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The self-management of skills by persons with mental retardation.

The transition of persons with mental retardation to less restrictive environments is often hindered by difficulties in managing their own behavior in the absence of external controls. This observation has led to an upsurge of interest in the advantages of teaching self-management skills to persons with mental retardation. This article reviews evidence about the effects of self-management training on the acquisition, maintenance, and generalization of skills. The analyses show that self-management training has been useful in promoting the maintenance of behavior change first effected by external control procedures, but that a dearth of evidence and a number of methodological problems preclude convincing conclusions about its value in promoting generalization. The empirical evidence also suggests that the effectiveness of these procedures may depend upon the cognitive and linguistic abilities of the persons receiving self-management training. Finally, the design of much of the empirical research does not enable the disentanglement of the specific effects of self-management training from those arising from the concurrent application of external control procedures. Carefully controlled componential studies of the effects of self-management training and external control procedures are sorely needed.

Activities of Daily Living↗

HIV infection in persons with prior mental retardation.

This paper presents five case studies of persons who were mentally retarded prior to infection with human immunodeficiency virus (HIV). Particular emphasis is placed on neuropsychological functioning, and its interaction with management and treatment. The effects of HIV on neurological functioning in such persons has not been previously documented. Although it may be hypothesized that mentally retarded people are at increased risk for developing neurological complications, these preliminary data suggest this is not so and that the development of HIV-related encephalopathy is no different in the mentally retarded, manifesting in the AIDS-related complex (ARC) and AIDS stages of illness. Within the context of HIV infection, the cognitive status of the mentally retarded creates unique treatment and management difficulties, and some guidelines are presented.

AIDS Dementia Complex↗

Training social skills to severely mentally retarded multiply handicapped adolescents.

Three severely mentally retarded, multiply handicapped, adolescents were treated in a classroom setting for social skills deficits. Two of these children exhibited symptoms of autism including periods of echolalia, and fascination with tactile and visual stimulation. One of the pair was deaf. The third child was profoundly mentally retarded and had minimal expressive language skills. All had received sign language training to facilitate communication. Treatment focused on increasing the frequency of eye contact, in seat and response to verbal prompt behaviors, skills deemed necessary to facilitate use of sign language communication and to increase social interaction. Baseline and treatment were evaluated in a multiple baseline, alternating treatment design across children. Baseline was taken on responses to 10 standard questions, asked by the teacher, based on verbal presentation and sign language. This same procedure was then continued during the initial treatment phase following training sessions. During training, the children received social reinforcement, performance feedback and edible reinforcement, in the form of candy, for appropriate performance. Physical and verbal prompts as well as pictorial cues were employed to shape appropriate behavior. In the second treatment phase, training was implemented in the classroom in which baseline data had been collected. Improvement in target behaviors, via training sessions held four days a week, was noted. These data suggest that use of a combination of visual stimuli, operant and social learning methods can remediate social skills deficits in children with multiple psychological and physical deficits. The implications of these findings for current and future research are discussed.

Achievement↗

Surgical management of gastroesophageal reflux in severely mentally retarded children.

Thirty-three severely mentally retarded children with profound malnutrition and aspiration pneumonitis were treated by gastrostomy and Nissen fundoplication. Early and late complications were 27.3% and 16.6% respectively. Mortality was 9.4%. All surviving patients gained weight. Time required for feeding was greatly reduced. This study supports earlier operative intervention for the mentally retarded child with gastroesophageal reflux, malnutrition and aspiration pneumonitis.

Body Weight↗

Mental retardation in a North Swedish isolate.

Twenty-six mentally retarded patients from a North Swedish geographically isolated population were studied. The mentally retarded patients were short with pycnic body type. Neurological and psychiatric stigmata of variating functions and degrees were recorded. Neurogenic hearing defects, impaired vision, pathological EEG, widened subarachnoidal space found in CT, were among these stigmata. In three patients a urinary substance, probably urocanyl-glycine, was found. The erythrocyte uroporphyrinogen-I-synthetase activity was high in four patients. Chromosome analysis showed some abnormalities which, however, were not systematically present. In the near family 23 persons with schizophrenia were found. The relationship between the mental retardation and the schizophrenic psychosis in the present families has not been explained. Further studies are called for to elucidate the genetic and environmental factors responsible for the two conditions.

Female↗

Minor psychiatric disorders as possible complication of mental retardation.

In young adult life a large proportion of mentally retarded subjects develop psychiatric complications, both severe and mild, whose origin is undoubtedly multifactorial (biological and psychological). A series of 27 subjects (13 male, 14 female; mean age 27.6 years) with IQ ranging from 70 to 40 were studied in our Institute from 1994 to 1995 and followed up 1 and 2 years later. Paykel scale (life events), FACES III (type of family insight), MFFT and Watkin's test (cognitive styles) were used to evaluate all cases. We found that (1) minor disorders prevailed in mild and moderate mental retardation and severe disorders in profound mental retardation; (2) minor disorders were generally determined by psychological and environmental factors although biological ones may have an important role; (3) psychological factors prevailed in young adult life when the subject had to cope with relatively harder tasks, and (4) the prognosis is generally good if proper care is given.

Adult↗

A case of 46,XY,t(1;13) (q24;q32) with mental retardation.

A 14-year-old mentally retarded boy with congenital malformations of unknown etiology was found to have a de novo apparently balanced reciprocal translocation between chromosomal arms 1q and 13q. There is only one other case where a similar translocation was observed but the breakpoints could be localized only by regions and the individual was not mentally retarded.

Abnormalities, Multiple↗

Chromosomal aberrations in mildly mentally retarded children in a northern Swedish county.

Few studies of the frequency of chromosomal aberrations in an unselected material of mildly mentally retarded children have been published. The present paper summarizes the chromosomal abnormalities in children with mild mental retardation (MMR) in Västerbotten in the northernmost part of Sweden. Chromosome analyses were carried out by routine methods. In addition, children whose MMR had no clear diagnosis or cause were investigated as regarding X-chromosomes with a fragile site and selected cases with banding techniques. Every mentally retarded child born between 1959 and 1970 in the county of Västerbotten was traced. Out of a total number of 40,871 individuals, 171, i.e. 4.2 per 1,000, were found to be mildly mentally retarded. Chromosomal aberrations were seen in 11.9% of the children with MMR, compared with 39.1% of the 161 children with severe mental retardation (SMR) in the same population. The proportion of cases with mental retardation of unknown etiology is high, especially amongst those with MMR. The use of high resolution banding and other modern cytogenetic methods should reduce this figure.

Chromosome Aberrations↗

Effects of maternal mental retardation and poverty on intellectual, academic, and behavioral status of school-age children.

The impact of low maternal IQ and poverty was examined through comparison of 27 school-age children of mothers with mild mental retardation to 25 similarly impoverished children of mothers without mental retardation. The children whose mothers had mental retardation had lower IQs and academic achievement and more behavior problems. Not one child with a mother who had mental retardation was problem-free. Boys were affected more severely than were girls. Quality of the home environment and maternal social supports were lower in the group with maternal mental retardation; both measures were negatively correlated with child behavior disorders. Results suggest that being raised by a mother with mental retardation can have detrimental effects on child development that cannot be attributed to poverty alone.

Achievement↗

AGG interspersion analysis of the FMR1 CGG repeats in mental retardation of unspecific cause.

OBJECTIVES: To study the AGG interspersion pattern in mentally retarded patients of unspecified cause. METHODS: FMR1 CGG substructure in 104 normal and 232 mentally retarded (MR) males was determined by CGG repeat and AGG interspersion analyses. Genomic DNA of the study subjects was obtained for PCR and Southern hybridization analyses. RESULTS: All study subjects had less than 53 CGG repeats and none had fragile X syndrome of mental retardation. There was a significant difference (P < 0.006) in the AGG interspersion pattern. MR males had (1) more variable internal substructures, (2) proportionally less 2 and 3 AGG but more 0 and 1 AGG, less (CGG)(9)AGG(CGG)(9)AGG(CGG)(9) but more (CGG)(9)AGG(CGG)(19) alleles and (3) a longer pure 3' CGG repeat. CONCLUSIONS: Our results suggest that the MR alleles have a lesser number of interspersed AGG and a longer pure 3' CGG repeat than the normal population. They are thus more prone to instability and expansion to long repeat lengths as in the fragile X syndrome of mental retardation.

Alleles↗

Scatter variability in the WAIS-R profiles of mentally retarded adults.

One hundred and sixty mentally retarded adults were administered the WAIS-R, and the scatter variability of their test profiles was examined. Compared to the WAIS data of Coolidge et al. (1986), the WAIS-R was less variable in its intratest and intertest scatter. The data support Wechsler's original statements about the characteristics of intelligence test profiles among mentally retarded adults.

Adolescent↗

Relapse of aggressive and disruptive behavior in mentally retarded adults following antipsychotic drug withdrawal predicts psychotropic drug use a decade later.

BACKGROUND: Mental retardation is frequently associated with aggression toward self and others. Antipsychotic medications are frequently used as a major treatment of such aggression. However, national and state policies and guidelines are weighted toward stopping or decreasing the doses of these medications whenever possible, although exceptions are permitted. The purpose of this study was to determine if relapse during or after antipsychotic drug withdrawal in mentally retarded adults predicts continuing antipsychotic drug use an average of a decade later. METHOD: We report here on a 6- to 13-year (average 10-year) follow-up of 151 institutionalized mentally retarded adults. During the period 1990-1997, the subjects had been prescribed antipsychotic medications to treat maladaptive behaviors, primarily consisting of aggression, disruptive/destructive behaviors, or a combination of these. We compared subjects' psychotropic medication profiles in 2003 as they related to outcome during the earlier period. Our goal was to determine if rapid relapse (a clinically significant increase in maladaptive target symptoms, beginning 3 months or less after antipsychotic drug termination or dosage reduction, that was reversed by antipsychotic drug reinstitution or dosage increases) during or after routine withdrawal of an antipsychotic predicted psychotropic drug use in 2003. RESULTS: For those individuals successfully withdrawn from antipsychotic medications, 66.3% (55/83) were still psychotropic drug free in 2003. For those who rapidly relapsed during the period 1990-1997 following antipsychotic drug withdrawal or dosage decreases, only 9.0% (5/55) were psychotropic medication free in 2003. CONCLUSION: These observations support policies and guidelines indicating that attempts to stop treatment with antipsychotic medications in mentally retarded individuals are worthwhile. However, the results also indicate that eventual discontinuation of antipsychotic medications in institutionalized mentally retarded adults who have previously relapsed upon such withdrawal is unlikely to be successful. Rigid adherence to drug withdrawal policies and guidelines in such individuals should be reconsidered.

Adult↗

Contraceptive management for female adolescents with mental retardation and handicapping disabilities.

Adolescent girls with mental retardation and handicapping disabilities have a need for sexual counseling and contraceptive management. Societal attitudes have changed, allowing these patients to experience normal and satisfying sexuality. The reproductive health concerns of these patients are extensive. Contraceptive selection varies with regard to the mental and physical capabilities of individual patients. The use of barrier methods, intrauterine devices, oral contraceptives, intramuscular medroxyprogesterone acetate, and sterilization has been discussed thoroughly in the past. No formal studies have been performed to evaluate the use of subdermal levonorgestrel implants in patients with mental retardation and physical disabilities.

Adolescent↗

Assessing the acceptability of behavioral treatments to persons with mental retardation.

Individuals with mild to moderate mental retardation living in community residential settings (Experiment 1) or an institutional setting (Experiment 2) rated the acceptability of differential reinforcement and time-out applied to a mild and a severe behavior problem. Using analogue methodology similar to previous treatment acceptability studies (e.g., Miltenberger, Lennox, & Erfanian, 1989; Tarnowski, Rasnake, Mulicke, & Kelly, 1989), and a simplified rating scale developed for persons with mental retardation, community-based subjects rated differential reinforcement as more acceptable than time-out for the mild problem, whereas institutionalized subjects rated time-out as more acceptable than differential reinforcement when applied to the severe problem. These results were discussed and future research suggested.

Activities of Daily Living↗

Visual preferences of students with profound mental retardation and healthy, full-term infants.

Thirty students with profound mental retardation (age range: 3-5 to 19-11) and 30 healthy, full-term infants (5-8 months) were shown 12 stimuli, three times each. Four patterned stimuli were presented one to a card and each pattern appeared in black-and-white, black-and-yellow, and red-and-yellow. Both groups looked significantly longer at face patterns than other patterns. Students with profound mental retardation looked longer at black-and-white patterns than other color combinations. Infants looked longer at red and yellow cards than did students with profound mental retardation. The measurement method was practical, reliable, and sensitive to both within and between group differences. Results from this assessment method may help determine the most salient visual stimuli for evoking active-alert states for students with profound mental retardation. Individual variability was evident in the data, which demonstrates the importance of examining preferences for each individual when planning intervention. Implications for future research and intervention are discussed.

Child↗

Urinary levels of taurine in mentally retarded children.

Urinary taurine levels were estimated in 29 mentally retarded children. These levels were then compared with those of normal healthy children. The urinary taurine levels were significantly higher in the mentally retarded subjects. There is probably an intriguing relationship between taurine levels and mental retardation.

Adolescent↗

Dendritic pathology in mental retardation: from molecular genetics to neurobiology.

Mental retardation (MR) is a developmental brain disorder characterized by impaired cognitive performance and adaptive skills that affects 1-2% of the population. During the last decade, a large number of genes have been cloned that cause MR upon mutation in humans. The causal role of these genes provides an excellent starting point to investigate the cellular, neurobiological and behavioral alterations and mechanisms responsible for the cognitive impairment in mentally retarded persons. However, studies on Down syndrome (DS) reveal that overexpression of a cluster of genes and various forms of MR that are caused by single-gene mutations, such as fragile X (FraX), Rett, Coffin-Lowry, Rubinstein-Taybi syndrome and non-syndromic forms of MR, causes similar phenotypes. In spite of the many differences in the manifestation of these forms of MR, evidence converges on the proposal that MR is primarily due to deficiencies in neuronal network connectivity in the major cognitive centers in the brain, which secondarily results in impaired information processing. Although MR has been largely regarded as a brain disorder that cannot be cured, our increased understanding of the abnormalities and mechanisms underlying MR may provide an avenue for the development of therapies for MR. In this review, we discuss the neurobiology underlying MR, with a focus on FraX and DS.

Animals↗

Tourette syndrome in severely behavior disordered mentally retarded children.

Although generally overlooked in mentally retarded individuals, the occurrence of Tourette Syndrome (TS) in seven such children is documented here. These subjects also shared a very early and persistent severe behavior disorder consisting of hyperactivity, agitation, aggressivity, and noncompliance. Correct diagnosis of TS is important to appropriate treatment, rather than assuming that motor and phonic tics and other associated TS symptoms are necessarily a function of a more pervasive developmental disorder in a disturbed mentally retarded person.

Child↗