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PubMed2007

Modifiable risk factors for low birth weight and their effect on cerebral palsy and mental retardation.

OBJECTIVE: to estimate the effect of modifiable risk factors on low birth weight and two of its sequelae-cerebral palsy and mental retardation. METHODS: The population attributable risk percent (PARP) was used as a measure of effect. A literature search was conducted to determine estimates of the percent of CP and MR attributable to low birth weight. Data from the 1996-1997 Georgia Pregnancy Risk Assessment Monitoring System (PRAMS), a population based surveillance system, were used to estimate the percent of low birth weight attributable to modifiable risk factors. The PARP was calculated for smoking and unwanted conception. RESULTS: Unwanted pregnancy and smoking were statistically significant risk factors for LBW. Four percent of all LBW births were attributable to unwanted pregnancy. If all unwanted pregnancies were prevented, 13% of cases of CP (27 cases per year in GA) and 14% of cases of MR (151 cases per year in GA) would be prevented. In wanted or mistimed pregnancies, 6% of LBW births were attributable to smoking. If all smoking during wanted or mistimed pregnancy was prevented, an additional 2.5% (5 cases) of CP and an additional 0.8% (8 cases) of MR would be prevented in Georgia each year. If all unwanted pregnancies and all smoking during wanted or mistimed pregnancies were prevented, 1692 LBW births could be prevented per year and the rate of LBW in Georgia would fall from 7.6% to 6.8%. Additionally, 32 cases of cerebral palsy and 159 cases of mental retardation could be prevented each year in Georgia. CONCLUSIONS: The PARP approach is useful in estimating the benefit of evidence-based prevention services. Preventing unwanted pregnancy and smoking during pregnancy would substantially reduce the burden of cerebral palsy and mental retardation in Georgia.

Adult↗
PubMed2000

Review of adaptive behavior studies in mentally retarded persons with autism/pervasive developmental disorder.

A review is presented of the investigations carried out concerning the adaptive behavior of persons with the dual disability of mental retardation and autism/PDD. A close correspondence is found between the results obtained by means of a Dutch set of scales, the SRZ, SGZ, and SMZ, and those obtained by means of the Vineland Adaptive Behavior Scales. Compared with matched nonautistic persons, the performance of dually disabled persons is found to be particularly poor in the domain Social Skills/Socialization and somewhat less poor in the domain Communication. Performance of the two categories does not differ in the domains Self Help/Daily Living Skills and Gross Motor Skills/Motor Skills. As a net result, persons of the dually disabled category are found to obtain comparatively low total scores for social competency, the SRZ-Total/Adaptive Behavior Composite. In addition, autistic mentally retarded persons are found to display more maladaptive behavior. For comparative studies in the mental retardation field the use of level of Self Help/Daily Living Skills and, to a lesser extent, level of Gross Motor Skills/Motor Skills are recommended as suitable matching variables.

Activities of Daily Living↗
PubMed2006

Large state-level fluctuations in mental retardation classifications related to introduction of renormed intelligence test.

Oppositely valenced forces may be at work to influence rates of placement of children into mental retardation programs. On one hand, educational policies regarding intellectual disability and concerns about overrepresentation of minorities in special education may contribute to lower placement rates; on the other hand, more difficult intelligence test norms may be a countervailing force, increasing placement rates. An analysis of longitudinal data on state and national level placement rates reveals that a lengthy and steep 12-year decline in students receiving mental retardation services reversed shortly after the introduction of the WISC-III in 1991. This phenomenon has relevance for death-penalty cases, because this historical pattern may affect the ability to establish whether an adult meets the developmental period onset criterion for mental retardation.

Adolescent↗
PubMed1981

Simple reaction times for mentally retarded and epileptic individuals.

A comparison was made to determine the effect of epilepsy on simple reaction time among 57 school-aged subjects. The subjects were classified intellectually as average, educable mentally retarded, or trainable mentally retarded, and neurologically as epileptic or nonepileptic. Following an explanation of the testing apparatus, 24 trials per day for five consecutive afternoons were given. The microswitch was depressed after a warning light and a foreperiod activated an audio response stimulus. A significant difference in RT was found between subjects with epilepsy and those without epilepsy within each intellectual classification.

Child↗
PubMed1999

Family perceptions of future programming needs in a sample of individuals with mental retardation.

The present study examined parents' perceived programming needs of their children with mental retardation. Parents (44 mothers, 8 fathers) of individuals attending programming in an agency for mental retardation responded to a telephone survey. Analysis showed parents were most concerned about future housing needs. A significant difference was found between older and younger parents in their perception of future needs.

Adult↗
PubMed1998

Risperidone for aggression and self-injurious behavior in adults with mental retardation.

Risperidone has proven efficacy with reduced likelihood of causing extrapyramidal symptoms in the treatment of schizophrenia. Initial work suggests its utility in the management of aggression and self injury in patients with mental retardation. The use of risperidone in eight adult patients with moderate to profound mental retardation is described. Risperidone in these individuals was associated with significant reduction in aggression and self injurious behavior. Side effects were primarily those of sedation and restlessness. These cases illustrate the possible utility of risperidone in the treatment of aggression and self injury in adult patients with moderate to profound mental retardation.

Adult↗
PubMed1999

X linked severe mental retardation, craniofacial dysmorphology, epilepsy, ophthalmoplegia, and cerebellar atrophy in a large South African kindred is localised to Xq24-q27.

To date over 150 X linked mental retardation (XLMR) conditions have been documented. We describe a five generation South African family with XLMR, comprising 16 affected males and 10 carrier females. The clinical features common to the 16 males included profound mental retardation (100%), mutism despite apparently normal hearing (100%), grand mal epilepsy (87.5%), and limited life expectancy (68.8%). Of the four affected males examined, all had mild craniofacial dysmorphology and three were noted to have bilateral ophthalmoplegia and truncal ataxia. Three of 10 obligate female carriers had mild mental retardation. Cerebellar and brain stem atrophy was shown by cranial imaging and postmortem examination. Linkage analysis shows the gene to be located between markers DXS424 (Xq24) and DXS548 (Xq27.3), with a maximum two point lod score of 3.10.

Abnormalities, Multiple↗
PubMed1982

Emotional disturbance and mental retardation: diagnostic overshadowing.

Two experiments evaluated the effects of the condition of mental retardation on psychologists' impressions of emotional problems of a retarded subject. In Experiment 1 we found that the same debilitating phobia was less likely to be considered an example of a neurosis or an emotional disturbance when the subject also was suggested to be mentally retarded as compared to intellectually average. Experiment 2 provided a conceptual replication of the results of Experiment 1 and extended findings of diagnostic overshadowing to cases involving schizophrenia and personality disorder. The magnitude of these effects did not differ significantly as a function of whether the case description suggested schizophrenia or personality disorder. The results validate the existence of a diagnostic overshadowing phenomenon.

Adult↗
PubMed2001

Comparative genomic hybridisation in mentally retarded patients with dysmorphic features and a normal karyotype.

Segmental aneusomy for small chromosomal regions has been shown to be a common cause of mental retardation and multiple congenital anomalies. A screening method for such chromosome aberrations that are not detected using standard cytogenetic techniques is needed. Recent studies have focused on detection of subtle terminal chromosome aberrations using subtelomeric probes. This approach however excludes significant regions of the genome where submicroscopic rearrangements are also liable to occur. The aim of the present study was to evaluate the efficiency of comparative genomic hybridisation (CGH) for screening of submicroscopic chromosomal rearrangements. CGH was performed in a cohort of 17 patients (14 families) with mental retardation, dysmorphic features and a normal karyotype. Five subtle unbalanced rearrangements were identified in 7 patients. Subsequent FISH studies confirmed these results. Although no interstitial submicroscopic rearrangement was detected in this small series, the study emphasises the value of CGH as a screening approach to detect subtle chromosome rearrangements in mentally retarded patients with dysmorphic features and a normal karyotype.

Cytogenetic Analysis↗
PubMed1983

Effects of thioridazine on the intellectual performance of mentally retarded drug responders and nonresponders.

Forty mentally retarded adolescents were divided into two divisions on the basis of prestudy assessments that identified subjects as thioridazine hydrochloride responders or nonresponders. Within each division, the participants were assigned randomly to one of four groups. Members of each group received three administrations of the Leiter international performance scale. Four conditions were examined: testing under standard conditions and testing under reinforcement conditions while receiving and not receiving thioridazine. Condition sequences were random in all four groups. Assessments were double-blind and placebo controlled. The results showed that while both drug responders and nonresponders were receiving thioridazine there were no major differences in test scores obtained under standard and reinforcement conditions. While subjects were not receiving thioridazine there were considerable increases in scores obtained under the reinforcement conditions.

Adolescent↗
PubMed1996

Fragile X syndrome and fragile XE mental retardation.

There are two forms of mental handicap associated with fragile sites on the end of the long arm of the X chromosome. The well known common disorder Fragile X syndrome is associated with FRAXA and a rare non-specific form of mental handicap is associated with FRAXE. The cytogenetics of these fragile sites is considered. For Fragile X syndrome details are given of the molecular genetics, inheritance patterns, genetic counselling, methods for diagnosis of index cases, carrier detection and prenatal diagnosis. Series of prenatal diagnoses are briefly reviewed and technical and biological problems associated with this procedure are considered. Prenatal diagnosis of Fragile X syndrome using molecular genetic techniques is now a well established procedure, with the only significant problem being the inability to accurately predict phenotype in female fetuses with full mutations. Few prenatal diagnoses of Fragile XE non-specific mental retardation have been recorded. In principle the technical aspects of such a prenatal diagnosis should be little different from those for Fragile X syndrome. Incomplete knowledge of the phenotypic effect of the full mutation in males and females would make phenotypic prediction for any fetus shown to have such a mutation very difficult. At this stage all that could be determined with precision is that the mutation was present or absent in the fetus. Possible consequences of this are discussed.

Female↗
PubMed1999

Simple mental addition in children with and without mild mental retardation.

The speeded performance on simple mental addition problems of 6- and 7-year-old children with and without mild mental retardation is modeled from a person perspective and an item perspective. On the person side, it was found that a single cognitive dimension spanned the performance differences between the two ability groups. However, a discontinuity, or "jump," was observed in the performance of the normal ability group on the easier items. On the item side, the addition problems were almost perfectly ordered in difficulty according to their problem size. Differences in difficulty were explained by factors related to the difficulty of executing nonretrieval strategies. All findings were interpreted within the framework of Siegler's (e.g., R. S. Siegler & C. Shipley, 1995) model of children's strategy choices in arithmetic. Models from item response theory were used to test the hypotheses.

Case-Control Studies↗
PubMedDate not supplied

Genetic analysis of a group of mentally retarded children.

Genetic analysis of 169 mentally retarded (MR) children from Madras, revealed chromosomal abnormalities in 17%. Down syndrome was the major chromosomal anomaly (24/169 = 14.2%). These included three cases of trisomy-21 mosaics, and one case of de novo Robertsonian translocation. MR children with chromosomal abnormalities were either mildly or moderately retarded. Syndromes with known etiology occurred in 3% of the MR cases. Microcephaly, neonatal anoxia, perinatal stress and pharmacological attempt for abortion were found to be important pathogenic factors associated with MR. Most of the microcephalics (11/169 = 6.5%) were severely retarded, whereas those associated with neonatal anoxia and perinatal stress were either mildly or moderately retarded. Birth-order effects were found only among Down syndrome patients. Segregation analysis of the three groups of proband families (viz. mild, moderate and severe MR) indicated that autosomal recessive mode of inheritance is compatible in moderate and severe MR proband families. The proportion of X-linked instances of MR is estimated to be about 22% of the cases.

Child↗
PubMed2005

Maternal mosaicism for mutations in the ARX gene in a family with X linked mental retardation.

We describe two brothers with mental retardation (MR) due to a c.428_451dup24 in the ARX gene. The mother did not apparently carry the mutation, as determined by dHPLC and by fragment size analysis. Using semiquantitative fluorescent PCR, we show however that 4% of her lymphocytes and 24% of her fibroblasts harbored the duplication. We thus show that the mother displays somatic mosaicism for the duplication thereby highlighting the need to reconsider the molecular screening in sporadic cases of MR.

Amino Acid Sequence↗
PubMed1982

Phonological process identification of misarticulations of mentally retarded children.

Speech samples of 20 mentally retarded children between the ages of 6 yr, 4 mo and 15 yr were analyzed for the purpose of identification of systematic patterns. Liquid deviations and cluster reductions were the most prevalent phonological processes evidenced in their misarticulations. Postvocalic obstruent omissions, deviations of other sonorants (glides and nasals), velar deviations, stridency deletion, stopping, and /theta, sigma/ deviations were demonstrated less frequently. In addition, the children demonstrated pre- and postvocalic devoicing.

Adolescent↗
PubMed1989

Establishing control by spoken words with profoundly mentally retarded individuals.

Five low-functioning mentally retarded subjects were taught initially to insert tokens in a slot only when an auditory-visual complex stimulus was presented. The auditory component was a spoken reinforcer name (e.g., "Food"), and the visual component was a flashing red light in the slot opening. Later, the name was presented without the light on probe trials. Three subjects did not respond, suggesting that their token insertions had been controlled by the visual but not by the auditory component of the auditory-visual complex. These subjects then received a fading program designed to establish auditory stimulus control. The program was successful with two subjects. The third subject required post-program remedial teaching, but ultimately demonstrated exclusive control by the spoken name. The results suggest methods for establishing auditory stimulus control with this population and highlight variables that may influence the reliability of such control in some subjects.

Acoustic Stimulation↗
PubMedDate not supplied

Initiating requests during community-based vocational training by students with mental retardation and sensory impairments.

Students with mental retardation and deafness or deaf-blindness often need some type of communication system to communicate effectively with communication partners during community-based vocational training. However, students may need specific training to learn how to initiate requests for items or assistance, a skill identified as critical for job success. Students were taught to initiate requests using dual communication boards and gestures. Data were recorded on student performance using a multiple-baseline probe design in which data were collected during baseline, intervention, and generalization phases. Students were able to initiate requests with 80% to 100% accuracy with the communication system at vocational sites. Training students to initiate requests may need to be targeted when students are first learning a job, as this is when most naturally occurring opportunities exist.

Adolescent↗