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PubMed2000

Mental retardation with rare fragile site expressed at 2q11.

Several rare autosomal folate sensitive fragile sites were reported in individuals with mental retardation, neurological abnormalities, and multiple congenital malformations. Only three of them: fra(11)(q22.3), fra(X)(q27.3) and fra(X)(q28), are known to be associated with mental retardation and phenotypic abnormalities. A possible association of the other rare fragile sites with idiopathic mental retardation is still being discussed. Here, a girl who has a fragile site at 2q11 with minor congenital anomalies and mental retardation is presented. This case has recalled the question of idiopathic mental retardation that might be the clinical expression of rare FSFS. Fragility was observed at 2q11 with a frequency of 3% in her cells along with a partial endoreduplication at 2 q11-->qter.

Child↗

PubMed1992

Imaginative play in children with mental retardation.

We examined the play behavior of 18 kindergarten children without mental retardation and 55 children with different levels of mental retardation, all with a developmental age of 4 to 5 years, and found few differences between the groups with regard to activity, types and quality of play, and play content. Qualitative differences as described in the literature were not confirmed. This may have been due to (a) the play situation, which was an individual contact with a stimulating adult and/or (b) the fact that MA and not CA was the standard of comparison. Consequently, mild to moderate mental retardation seems in itself insufficient reason to exclude children from play intervention programs.

Adolescent↗

PubMed1991

Self-concepts of adolescent siblings of children with mental retardation.

Self-concepts of 70 adolescent siblings of children with profound levels of mental retardation were investigated. Their mean self-concept was compared with a matched group of 70 adolescent siblings of children without profound levels of retardation. Using the Tennessee Self-concept Scale, two variables (maternal self-concept and siblings' self-concept) were assessed for differences between siblings and mothers (the target group) of mentally retarded children and siblings and mothers (the comparison group) of children without mental retardation. No significant mean difference between the two groups suggests no special difficulties in psychosocial adjustment for siblings of the mentally retarded.

Adaptation, Psychological↗

PubMed1991

Self-concepts of mothers who show mental retardation.

The Tennessee Self-concept Scale was orally administered to 27 mothers with mental retardation and self-administered to 95 mothers whose children attended daycare in the areas in which the former mothers lived. Analyses showed that the mothers with mental retardation scored significantly lower on all subscales. Where 72% of the comparison group scored above a recommended cut-off indicative of a positive self-concept, only 33% of the mothers with mental retardation did. Comparisons of the 27 mothers with mental retardation (18 with positive versus 9 with negative self-concepts) on personal, child, family, and other background variables suggested different relationships for the two groups. Cross-validation of the present work and studies of the actual childcare of these latter two groups of mothers are needed.

Adult↗

PubMed1994

Automatized responding and cognitive inertia in individuals with mental retardation.

The relation between cognitive rigidity and cognitive inertia was examined. Results showed that cognitive inertia was greater in individuals with mental retardation than in CA- but not MA-matched subjects without mental retardation. For individuals with mental retardation, cognitive inertia was positively correlated with CA. Results of comparisons of MA-matched individuals with and without mental retardation were not consistent with Kounin's (1941) theory. However, the current findings provided some support for Kounin's notion that there are age-related inherent structural differences that lead to greater rigidity in older adults.

Adolescent↗

PubMed1997

Mentally retarded persons as parents: prevalence and the situation of their children.

This paper describes a research program on mentally retarded parents and their children. For this nation-wide study, two structured questionnaires were sent to all municipalities in Norway. Both questionnaires were sent to public health nurses and were followed-up by a structured telephone interview based on the questionnaires. The primary aim of the study was to survey the number of children born to mentally retarded parents. In addition, the children's needs and functional abilities were to be assessed. Twenty-three mentally retarded persons had given birth in the course of the past twelve months. A total of 126 children with mentally retarded parents were identified, with an incidence of 27 children per year, and a prevalence of approximately 430 children under 16 years of age in a population of 4 mill, people with a mean of 1.05 child per family. About 43% of the children of mentally retarded parents appeared to have learning difficulties. Forty percent of the children suffered from failures of care. Between 25% and 68% of the children with learning problems had poorly developed motor or language abilities, sense modalities or psycho-social status.

Adolescent↗

PubMed1995

Mental and chronological age as predictors of age-appropriate leisure activity in children with mental retardation.

Effects of MA and CA on everyday leisure-time behaviors of children with mild and moderate mental retardation were examined by comparing 40 children with mental retardation to groups of 40 MA- and 40 CA-matched children without mental retardation on an adult-reported scale. Child's overall score, highest single behavior performed by the child, and highest behavior not performed because it was considered below the child's level were measured. Children with retardation performed most similarly to their MA-matches and well-below levels of CA-matches. Older children received higher scores in all groups. Late adolescents with retardation performed between MA- and CA-levels on the highest behavior considered too immature. Although MA best predicted overall functioning on leisure-time behavior of children with retardation, CA best predicted both single highest behavior performed and highest behavior considered too immature. Implications for teaching leisure-time behaviors to individuals with mental retardation were discussed.

Activities of Daily Living↗

PubMed1988

A comparison of peer-directed and teacher-directed employment interview training for mentally retarded adults.

Interview skills deficits may limit employment prospects of mentally retarded adults. Although numerous papers highlight the importance of interview skills, few have validated effective strategies for use with mentally retarded persons. Further, there has been a lack of research contrasting rival interview skills training strategies. The present study was conducted with two mentally retarded young adults. It contrasted peer-directed instruction, in which both participants were equally deficient in the target skills, with teacher-directed instruction. Results of the investigation indicated that instruction, rehearsal, and feedback may be effective strategies regardless of who provides instruction. Comparisons of teacher-directed and peer-directed instruction indicated little or no difference in the effectiveness of the two procedures. However, the peer-directed procedure involved considerably less staff time than did the teacher-directed procedure.

Adult↗

PubMed1991

Ascribed role relations between children with mental retardation and their younger siblings.

Ascribed, parent-mediated childcare roles of younger same-sex siblings of children with mental retardation were compared to roles assumed by a matched group of comparison siblings. Role reversals were evident for younger siblings of children with mental retardation. These reversals were consistent with roles assumed by the siblings during observed interactions. Increased younger sibling childcare roles were related to less conflicted sibling relationships but were not predictive of friendship or socialization experiences of younger siblings. Younger siblings of children with mental retardation had fewer household chores than did comparison agemates. Children with mental retardation had fewer friendship contacts and out-of-home activities than did their peers without mental retardation. Important within-group differences were evident.

Activities of Daily Living↗

PubMed1999

Psychometric assessment of families with X-linked mental retardation.

As part of an integrated approach to DNA-linkage analysis in X-linked mental retardation (XLMR), 29 members of five families suspected of having XLMR underwent psychometric assessment. Mental retardation was confirmed in all participants. The range of mental retardation varied from mild to profound within and between families. In addition, these preliminary results indicated family-specific cognitive profiles in MRX45 and MRX46. The fact that two non-overlapping loci were involved provides strong evidence that specific cognitive profiles are linked to specific loci (genes) in mental retardation. We therefore recommend the application of standardised psychometric tests for the assessment of XLMR.

Adult↗

PubMed1995

Simple reaction times and timing of serial reactions of adolescents with mental retardation, autism, and Down syndrome.

The purpose of this study was to examine the serial information processing in adolescents with mental retardation, autism, and Down syndrome by using a serially patterned tracking task. Analyses indicated that 7 adolescents with mental retardation, 8 with autism, and 3 with Down syndrome had significantly slower and more variable simple reaction times than did 10 college students. Also, the autistic adolescents had significantly faster mean simple reaction time than those with Down syndrome. On a task of tracking serial light stimulation, mentally retarded adolescents had significantly faster reaction time than college students. The autistic subjects excessively had faster anticipatory reaction time than did the subjects in the other three groups. On the other hand, adolescents with Down syndrome had markedly slower and more variable reaction time than did adolescents with non-Down-syndrome mental retardation. As for motor organization of keystrokes on the tracking task, mentally retarded adolescents responded with six movements, in which these individuals pressed a series of keys 1, 2, 3, 4, 5, and 6, as a chunk, as exhibited by college students. Adolescents with autism and Down syndrome, however, did not produce this movement-output chunking.

Adolescent↗

PubMed1979

Problems with the use of conventional anticonvulsant drugs in mentally retarded individuals.

Special problems in the use of anticonvulsant agents in the mentally retarded are reviewed. Effects of barbiturates on mental abilities and of phenytoin on facial appearance are especially undesirable side effects of these agents in mentally retarded individuals. Careful consideration should be given to selection of anticonvulsants that minimally interfere with the long-term rehabilitation of mentally retarded persons. The relationship of paroxysmal EEG abnormalities, episodic violence and hysteroepilepsy to epilepsy is discussed.

Adolescent↗

PubMed1995

A new X-linked mental retardation syndrome.

There are now more than 70 X-linked mental retardation syndromes associated with physical findings documented in McKusick's "Mendelian Inheritance in Man". We describe a boy and his maternal uncle who appear to have a previously unreported X-linked mental retardation syndrome, consisting of moderate mental retardation, coarse facial features, epilepsy and slowly progressive joint contractures.

Adolescent↗

PubMed2004

[Dental and periodontal status and treatment needs of institutionalized mentally retarded children from the province of West Pomerania].

The aim of this study was to assess the effect of mental retardation and lack of parental care on oral health and dental treatment needs in schoolchildren from the province of West Pomerania. Children with moderate mental retardation, healthy children living at an orphanage, and healthy children living with their parents were compared. Dental health, periodontal status, treatment needs, and oral hygiene indicators were studied in 365 children aged 11 to 13 years and 14 to 16 years. The incidence of caries in mentally retarded children was 100%. Mean DMF was 7.31 and 10.14 in the younger and older age group of these children, respectively. In comparison with healthy children, mentally retarded children demonstrated worse dental and periodontal status and poorer oral hygiene. Moreover, caries prophylaxis was not satisfactory in this group. The results show that mentally retarded children need professional and regular dental care within the framework of a dedicated prophylactic and therapeutic program.

Adolescent↗

PubMed1998

Prevalence and correlates of mental retardation among children in Karachi, Pakistan.

This paper reports estimates of the prevalence of mental retardation and associated factors based on a population survey of 2- to 9-year-old children in Greater Karachi, Pakistan. A two-phase survey was implemented during the years 1988-1989. In the first phase, a cluster sample of 6,365 children (5,748 from urban areas and 617 from rural areas) was screened for disabilities using a parental report known as the Ten Questions instrument. In the second phase, all children with positive screening results and a 10% sample of those with negative results were referred for structured medical and psychological assessments. Estimates of the prevalence of mental retardation were 19.0/1,000 children (95% confidence interval (CI) 13.5-24.4) for serious retardation and 65.3/1,000 children (95% CI 48.9-81.8) for mild retardation. Both estimates were considerably higher than respective prevalence estimates obtained in industrialized countries and in selected less developed countries. In this population, lack of maternal education was strongly associated with the prevalence of both serious (odds ratio = 3.26, 95% CI 1.26-8.43) and mild (odds ratio = 3.08, 95% CI 1.85-5.14) retardation. Other factors that were independently associated with mental retardation in Karachi included histories of perinatal difficulties, neonatal infections, postnatal brain infections, and traumatic brain injury, as well as current malnourishment. Further research is needed to assess the contribution of consanguineous marriage, improvements in child survival, and other factors to the unusually high prevalence of mental retardation in this population.

Age of Onset↗

PubMed1981

Nonprogressive cerebellar disorder with mental retardation and autosomal recessive inheritance in Hutterites.

We report a nonprogressive neurological disorder in at least 11 Hutterites with healthy but consanguineous parents. In several of the affected, hypotonia was noted at birth. Retarded motor and mental development became apparent during the first year of life. The age of unsupported walking varied from 5-21 years. Consistent signs were unsteady, broadly based gait and stance, exaggerated deep tendon reflexes mainly in the lower limbs, and mild to moderate mental retardation. Variable signs were extensor plantar reflexes (9/11), short stature (-2SD in 8/11), strabismus (7/11), small muscle mass (6/11), mild intention tremor (3/11), cataracts (1/11), and epilepsy (1/11). CAT scans in two affected sisters showed slight enlargement of the fourth ventricle in one and hypoplasia of the cerebellum in both. The disorder is probably the same as that described earlier under the heading, dysequilibrium syndrome.

Adolescent↗

PubMed1989

Autosomal recessive intestinal lymphangiectasia and lymphedema, with facial anomalies and mental retardation.

We report on two male and two female relatives with intestinal lymphangiectasia; severe lymphedema of limbs, genitalia, and face; facial anomalies; seizures; mild growth retardation; and moderate mental retardation. Main facial anomalies are a flat face, flat nasal bridge, hypertelorism, small mouth, tooth anomalies, and ear defects. Their parents are consanguineous. This disorder probably is an hitherto undescribed autosomal recessive syndrome.

Abnormalities, Multiple↗

PubMed2002

Premature chromosome condensation in humans associated with microcephaly and mental retardation: a novel autosomal recessive condition.

We report a novel autosomal recessive disorder characterized by premature chromosome condensation in the early G2 phase. It was observed in two siblings, from consanguineous parents, affected with microcephaly, growth retardation, and severe mental retardation. Chromosome analysis showed a high frequency of prophase-like cells (>10%) in lymphocytes, fibroblasts, and lymphoblast cell lines with an otherwise normal karyotype. (3)H-thymidine-pulse labeling and autoradiography showed that, 2 h after the pulse, 28%-35% of the prophases were labeled, compared with 9%-11% in healthy control subjects, indicating that the phenomenon is due to premature chromosome condensation. Flow cytometry studies demonstrate that the entire cell cycle is not prolonged, compared with that in healthy control subjects, and compartment sizes did not differ from those in healthy control subjects. No increased reaction of the cells to X-irradiation or treatments with the clastogens bleomycin and mitomycin C was observed, in contrast to results in the cell-cycle mutants ataxia telangiectasia and Fanconi anemia. The rates of sister chromatid exchanges and the mitotic nondisjunction rates were inconspicuous. Premature entry of cells into mitosis suggests that a gene involved in cell-cycle regulation is mutated in these siblings.

Cell Cycle↗