baseline, all possible social contingencies maintaining the inadequate behaviors were eliminated. Promising or saying what a subject would do was then implemented and followed by the differential reinforcement of say-do correspondence reports. All behaviors changed and were maintained at an appropriate level, even after eliminating the components involved in the say-do-report procedure, that is, the reports, the extra consequences, and even the promise. Results are discussed in the context of verbal behavior altering the function of present conditions in subjects with limited verbal repertory, as well as in the context of new applications to make a difference in chronic behaviors.

Adolescent↗