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PubMed1978

Behavioral changes in nonambulatory, profoundly mentally retarded individuals.

Nonambulatory, profoundly mentally retarded (NPMR) individuals seldom have been studied as a distinct population. This investigation sought to describe the pshysical, etiological, and behavioral characteristics of NPMR children and adults and to evaluate the effects of intensive physical and social stimulation on their ongoing behavior. Sixteen NPMR subjects were studied for 2 1/2 years. All were PMR, bedridden, abnormally small, and unresponsive to external stimulation. Baseline data concerning developmental status, diurnal patterns of sleep and activity, behavior in a novel situation, ongoing ward behavior, and anthropometric measures were obtained. Subjects were highly variable in their responses to items from the Bayley Scales of Infant Development, with overall performance below that of a normal six month old. Although subjects showed some diurnal pattern of sleep, they frequently fell asleep for short periods at random time during the day. In a novel situation subjects responded minimally to stimulus objects, or not at all. Typically, their ongoing ward behavior consisted of very low level activity, involving small peripheral limb movements, wandering or blinking eyes, mouthing or grimacing, and repetitive, reflexive types of patterns labeled "fixed action sequences." Experimental treatment consisted of three phases of daily stimulation. (1) Subjects were placed in an upright or elevated position in specially constructed "playpens." (2) Subjects were placed in "playpens" and many toys were presented. (3) Subjects were positioned in pairs so that direct physical contact was provided. Each phase resulted in changed behavioral profiles during treatment. Furthermore, all experimental subjects showed beneficial effects of treatment in their ongoing ward behavior, diurnal sleep patterns, Bayley performance, or response to novel situations. Implications of this research extend to understanding environmentally induced changes in neurological maturation and social development in individuals previously thought to be hopelessly devastated. The descriptive data are intended to provide a useful framework for further systematic research with NPMR populations.

Adolescent↗
PubMed1996

State-specific rates of mental retardation--United States, 1993.

Mental retardation (MR) is the most common developmental disability and ranks first among chronic conditions causing major activity limitations among persons in the United States (1). National and state-specific surveillance to measure the prevalence of MR can assist in targeting areas of need and allocating resources. State-specific prevalences for MR can be determined by using data about persons who receive specialized services for MR through entitlement programs. To estimate state-specific prevalences of MR in 1993, data were analyzed from the U.S. Department of Education (DOE) for children with MR who were enrolled in special education programs and from the Social Security Administration (SSA) for adults with MR. This report summarizes the findings, which suggest substantial state-specific variation in the prevalence of MR in the United States.

Adolescent↗
PubMed1986

Definition and classification of mental retardation: a reply to Zigler, Balla, and Hodapp.

Zigler, Balla, and Hodapp (1984) have proposed that mental retardation be defined as an IQ at least 2 standard deviations below the mean and classified as having organic or genetic etiology. These proposals are found to be based upon questionable assumptions about mental retardation. An alternative conceptual framework for definition of mental retardation is proposed and used to evaluate the merits of IQ and social competence in determining mental retardation. The use of IQ alone is judged to be inappropriate and potentially harmful when applied to individuals and when used to formulate public policy. Other researchers have established that there is insufficient evidence to attribute genetic or organic etiology to most mental retardation.

Achievement↗
PubMed2005

Neuropsychological profiles of persons with mental retardation and dementia.

This study examined the use of neuropsychological tests to assist in the differential diagnosis of dementia among persons with mental retardation. The author compared performances of persons with mental retardation and dementia (n = 10) to persons with mental retardation without dementia (n = 12). Participants were matched by IQ (mild or moderate mental retardation), age, presence of Down syndrome, and gender. In addition, all participants in the dementia group had corroborative medical tests (i.e., imaging, EEG, or high tau low AB42 protein testing) consistent with diagnosis of dementia. Test performance was compared on measures of attention and executive functions, language, memory and learning, and a dementia screening. Results from MANOVAs and nonparametric tests revealed significantly lower performance for persons with mental retardation and dementia in all areas assessed. Cut-off scores were also developed for the sample in order to maximize sensitivity and specificity for the test battery. Despite the small sample size, these findings suggest that there are significant measurable differences in several neurocognitive domains between the two groups.

Adult↗
PubMed2002

Enhancing independent time-management skills of individuals with mental retardation using a Palmtop personal computer.

Achieving greater independence for individuals with mental retardation depends upon the acquisition of several key skills, including time-management and scheduling skills. The ability to perform tasks according to a schedule is essential to domains like independent living and employment. The use of a portable schedule prompting system to increase independence and self-regulation in time-management for individuals with mental retardation was examined. Twelve people with mental retardation participated in a comparison of their use of the technology system to perform tasks on a schedule with use of a written schedule. Results demonstrated the utility of a Palmtop computer with schedule prompting software to increase independence in the performance of vocational and daily living tasks by individuals with mental retardation.

Adolescent↗
PubMed1984

Incidence of oculo-visual anomalies in an adult population of mentally retarded persons.

Clinical examination of 298 mentally retarded adults revealed a higher frequency of ocular anomalies of all types than is typical of a group of nonretarded subjects of the same age. It also became apparent that fewer of the mentally retarded had had appropriate optometric care than an equivalent nonretarded sample. The evidence indicates that correcting their ocular problems helps the retarded, yet these subjects receive less than average care.

Adult↗
PubMed1997

A study of cognitive development and behavior problems in mentally retarded children.

Cognitive development in seventy-one mentally retarded children (19 autistic, 52 non-autistic; aged 7-19 years) from a school for handicapped children was studied, using Ohta's scale for evaluating cognitive development level based on language comprehension (Ohta's stage), and other developmental scales. Behavior problems were also examined. The present study reports on the utility of Ohta's stage in non-autistic children, and the relationship between cognitive development level and behavior problems in mentally retarded children. In non-autistic children, there were temporal correlations between Ohta's stage and other development scales (a standard developmental test, speech development, symbolic play development, imitation development), suggesting that in non-autistic children as well, Ohta's stage may serve well as a scale for cognitive development, and reflect symbolic representational functioning. In non-autistic children, most behavior problems in feeding, elimination and sleeping, hyperkinesis, hypokinesis, stereotyped behaviors, self-injurious behavior and licking were closely associated with cognitive development level, and were more often noted in children of lower cognitive development level rather than only in the severely mentally retarded children. Some behavior problems may often occur in the sensorimotor period and hardly occur in the symbolic representational period.

Adolescent↗
PubMed1994

Mental retardation and adult women's perceptions of adolescent sexual abuse.

This study examined the effect of mental retardation and an adolescent girl's behavior on adult women's perceptions of sexual abuse and the girl's responsibility. Subjects were 288 women, age 18 to 33, who were randomly assigned a vignette describing a sexual encounter between an adolescent girl and boy. Girl's diagnosis (mentally retarded or nonretarded), boy's diagnosis (mentally retarded or nonretarded) and girl's behavior (encouraging, passive, or resisting) were experimentally manipulated. Factor analysis of responses yielded three factors: girl's responsibility, boy's abusiveness, and parents' responsibility. Results indicate that subjects perceive the girl's responsibility differently among girls with and without mental retardation. Regardless of her behavior, subjects perceive the girl as bearing little responsibility when she is retarded. However, when she is nonretarded, she bears more responsibility when she is encouraging than when she is passive or resisting, and she bears greater responsibility when she is passive than when she is resisting. Also, when the girl is encouraging, the boy's perceived sexual abusiveness is less when he is mentally retarded than when he is nonretarded. Finally, parents are assigned greatest responsibility when the girl is passive, regardless of her diagnosis.

Adolescent↗
PubMed1997

[Organization of epilepsy care for people with mental retardation. A situational analysis after the care reform].

The special care services for the mentally retarded in Norway were closed down by law on 1 January 1991. This implied the termination of centralized care, and integration of these individuals into the local communities. To map the epilepsy service facilities for mentally retarded patients after the care reform, questionnaires were sent to all officially responsible community physicians and to all Departments of Neurology at Norwegian hospitals. 20% of the community physicians expressed the view that mentally retarded patients did not receive adequate specialist service for their epilepsy. There were wide variations within the country. 37% of the responders stated that the community care was inadequately equipped to attend to the special needs of these patients. Firstly, there seems to be a lack of professionals able to transfer their competence concerning epilepsy. Many of the neurological departments felt that the number of referrals of patients with epilepsy and mental retardation had increased since the care reform. 50% of the responders had no opinion about whether the quality of the epilepsy service was not the same for the mentally retarded as for other patients. Many did not realize the need for special measures to provide adequate medical follow-up of this group. It is concluded that education and guidance to personnel and carers should receive high priority in the further development of a comprehensive epilepsy service. The development of multiprofessional epilepsy units at all university hospitals should be facilitated, and the appointment of specially assigned epilepsy nurses at all neurological departments is recommended.

Community Mental Health Services↗
PubMed2003

Identification of a 650 kb duplication at the X chromosome breakpoint in a patient with 46,X,t(X;8)(q28;q12) and non-syndromic mental retardation.

A female patient with non-syndromic mental retardation was shown by high resolution GTL banding to have inherited an apparently balanced translocation, 46,X,t(X;8)(q28;q12)mat. Replication studies in the mother and daughter showed a skewed X inactivation pattern in lymphocytes, with the normal X chromosome preferentially inactivated. The mother also had significant intellectual disability. To investigate the possibility that a novel candidate gene for XLMR was disrupted at the X chromosome translocation breakpoint, we mapped the breakpoint using fluorescence in situ hybridisation (FISH). This showed that the four known genes involved in non-syndromic mental retardation in Xq28, FMR2, SLC6A8, MECP2, and GDI1, were not involved in the translocation. Intriguingly, we found that the X chromosome breakpoint in the daughter could not be defined by a single breakpoint spanning genomic clone and further analysis showed a 650 kb submicroscopic duplication between DXS7067 and DXS7060 on either side of the X chromosome translocation breakpoint. This duplicated region contains 11 characterised genes, of which nine are expressed in brain. Duplication of one or several of the genes within the 650 kb interval is likely to be responsible for the mental retardation phenotype seen in our patient. Xq28 appears to be an unstable region of the human genome and genomic rearrangements are recognised as major causes of two single gene defects, haemophilia A and incontinentia pigmenti, which map within Xq28. This patient therefore provides further evidence for the instability of this genomic region.

Child↗
PubMed2000

Molecular cloning of Xp11 breakpoints in two unrelated mentally retarded females with X;autosome translocations.

Mental retardation is a very common and extremely heterogeneous disorder that affects about 3% of the human population. Its molecular basis is largely unknown, but many loci have been mapped to the X chromosome. We report on two mentally retarded females with X;autosome translocations and breakpoints in Xp11, viz., t(X;17)(p11;p13) and t(X;20)(p11;q13). (Fiber-) FISH analysis assigned the breakpoints to different subbands, Xp11.4 and Xp11.23, separated by approximately 8 Mb. High-resolution mapping of the X- chromosome breakpoints using Southern blot hybridization resulted in the isolation of breakpoint-spanning genomic subclones of 3 kb and 0. 5 kb. The Xp11.4 breakpoint is contained within a single copy sequence, whereas the Xp11.23 breakpoint sequence resembles an L1 repetitive element. Several expressed sequences map close to the breakpoints, but none was found to be inactivated. Therefore, mechanisms other than disruption of X-chromosome genes likely cause the phenotypes.

Adolescent↗
PubMed1989

Child life services and persons with mental retardation.

We admitted an adult with mental retardation and an eating disorder to a pediatric inpatient unit. Child life services were a core feature in the assessment and treatment of the patient. The role of child life services in the care of persons with mental retardation should be expanded.

Adult↗
PubMed1998

[Clinical and molecular screening for fragile X syndrome in 300 patients with non-specific mental retardation].

BACKGROUND: Fragile X syndrome is the most frequent cause of mental retardation linked to the X chromosome. In the majority of cases, the mutation responsible for the syndrome is an expansion of the trinucleotide repeat (CGG)n, present in the 5' region of exon 1 of the gene for mental retardation associated with fragile X syndrome (FMR-1). AIM: To report the results of a fragile X screening in patients with mental retardation. PATIENTS AND METHODS: Fragile X screening using polymerase chain reaction methods was done in 386 X chromosomes from 300 patients (214 male), aged 4 to 26 years old. The modified Hagerman test was applied to male patients. Hybridization techniques were applied in a subgroup of 51 patients. RESULTS: (CGG)n 30 was the allele found with the highest frequency in 50.2% of patients. (CGG)n 29 was found in 29% of patients. One subject had an allele with 46 CGG repeats, which corresponds to the gray zone. Hybridization studies were highly concordant with PCR, detecting four males with fragile X syndrome and a carrier female. The average clinical score of mental retardation not due to fragile X syndrome was 10.3 +/- 3.4 (range 3 to 23), and 97% of males had a score below 19. The concordance between scores over 20 and molecular genotype was 98%. CONCLUSIONS: The distribution of (CGG)n repeats, observed in this study, was significantly different to that previously reported for a normal Chilean population. The dispersion of molecular status and clinical score was lower than previously described using cytogenetic techniques.

Adolescent↗
PubMed1995

Upper gastrointestinal tract bleeding in institutionalized mentally retarded adults. Primary role of esophagitis.

BACKGROUND: Upper gastrointestinal (UGI) tract bleeding is a common reason for hospitalization of mentally retarded adults. The purpose of this study was to determine the frequency and cause of and risk factors for UGI tract bleeding in institutionalized mentally retarded adults admitted to our hospital. METHODS: Case-control study, with a retrospective review of medical records. SETTING: Inner-city tertiary care hospital. PATIENTS: Mentally retarded adults who reside in one of three long-term care facilities. MEASUREMENTS: Data on demographic features, clinical features, and concurrent medical conditions were collected on mentally retarded adults admitted to our hospital for UGI tract bleeding (n = 40). Data from these patients were compared with those from a control group of mentally retarded adults admitted for nonbleeding conditions (n = 124). Endoscopic data were reviewed on the patients who bled to further clarify the causes of UGI tract bleeding. The patients who bled were compared with those who did not bleed using the chi 2 and Mann-Whitney rank sum tests. RESULTS: Upper gastrointestinal tract bleeding was the most common reason for hospitalization in this patient population. Such bleeding was most commonly caused by erosive esophagitis (70% of patients who bled). Conditions associated with an increased risk for UGI tract bleeding were hiatal hernia, reduced activity, incontinence, hypoalbuminemia, kyphoscoliosis, and spastic quadriplegia. CONCLUSIONS: Upper gastrointestinal tract bleeding is a common cause of hospitalization in mentally retarded adults and is usually due to erosive esophagitis. It is hoped that vigorous efforts to treat gastroesophageal reflux disease may reduce hospitalization in these patients.

Adult↗
PubMed1991

Identification of two novel cerebrospinal fluid proteins in non specific mental retardation.

Cerebrospinal fluid (CSF) from twenty three patients with non specific mental retardation and fourteen age matched normal samples was subjected for qualitative analysis of protein profiles by two-dimensional gel electrophoresis (2-DE) and the proteins were visualised by ultra sensitive silver staining. Two proteins designated as mental retardation associated proteins (MRAP-I and MRAP-II) were identified in six male patients out of twenty three patients CSF samples. MRAP-I had an isoelectric point of 7.4 with a relative molecular weight 16.5 kDa, while MRAP-II had an iso-electric point of 7.2 with a relative molecular weight 16.8 kDa. The two proteins are presumed to be originated from brain, as they could not be traced in the serum of patients, nor due to proteolytic degradation. Despite unknown origin and identity, their presence in the CSF of a specific group of mentally retarded male patients suggest their possible clinical utility and to define protein alterations in mental retardation.

Adolescent↗
PubMed1997

Comprehensive management of epilepsy in persons with mental retardation.

Epilepsy is a common occurrence in persons with mental retardation. The application of recent advances in epilepsy research to patients with mental retardation has shown that well-accepted principles of management are as relevant for multiply handicapped people as for those with epilepsy alone. Quality enhancement is emphasized as the overarching concept that determines the quality of care provided to patients with epilepsy and mental retardation. This article reviews the comprehensive management of epilepsy, discusses what is known at present, and indicates what is not known and needs further research.

Anticonvulsants↗
PubMedDate not supplied

Dendritic and synaptic pathology in mental retardation.

Histologic changes in brains from mentally retarded patients are often subtle and may be limited to abnormalities in the dendritic and synaptic organization of the cerebral cortex. Special methods may be necessary for their demonstration. This review summarizes data on dendritic and synaptic pathology in human mental retardation syndromes. Findings in humans are compared to those in animal models that are of relevance to human disorders. Both in humans and in animal models there is evidence that development modifies the observed changes and that findings differ depending on the developmental age at which the tissue is studied.

Brain↗
PubMed1998

Psychiatric illness in mentally retarded adolescents: clinical features.

Attempts have been made to establish objective diagnostic criteria for psychiatric disorders in persons with mental retardation. This paper describes the clinical features of the most important psychiatric disorders in mentally retarded adolescents: mood disorders, psychotic disorders, severe behavioral disorders, personality disorders, anxiety disorders, and attention deficit disorder with hyperactivity. The impact of mental retardation on personality development is confirmed by the high psychopathological vulnerability of the mentally retarded. All types of mental disorders can be observed, with an incidence estimated to be at least three or four times higher than in the general population. Adolescence is a particularly important phase for the mentally retarded, since adolescent turmoil can increase the risk of psychopathology.

Adolescent↗