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Verb usage by educable mentally retarded children.

The purpose of this study was to investigate the semantic and morphological nature of verb usage by educable mentally retarded (EMR) children. Three groups of children were studied in structured and non-structured situations in which the verb system was the core interest. There were 15 mentally retarded younger children (8.6-9.6 years), 19 mentally retarded older children (10.0-12.6 years) and a control group of 17 normal children (5.0-6.0 years). Results showed that EMR children use fewer conjugations than normal children of similar or lower mental age, and thus they can express fewer verb-case relations. In particularly EMR children show limited use of process-verb relations. It was further found that EMR children are restricted in the morphological processing of verbs in past and future tenses.

Child↗

Predicting adequacy of parenting by people with mental retardation.

While there has been increased attention placed upon the inadequacy of parenting by persons with mental retardation, such attention does little to increase our understanding of the complexities of parenting. A refocusing of research and intervention efforts is needed to examine the concomitants of adequacy of parenting. What we know about both the inadequacy as well as the adequacy of parenting by persons with mental retardation and the limitations of the information upon which we base these statements is reviewed. New foci are suggested in understanding both adequacy and the inadequacy of parenting by persons with mental retardation.

Adolescent↗

Group conversational-skills training and social validation with mentally retarded adults.

Seven community-dwelling, mildly and moderately mentally retarded adults participated in a group social skills training program designed to improve conversational skills. A group multiple-baseline design was used. Conversational components trained were asking questions about the conversational partner, giving compliments, and appropriately disclosing information about oneself. Training sessions consisted of instructions, modeling, and behavioral rehearsal. Assessment during baseline and treatment consisted of recording conversations between subject dyads after each session. Generalization of training was assessed during baseline and treatment and at 1-month, 3-month, and 6-month follow-ups by recording conversations between subjects and novel, nonretarded persons. Behavioral ratings of the tapes indicated substantial improvements in the targeted behaviors as a function of training. Subjective social validity ratings of pretraining and posttraining generalization tapes by community volunteers reflected improvements in their social perceptions of subjects as a function of training. The results suggest that community-dwelling, mentally retarded adults can be taught to make changes in their conversational behavior that are viewed positively by others living in their communities.

Adult↗

The effects of exercise on the health, intelligence, and adaptive behavior of institutionalized severely and profoundly mentally retarded adults: a systematic replication.

Institutionalized severely and profoundly mentally retarded adults participated in a 7-month program of rigorous aerobic-type exercises. The effects of the treatment on the physical fitness, intelligence, and behavior of subjects were assessed. Fifty men and women were matched in pairs based on IQ, CA, and sex and assigned randomly to an experimental (E) or control (C) group. Those in the E group met 3 hours per day, 5 days per week and received a treatment that consisted of an exercise program that included jogging, running, dance-aerobics, and circuit training. The C group continued their normal institutional training programs. The treatment produced significant improvement in the cardiovascular efficiency of subjects; however, no changes in intelligence or adaptive behavior were obtained. Although standardized tests reflected little improvement in psychological or behavioral variables due to treatment, subjective reports suggest that exercise training may serve as a more practical habilitation program for severely and profoundly mentally retarded individuals than those typically employed in institutional settings.

Adolescent↗

The genetics of mental retardation.

We review the advances that have been achieved using molecular genetic techniques to characterise the genetic basis of mental retardation in recognised syndromes and in discovering new genetic conditions that give rise to mental retardation. However, even with the cloning of a number of mutant genes, the pathogenesis of the condition is not understood. The genetics of mental retardation are turning out to be remarkably complex and are likely to remain a challenge for clinicians and scientists for years to come.

Chromosome Aberrations↗

Duplication of 11p14.3-p15.1 in a mentally retarded proband and his mother detected by G-banding and confirmed by high-resolution CGH and BAC FISH.

A 10-year-old African-American male has been followed since 2 years of age due to his mental retardation, severe behavioral problems, and dysmorphism. Conventional cytogenetic analysis, chromosome painting, high-resolution comparative genomic hybridization (HR-CGH), and bacterial artificial chromosome fluorescent in situ hybridization (BAC FISH) revealed an apparent duplication in the short arm of a chromosome 11, dup(11)(p14.3p15.1), seen also in his mentally retarded mother. The proband had moderate to severe mental retardation, a history of IUGR, infantile hypotonia, FTT, exotropia, inguinal hernia repair, and several dysmorphic features. His mother had mild mental retardation, a history of impulsivity, assaultive outbursts, and similar dysmorphism. Although G-banding and FISH indicated a duplication, HR-CGH confined the localization of material to bands 11p14-11p15 and aided the selection of locus-specific BAC clones to more precisely characterize the duplicated region. To our knowledge, the results represent the first example of a familial, cytogenetically visible duplication of euchromatin in 11p that excludes the Beckwith-Wiedemann syndrome critical region. It is possible that one or more genes had been disrupted at the breakpoints of the above structural chromosomal rearrangement giving rise to the present phenotype.

Abnormalities, Multiple↗

Direct-care workers' attributions of psychopathology in adults with mental retardation.

The processes by which direct-care workers identify psychological disorder and need for treatment in adults with mental retardation was examined. A series of vignettes-varying in problem type, client's mental retardation level, and client's gender-was administered to 144 direct-care workers from four types of work settings. Results indicate that although the workers demonstrated an overall ability to accurately identify psychopathology, problem type, and client's mental retardation level were significantly related to workers' judgments regarding the presence of psychological disorder and need for treatment. Worker's setting and client's gender were not significant predictors. Implications for staff training and for increasing the accessibility of mental health services are considered.

Attitude to Health↗

Cytogenetic surveillance of mentally-retarded school children in southern Taiwan.

BACKGROUND AND PURPOSE: Mental retardation (MR), defined as having an IQ of less than 70, is present in approximately 2 to 3% of the population. Data on chromosomal abnormalities, an important cause of MR, are limited in the Taiwanese literature. This study evaluated the frequency and pattern of chromosomal abnormalities in school children with MR in southern Taiwan. METHODS: Peripheral blood samples of 419 children were collected from November 1999 to January 2003. Those with Prader-Willi syndrome (PWS), Angelman syndrome (AS) or fragile-X syndrome were excluded from the study. Metaphase chromosome preparations were obtained from peripheral blood cultures, and trypsin-giemsa (GTG) banded chromosomes were examined at the level of 500 to 600 bands. Fluorescence in situ hybridization was done for cases whose karyotypes could not be determined by conventional cytogenetic analysis. RESULTS: Of the 419 enrollees with MR, 10 had mild MR, while most had moderate to profound MR. Chromosomal abnormalities were found in 22.43% of the cases, with trisomy 21 being the major chromosomal abnormality, occurring in 77 cases (18.38%, 77/419 cases). Sex chromosome aneuploidies were found in 3 cases (0.72%, 3/419 cases). Structural abnormalities of autosomes were found in 13 cases (3.10%, 13/419 cases), including deletion, markers, unbalanced translocations, and inversions. One subject was found to have monosomy 20 mosaicism (0.24%, 1/419). CONCLUSIONS: Chromosomal abnormalities occurred in a high proportion of mentally retarded school children from southern Taiwan, with trisomy 21 being the most prevalent. These findings indicate the need for increased attention to prenatal, perinatal and postnatal screening in this population.

Child↗

Mental retardation in teenagers: prevalence data from the Niagara region, Ontario.

OBJECTIVES: There are few Canadian prevalence studies of mental retardation (MR); those that do exist were conducted prior to the era of community integration. We undertook a population-based study to explore mental health disturbances in young persons with MR. The first requirement was to identify a population with MR and to establish its prevalence. Here, we report data on the prevalence of MR in a population aged 14 to 20 years. METHOD: We conducted the study in the Niagara Region of Ontario, which has a population base of around 400,000. Researchers worked closely with schools and with agencies providing services to persons with MR to identify the study group. We confirmed the functioning level of participants through standard tests of nonverbal intelligence and receptive language; teachers and other service personnel provided information relevant to the estimation of nonparticipants' functioning level. RESULTS: We identified 255 individuals as having MR (IQ < or = 75). Of these, 171 chose to participate (defined as "participants with MR"; the remaining 84 were "nonparticipants with MR"). Thus, the participation rate was 67% (171/255). Participants and nonparticipants with MR did not differ on age, sex, or IQ, although there were more nonparticipants in the lower social strata. Overall prevalence for MR was 7.18/1000. For mild mental retardation (MMR; that is, IQ = 50 to 75), prevalence was 3.54/1000, and for severe mental retardation (SMR; that is, IQ < 50), it was 3.64/1000. CONCLUSIONS: Our prevalence estimate for SMR is similar to rates from previous studies conducted worldwide. Our estimate for MMR parallels the lower rates found in Scandinavian countries and contrasts with the higher rates generally reported in the US.

Adolescent↗

Onychotrichodysplasia and chronic neutropenia without mental retardation (ONS): a second case report.

Onychotrichodysplasia, chronic neutropenia and mental retardation syndrome (ONMRS) is a rare autosomal recessive mutation, mostly reported in patients of Mexican ancestry. We describe a second patient with onychotrichodysplasia and chronic neutropenia without mental retardation (ONS). It is unclear if ONS found in the two European patients with normal mental development is due to genetic heterogeneity or variable expressivity of the same syndrome.

Adult↗

Exonic microdeletions in the X-linked PQBP1 gene in mentally retarded patients: a pathogenic mutation and in-frame deletions of uncertain effect.

Mutations in PQBP1 were recently identified in families with syndromic and non-syndromic X-linked mental retardation (XLMR). Clinical features frequently associated with MR were microcephaly and/or short stature. The predominant mutations detected so far affect a stretch of six AG dinucleotides in the polar-amino-acid-rich domain (PRD), causing frameshifts in the fourth coding exon. We searched for PQBP1 exon 4 frameshifts in 57 mentally retarded males in whom initial referral description indicated at least one of the following criteria: microcephaly, short stature, spastic paraplegia or family history compatible with XLMR, and in 772 mentally retarded males not selected for specific clinical features or family history. We identified a novel frameshift mutation (23 bp deletion) in two half-brothers with specific clinical features, and performed prenatal diagnosis in this family. We also found two different 21 bp in-frame deletions (c.334-354del(21 bp) and c.393-413del(21 bp)) in four unrelated probands from various ethnic origins, each deleting one of five copies of an imperfect seven amino-acid repeat. Although such deletions have not been detected in 1180 X chromosomes from European controls, the c. 334-354del(21 bp) was subsequently found in two of 477 Xs from Indian controls. We conclude that pathogenic frameshift mutations in PQBP1 are rare in mentally retarded patients lacking specific associated signs and that the 21 bp in-frame deletions may be non-pathogenic, or alternatively could act subtly on PQBP1 function. This touches upon a common dilemma in XLMR, that is, how to distinguish between mutations and variants that may be non-pathogenic or represent risk factors for cognitive impairment.

Adolescent↗

[Diagnosis of pervasive developmental disorders without mental retardation and its impact on obtaining social and educational services in Quebec].

Pervasive developmental disorders without mental retardation is a new clinical category including high-functioning autism, Asperger syndrome and pervasive developmental disorder non otherwise specified. Its recognition is recent and still problematic in many regards. This article reviews the historical, theoretical and clinical relations between pervasive developmental disorders without mental retardation and bordering disorders. The consequences of an inadequate diagnosis on measures of assistance for these patients is also investigated. The authors conclude on the necessity in considering, independently of the diagnosis, the description of symptoms, the intellectual level and the adaptative level in order to take the most appropriate educational and psychosocial decisions regarding pervasive developmental disorders without mental retardation.

Child↗

Urinary tract infections during pregnancy and mental retardation and developmental delay.

OBJECTIVE: To investigate the association between urinary tract infections during pregnancy and mental retardation or developmental delay in infants. METHODS: An inception cohort design was used to analyze Medicaid maternal and infant-linked records and vital records for 41,090 pregnancies from 1995-1998. RESULTS: The relative risk (RR) for mental retardation or developmental delay among infants of mothers with diagnosed urinary tract infections but no antibiotic claims was 1.31 with a 95% confidence interval (CI) of 1. 12, 1.54 compared with the group without urinary tract infections. The RR for infants of mothers with urinary tract infections without antibiotic claims was 1.22 (95% CI 1.02, 1.46) compared with infants of mothers with urinary tract infections and antibiotic claims. The RR was significant in the first trimester (1.46, 95% CI 1.07, 1.99) and third trimester (1.41, 95% CI 1.11, 1.79) after controlling for race and gestational age at birth. CONCLUSION: There was a statistically significant association between maternal urinary tract infections without evidence of antibiotics and mental retardation or developmental delay in infants. The relationship persisted when we assumed that over 30% of women who had antibiotic claims filled but did not take the medicine, and 40% of the women who did not have antibiotic claims did take the medication.

Adolescent↗

An epidemiological study on severe mental retardation among schoolchildren in Bologna, Italy.

The prevalence, causal origin of and impairments associated with severe mental retardation (SMR) were investigated among all school-age children (six to 13 years) living in the city of Bologna, Italy. 90 children (57 boys, 33 girls) with IQs less than or equal to 50 were identified. The prevalence of SMR was 4.2 per 1000 for males, 2.5 per 1000 for females and 3.4 per 1000 for both sexes. Causal origin was prenatal for 33.3 per cent, perinatal for 14.4 per cent, combined pre- and perinatal for 5.6 per cent and postnatal for 13.3 per cent. Another 12.3 per cent of the children with IQs less than or equal to 50 had autism or childhood psychosis, while there was no evident cause of mental retardation for the remaining 21.1 per cent. 50 per cent had at least one associated physical or neurological impairment other than mental retardation, with epilepsy and cerebral palsy predominating.

Adolescent↗

The pattern of childhood epilepsy with mental retardation in Nigeria.

Of 580 epileptic children, 353 males and 227 females, seen at the Paediatric Neurology Clinic of the University of Nigeria Teaching Hospital Enugu, from 1985 to 1987, 18 per cent were mentally retarded, a much higher prevalence rate than in the general population. Nine different seizure types were seen, with generalized tonic-clonic seizures (grand mal) leading in frequency. The highest incidence of mental retardation occurred among the children with infantile spasms (51 per cent). For all the seizure types, there was a long delay in seeking medical attention (mean interval, 1.7 years). The mean interval for epileptics with mental retardation was even higher (2.77 years). Factors found to increase the chances of an epileptic child having mental retardation include episodes of status epilepticus, early age at onset of seizures, and long delay before presentation to hospital for treatment. There is need for increased efforts aimed at the elimination of these factors and also for a well organized programme to educate the population about the nature of epilepsy, and the importance of compliance with the treatment schedule.

Adolescent↗

Long-term administration of valproic acid in the treatment of affective symptoms in people with mental retardation.

We report the results of an open trial of valproic acid in the treatment of affective symptoms in people with mental retardation. The study population consisted of 209 people with mental retardation who were serially referred to a tertiary-care medical center for the evaluation of behavioral symptoms. Criteria for treatment included the presence of three of the four following symptoms: irritability, sleep disturbance, aggressive or self-injurious behavior, and behavioral cycling. Twenty-one patients met enrollment criteria and were studied prospectively for a 2-year period. Two patients were lost to follow-up. One patient experienced severe drug side effects. Eighteen patients completed the study. Fourteen patients (78%) responded favorably to treatment and were maintained on valproic acid for the 2 years of the study (p < 0.001). Medications prescribed at the time of enrollment were usually discontinued, including neuroleptic medication in 9 of 10 patients and in all patients (N = 3) who were receiving phenobarbital. A history of epilepsy or a suspicion of seizures was strongly associated with a favorable response to valproic acid (p < 0.005). The results of this study suggest that people with mental retardation and concurrent affective disorders can be recognized by a cluster of developmentally appropriate symptoms such as those listed above. In addition, affective symptoms can be successfully treated with valproate with reductions in neuroleptic and barbiturate anticonvulsant medication. Further study of the comparative benefit of valproate and carbamazepine in this population is warranted.

Adolescent↗

Control of epilepsy in a mentally retarded population: lack of correlation with IQ, neurological status, and electroencephalogram.

An investigation of 90 children with mental retardation and epilepsy who were seen at a developmental evaluation clinic was conducted to determine whether control of their epilepsy was related to their level of mental retardation. Their level of retardation, neurological findings, and EEGs were classified and compared with the quality of seizure control. No correlation was found between quality of control and any of these parameters. Results suggest that there is no firm relation between the level of mental retardation and control of epilepsy. It also appears that the neurological status and EEG do not affect quality of seizure control for this group.

Adolescent↗

Attitudes toward mentally retarded persons: effects of attitude referent specificity.

The effects of the specificity of the attitude referent on female adults' expressed attitudes were studied. Specifically, attitudes towards "mentally retarded person" referent were compared with attitudes toward mentally retarded referents who were described in terms of their severity of retardation and CA. Results indicated that expressed attitudes toward a nondescript mentally retarded person referent were generally intermediate in favorability between mildy and severely retarded person referents. The response format of the attitude questionnaire (e.g., Likert, forced-choice) was also found to affect attitude scores differentially as a function of the referent employed.

Age Factors↗