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PubMed1995

The Developmental Behavior Checklist: the development and validation of an instrument to assess behavioral and emotional disturbance in children and adolescents with mental retardation.

Describes the development and validation of the Developmental Behavior Checklist (DBC), a standardized instrument completed by lay informants to assess behavioral and emotional disturbance in children and adolescents with mental retardation (MR). Items describing common behavioral and emotional problems in this population were generated by extracting descriptions from 664 case files of children and adolescents with behavior disorders seen at a specialist developmental assessment service over 12 years. These items were reduced to a set of 96 items administered to a sample of 1,093 children and adolescents with mental retardation and then submitted to a principal components analysis. Six interpretable and partly validated subscales were obtained which explained 36% of the total variance and had satisfactory internal consistency. Interrater and test-retest agreement were satisfactory for both total scale score and for scores on each of the subscales. Good evidence of concurrent validity was provided by substantial positive correlations between total scores on the DBC completed by lay informants and the ratings of experienced psychiatrists based upon interviews and scores on two standardized instruments that must be completed by health professionals. The discriminative validity of the total score as assessed by area under the ROC curve was excellent (92%). Standardized norms for the DBC are derived from an epidemiological study of behavior problems in children and adolescents with mental retardation undertaken in two Australian States. Norms are available for the mild, moderate, severe, and profound MR groups and for the MR population as a whole.

Adolescent↗
PubMed1979

Vigilance of performance of mildly mentally retarded children.

The contribution of developmental factors to the vigilance performance of mildly mentally retarded children was investigated by comparing their vigilance performance with that of nonretarded children of the same CA and with younger nonretarded children of about the same MA. The retarded children showed an earlier and faster decline in vigilance performance than did the CA control group. This result was similar to that of Semmel (1965), but contrasted with that of Kirby, Nettlebeck, and Bullock (1978), who found no such difference in vigilance performance when comparing mildly mentally retarded and nonretarded adults. The MA control group performed in a similar way to the retarded children, suggesting that the more rapid decline in vigilance performance was largely a developmental phenomenon.

Adolescent↗
PubMed1994

Guidelines for handling domestic violence cases in community mental health centers. Kentucky Department for Mental Health and Mental Retardation Services.

Community mental health centers are becoming increasingly involved in the delivery of services to victims and perpetrators of domestic violence. To help centers plan a domestic violence program and address the risk of liability in treating clients who may be dangerous, the authors suggest principles to guide clinical decisions, standards for service delivery, and standards for staff development. Domestic violence is clearly defined as criminal behavior. In treatment, cessation of violence takes priority over family reunification and resolution of issues between victim and perpetrator. Decisions about accepting a perpetrator in treatment should be made by the treatment provider, even if treatment is mandated by the court. Suggestions for reducing the burden of domestic violence cases on individual clinicians include using treatment teams, establishing guidelines for maximum caseloads, and encouraging mixed caseloads. CMHCs have an important role in a comprehensive approach to domestic violence that includes a wide array of services and careful coordination among agencies that provide them.

Adult↗
PubMed1985

Psychosocial correlates of depression in mentally retarded adults: I. Minimal social support and stigmatization.

Two hypotheses were tested in a study with 28 mildly mentally retarded, emotionally disturbed adults and 17 mildly mentally retarded adults for whom an emotional disorder had not been diagnosed: depression is associated with (a) low levels of social support and (b) high levels of perceived stigmatization. Self-report and informant measures of depression, social support, and perceived stigmatization were collected. The self-report and informant measures of the same construct were significantly correlated. Results revealed negative correlations between depression and social support ranging between -.25 and -.76 and positive correlations between depression and perceived stigmatization ranging from .11 to .44. After additional analyses comparing depressed subjects, disturbed/nondepressed subjects, and nondisturbed/nondepressed subjects, the results were interpreted as showing a powerful association between low levels of social support and depression. Convincing evidence of an association between perceived stigmatization and depression was not found.

Depressive Disorder↗
PubMed1987

Hepatitis B infection in a day school for mentally retarded students: transmission from students to staff.

We carried out a seroepidemiologic study to evaluate the transmission of hepatitis B virus (HBV) from students to staff in a day school for mentally retarded students. Of 505 students tested, 37 (7.3 per cent) had HBsAg; 74 per cent of HBsAg-positive sera tested were HBeAg-positive. Of 162 staff members tested, 21 (13.0 per cent) were HBV marker positive and two (1.2 per cent) had HBsAg; specialized educators and teachers showed the highest HBV marker prevalence (22.2 per cent and 21.3 per cent, respectively). The prevalence of HBV markers among staff was independently associated with classroom contact with a HBsAg-positive student, duration of employment at the school, and previous work with mentally retarded individuals. The incidence of HBV infection among teaching staff with regular classroom contact, estimated by logistic regression analysis, was 2.6 per cent per year. This observation indicates that teaching staff in schools for mentally retarded students with direct classroom contact may carry an occupational risk of HBV infection.

Adolescent↗
PubMed1993

The association between psychiatric diagnoses and severe behavior problems in mental retardation.

To investigate the relationship between psychiatric disorders and severe behavior problems in mental retardation, statewide client databases from developmental disabilities services in California (N = 89,419) and New York (N = 45,683) were analyzed and juxtaposed. The study focussed on nine major DSM-III-R psychiatric categories (or their equivalents), and severe forms of aggressive behavior, property destruction, self-injurious behavior, and stereotyped behavior in individuals 45 years old and younger with mental retardation of all levels of severity. In California, 3.9% had at least one psychiatric diagnosis; in New York, 5.4%. The rate of specific psychiatric diagnoses was variable across states, suggesting local preferences in diagnostic practices. Severe behavior problems occurred in 22.1% in California and in 41.4% in New York. This difference in rates can be attributed in part to different recording criteria for behavior problems. With regard to the association between psychiatric diagnoses and problem behaviors the results were consistent across databases: No compelling correlations were found. This means that neither aggression, self-injury, destruction, nor stereotypies determine whether a person receives a psychiatric diagnosis or not.

Adolescent↗
PubMed1988

Daily decision-making in community residences: a social comparison of adults with and without mental retardation.

In addition to physical placement in the community, the extent to which individuals experience personal autonomy may provide a crucial measure of the attainment of a more normalized lifestyle. Everyday choices and choice-making opportunities of 24 persons with mental retardation living in community group homes were compared to choices of 42 nonretarded adults. The adults with mental retardation had significantly fewer opportunities to make decisions on such matters as what to eat or wear, how to spend free time, and with whom to live. Results were discussed in terms of the need to operationalize meaningful improvements in the lives of persons with mental retardation that go beyond the appearance of the physical environment.

Activities of Daily Living↗
PubMed1990

Effects of serum zinc supplementation on pica behavior of persons with mental retardation.

Although pica is one of the most frequently observed eating dysfunctions among people with mental retardation, it is significantly underreported in the literature. Of 806 institutionalized adults with mental retardation in the present study, 15.5% exhibited pica. Prevalence estimates from previous studies have ranged from 25.8% to 3%. Fifty-four percent of the pica group had serum zinc levels below normal range, whereas 7% of the control group had serum zinc levels below the normal range. After supplementation with chelated zinc, residents had significant reductions in pica. Data indicated zinc as a possible adjunct to other treatment approaches.

Adult↗
PubMed1981

In re Grady: the mentally retarded individual's right to choose sterilization.

In the case of In re Grady, the New Jersey Superior Court addressed important issues concerning the propriety of a court's exercise of parens patriae jurisdiction to ratify parents' substituted consent to the sterilization of their mentally retarded child. This Note discusses the genesis of the fundamental right to choose sterilization, its application to mentally retarded individuals, and the adequacy of the procedural framework enunciated in Grady to assure that substituted consent by the parents is exercised solely in the mentally retarded individual's "best interests". This Note concludes that, while the Grady court properly exercised its parens patriae jurisdiction, the procedural framework enunciated is inadequate. The Note proposes a model that would implement the procedural elements the Note determines are essential to a "best interests" inquiry. The proposal requires: (1) that the individual be required to argue that sterilization is not in the incompetent's "best interests"; and (3) that the court determine, as a question of fact, whether the parents' exercise of substituted consent is in the incompetent's "best interests." The Note also suggests criteria which can be used in making the "best interests" determination, and recommends that "clear and convincing" evidence be required to support the "best interests" standard.

Child↗
PubMed1996

[Invasive amebiasis at an institution for the mentally retarded in Shizuoka Prefecture].

Amebiasis caused by Entamoeba histolytica at an institution for mentally retarded in Shizuoka Prefecture is reported. Five of the 50 patients showed E. histolytica cysts in their stools and 4 were positive serologically. The polymerase chain reaction and restriction fragment length polymorphism revealed that the isolates were pathogenic-type E. histolytica. Epidemiological analysis revealed that the amebic infection was caused by the abnormal behavior of mentally retarded patients. Administration of diloxanide furoate and metronidazole for cyst-carriers eliminated cysts from the stool and lowered the antibody titer.

Adult↗
PubMed1995

The relationship of birth weight and birth weight discordance to cerebral palsy or mental retardation later in life for twins weighing less than 2500 grams.

OBJECTIVE: This study tested the hypothesis that twins with a birth weight < 2500 gm who subsequently had cerebral palsy or mental retardation had a birth weight distribution or birth weight discordance distribution differing from that of the total live-born twin population also weighing < 2500 gm at birth. STUDY DESIGN: All twins born in Sweden between 1973 and 1980 (n = 5382) and having a birth weight < 2500 gm were identified by using information stored at the Medical Birth Registry, the National Board of Health and Welfare, Stockholm, or at Statistics Sweden. To identify twins with cerebral palsy or mental retardation, a questionnaire bearing the personal identification number (given to all newborns in Sweden shortly after birth) was distributed to all rehabilitation centers, all county school boards, all local boards of education, and to all County Councils for the Provisions and Services for the Mentally Retarded. The questionnaire was distributed in 1988 and 1989 when the twins were > or = 8 years old. RESULTS: Altogether 115 disabled twins resulting from 99 pregnancies were identified. The incidence of disabled twins per 1000 was 21.4 (95% confidence interval 17.5 to 25.2). No obvious difference was evident in the distribution of birth weight discordance when the twins with disability were compared with the population of all live-born twins having a birth weight < 2500 gm. Disabled twins had a significantly lower birth weight for gestational age (t = -3.5, p < 0.001), but in fact only 10 (8.7%) twins had a birth weight < -2 SD. No difference in the incidence of disability was found for twin A versus twin B (relative risk 1.3, 95% confidence interval 0.8 to 1.9) or for like-sex versus unlike-sex twins (relative risk 1.0, 95% confidence interval 0.6 to 1.6). However, the larger twin in the pair had a significantly higher incidence of cerebral palsy than the smaller one did (relative risk 2.6, 95% confidence interval 1.4 to 4.8). CONCLUSIONS: Birth weight discordance for twins seems not to be related to disability later in life. The great majority of twins with a birth weight < 2500 gm who later became disabled were appropriate for gestational age at birth.

Birth Weight↗
PubMed1993

Reducing barriers to sex education for adults with mental retardation.

Techniques were identified that staff of agencies serving adults or adolescents with mental retardation perceived would enable them to provide systematic sex education and counseling. Through the use of a structured interview process, we obtained opinions of professionals from 16 agencies. On the basis of these interviews and a thorough review of related literature, we made recommendations aimed at increasing professionals' ability to provide systematic sex education and counseling for individuals with mental retardation. Suggestions for further study were presented.

Adolescent↗
PubMed1978

The X-linked syndrome of macroorchidism and mental retardation: further observations.

We report six males with the syndrome of macroorchidism and mental retardation. The trait is inherited as though X-linked, or possibly autosomal dominant male-limited. We also found no evidence of gonadal dysfunction. Associated abnormalities were abnormal EEG (3/4), seizures (2/6), and one instance each of cervical vertebral fusion, cataract, esophoria, and abnormal cutaneous pigmentation. One woman with a 50% a priori risk of bearing the mutant gene had mental retardation and seizures. Results of Xg blood-group typing were uninformative for the purpose of linkage analysis.

Abnormalities, Multiple↗
PubMed1982

A review and analysis of applied research in mental retardation: 1975-1980.

A review of the number and type of studies in mental retardation published in six prominent journals was made. Variables examined included whether the study was applied or not and in the latter case, whether the study was an assessment, treatment or epidemiological paper. Other variables evaluated were length of follow-up, frequency of applied studies published in each journal, whether subjects were children or adults, inpatients or outpatients, level of mental retardation and form of treatment employed. Implications of the present studies with regard to future directions of applied research are discussed.

Adult↗
PubMed2005

C21orf5, a new member of Dopey family involved in morphogenesis, could participate in neurological alterations and mental retardation in Down syndrome.

Availability of the human genome sequence promises important progress in the understanding of human pathologies, particularly for multifactorial diseases. Among these, Down syndrome (DS) is the most frequent genetic cause of mental retardation. A critical region of chromosome 21, the Down syndrome Chromosomal Region-1 (DCR-1), is responsible for many features of the DS phenotype including mental retardation. We studied DCR-1 C21orf5 as a new candidate gene for DS considering its restricted expression in key brain regions altered in DS patients and involved in learning and memory processes. To elucidate C21orf5 molecular function, we performed a comparative study of protein sequences in several species and showed that C21orf5 represents a new member of the Dopey leucine zipper-like family. The C21orf5 C-termini contains two highly conserved leucine-like zipper domains in invertebrate and vertebrate species. Evolution analysis indicated a common ancestral origin of these protein sequences also suggesting a conserved function of this gene throughout phylogenesis. Mutations of the known C21orf5 homologous genes Aspergillus nidulans DopA, Saccharomyces cerevisiae Dop1 and Caenorhabditis elegans pad1, determine morphological abnormalities. We studied transgenic mice carrying the human C21orf5 gene and we showed that this gene is overexpressed in brain regions by in situ hybridization and by real-time RT-PCR experiments. Interestingly, we also showed that these transgenic mice have an increased density of cortical cells overexpressing C21orf5. Similarly, DS patients have an altered lamination pattern in their cortex. Considering together our and previous findings, we suggest that the human dopey family member, C21orf5, could play a role in brain morphogenesis and, when overexpressed, it could participate in neurological features and mental retardation observed in DS patients.

Amino Acid Sequence↗
PubMed2000

Comparison of three probiotics in the treatment of acute diarrhea in mentally retarded children.

BACKGROUND: This article aims to compare the use of VIS-01 (Yovis) with two other probiotics (lactic acid bacteria), such as Lactogèrmine and Codex in the treatment of acute diarrhea in children with mental retardation. A recent paper highlights the superiority of the first drug, claiming new perspectives in probiotic therapy. METHODS: The authors perform a prospective study on 33 mentally retarded children (mean age 10.3 years), divided in three groups, each treated with a different probiotic (VIS-01, Lactogèrmine and Codex) at the beginning of an acute diarrhea. These children were admitted at the Pediatric Unit of the Oasi Maria SS. Institute in Troina. The dosage was suited to the technical record of the drug. During the clinical course the following items have been recorded: number of evacuations; time of alvine normalization; presence, quality, and length of fever; other associated features or side effects. RESULTS: The time of alvine normalization did not show statistically significant differences between the three groups. CONCLUSIONS: In conclusion, the use of Yovis does not modify in a statistically significant way with respect to the remaining drugs the clinical course of acute diarrhea in the mentally retarded children of the present study.

Child↗
PubMedDate not supplied

[A new syndrome of mental retardation and chromosomic instability in 3 siblings].

We report on three brothers with mental retardation, microcephaly and prominent ears whose cytogenetic studies showed no fragile X. However, an elevated frequency of spontaneous chromosome aberrations compared with that in normal individuals was found. The chromosome aberrations included acentric fragments, rings, dicentrics and radial figures. The lymphocytes did not show an increased sensitivity to mitomycin C nor to gamma radiation. It is suggested that this is a new hereditary syndrome of mental retardation with chromosome instability, probably recessive X linked.

Child↗
PubMed1996

Cloning and characterization of DXS6673E, a candidate gene for X-linked mental retardation in Xq13.1.

In several families with non-specific X-linked mental retardation (XLMR) linkage analyses have assigned the underlying gene defect to the pericentromeric region of the X chromosome, but none of these genes have been isolated so far. Here, we report on the cloning and characterization of a novel gene, DXS6673E, that maps to Xq13.1, is subject to X-inactivation and is disrupted in the 5' untranslated region by a balanced X;13 translocation in a mentally retarded female. The DXS6673E gene is highly conserved among vertebrates and its expression is most abundant in brain. It encodes a hydrophilic protein of 1358 amino acids (aa) that does not show sequence homology to other known proteins. A segment of this protein consisting of neutral and hydrophobic aa with a proline residue in every second position may represent a transmembrane domain. Almost complete sequence identity was found between the 3' end of the DXS6673E gene and two expressed sequence tags (ESTs) and between the 5' end of the DXS6673E gene and a third EST. Moreover, weaker sequence similarity was observed between coding regions and two other ESTs.

Amino Acid Sequence↗