Capacity of persons with mental retardation to consent to participate in randomized clinical trials

OBJECTIVE: Adults with mental retardation have histories of cognitive and adaptive deficits posing unique ethical challenges for research consent assessment. This study examined the capacity of persons with mental retardation to consent to participate in randomized clinical trials. METHOD: A total of 150 adults (50 each with mild and moderate mental retardation and 50 comparison subjects without mental retardation) responded to a set of consent questions for a hypothetical randomized clinical trial testing a medication for aggressive disorders. Intelligence, adaptive behavior, medical treatment history, and consent history were evaluated. Univariate and multivariate methods were used to compare performance across and within groups. RESULTS: Comparison subjects scored significantly higher on measures of consent capacity than participants with mild mental retardation, who scored higher than those with moderate mental retardation. Most subjects with mental retardation were able to make a participation choice, and many understood research methods and appreciated the protagonist's disorder and the consequences of participation. Almost half of those with mild mental retardation understood human subject protections. Performance was weakest on understanding the purpose of research and reasoning about whether to participate, suggesting vulnerability to the therapeutic misconception. Psychiatric and experiential factors did not predict consent capacity. CONCLUSIONS: While adults with mental retardation as a group showed consent deficits, many attained consent capacity scores comparable to those of comparison subjects. Investigators should consider individual differences and a consent format suited to deficits in language, memory, and attention before restricting consent opportunities for persons with mental retardation.     

Prevalence of physical inactivity and recommended physical activity in community-based adults with mental retardation

Little information has been reported on the leisure time physical activity (LTPA) habits of adults with mental retardation. Prevalence of physical inactivity and recommended LTPA of adults with mild to moderate mental retardation who live in community settings was described. Adults with mental retardation (76 men, 74 women) reported their physical activity habits. Overall, men and women who resided in community settings were similarly inactive, with 47% to 51% of individuals participating in little to no LTPA. Forty-two to 47% of them reported participation in moderate to vigorous LTPA five or more times per week. Limitations to quantifying physical activity through questionnaire process is discussed and development and implementation of programs designed to increase physical activity levels recommended.     

Simulated and community-based instruction involving persons with mild and moderate mental retardation

In this study of simulated instruction and/or community training of four functional living tasks was compared across groups of adolescents and young adults with mild and moderate retardation. Forty individuals participated in the assessment and training activities of this study, including 20 participants with mild retardation and 20 individuals with moderate retardation. A mixed factorial design was used to evaluate differences associated with level of mental retardation, simulated versus community-based instruction, and assessments in school and community settings. Participants with mild retardation performed better than their counterparts with moderate retardation on the simulated tasks and in the community settings. Participants with mild retardation were more successful in generalizing from the simulated instructional experiences to the community settings than were their counterparts with moderate retardation. However, in many of the situations, community training was sufficiently powerful to eliminate any advantage associated with some of the participants having experienced prior simulated instruction. Results are discussed in relation to the need to more closely examine the design and delivery of functional living skills instruction involving individuals with different levels of mental retardation. Specifically, the effectiveness of instructional simulations for teaching functional living skills does not appear to be uniform across level of retardation and targeted tasks. The highest priority question no longer appears to be whether or not simulated versus community-based instruction is more efficacious. Future research might more productively focus on the quality of different instructional simulations in combination with community assessment and/or training opportunities for teaching functional living skills to persons who experience different levels of mental retardation.     

Moderate and mild mental retardation in the Martin-Bell syndrome

ABSTRACT. A survey of children attending schools for the moderately or the mildly mentally handicapped has shown that two out of 25 boys and two out of 22 girls with idiopathic moderate mental retardation had the Martin-Bell syndrome, while none of 75 boys and one out of 51 girls with mild mental retardation were FRAXA positive. Consideration of these figures along with published studies suggests that 7% of moderate and 3–8% of mild idiopathic mental retardation in boys, and 2–5% of moderate and 3–3% of mild idiopathic mental retardation in girls may be due to the Martin-Bell syndrome.     

结节性硬化症神经系统损害的临床特点

目的探讨结节性硬化症(TSC)神经系统损害的临床特点。方法回顾性分析35例TSC患儿的临床资料。结果本组患者中,智力低下23例,其中轻度智力障碍13例(37.1%),中-重度智力障碍10例(28.6%);癫痫发作33例(94.3%);孤独症样表现10例(28.6%)。脑电图检查均异常,主要表现为慢波增多、癫痫样放电,多为全导出现,两侧波幅明显不对称。CT检查均发现大小不等的钙化灶或高密度影;MRI主要表现为脑内多个大小不等的片状及结节状短T1、T2异常信号;Flair呈高信号。经抗癫痫治疗,癫痫发作控制18例。癫痫发作没有控制的患儿中-重度智力障碍率(46.7%)及合并孤独症表现的比率(44.4%)显著高于癫痫发作控制良好的患儿(16.7%,11.7%)(均P<0.01)。随访结束时,智商与随访前差异无统计学意义;5例患儿的脑电图恢复正常,14例好转。结论 TSC的神经系统损害以癫痫发作和智力障碍为主,部分伴孤独症行为。经治疗和年龄增长部分患儿的癫痫可得到控制,但智力无明显改善。     

Association of adenosine deaminase polymorphism with mild mental retardation

The etiology of mild mental retardation remains undefined in about 60% of cases. Even though the causes of mild mental retardation are likely to be heterogeneous, the evidence for genetic involvement is increasing, along with the development of specific diagnostic techniques. To improve our understanding of the genetic basis of mild mental retardation, we explored the role of polymorphisms of adenosine deaminase, an enzyme that is supposed to act as a neuroregulatory protein. To this end, we conducted an association study comparing children with mild mental retardation of unknown origin with two groups of controls: (1) apparently healthy children and (2) children with moderate or severe mental retardation of known etiology. Overall, 338 participants were enrolled in the study. Cases (ie, 80 children) were more likely than controls (ie, 153 healthy children and 105 children with moderate or severe mental retardation) to have the low-activity ADA-Asn 8 (ADA(1) *2) polymorphism (P < .05) and to present the ADA(1) *2/ ADA(2) *1 haplotype. No significant differences were found with respect to adenosine deaminase polymorphisms when comparing the group with moderate or severe mental retardation of known causes and healthy controls. In conclusion, our findings suggest a possible role for a low-activity genotype (ADA-8Asn) (ADA(1) *2) of adenosine deaminase in the pathogenesis of mild mental retardation.     

Psychiatric morbidity with focus on obsessive–compulsive disorder in an Israeli cohort of adolescents with mild to moderate mental retardation

The study evaluated the prevalence of DSM-IV-TR-defined psychiatric disorders in adolescents with mental retardation, with a focus on obsessive-compulsive disorder (OCD), for which data at present are sparse. Eighty-seven adolescents with mild to moderate mental retardation attending the Israeli special-education system were screened for psychiatric disorders in general and obsessive-compulsive symptoms in particular. Sixty-one percent had at least one psychiatric disorder. Of the 13 participants receiving antipsychotic medication, none had an underlying psychotic disorder and most had anxiety or depressive disorders. OCD was detected in 11% of participants and was characterized by high rates of psychiatric comorbidities. The severity of autistic symptoms predicted 39% of the variance in the severity of OCD symptoms. Adolescents with mild to moderate mental retardation have high rates of psychiatric morbidities that are often inappropriately treated. OCD is prevalent in this population and is strongly associated with autistic symptoms. Further studies are required in adolescents with mental retardation to better delineate psychiatric morbidities and their appropriate treatment in this at-risk population.     

Recognition of facial expressions of emotion by persons with mental retardation. A matched comparison study.

Children and adults with mental retardation were tested on their ability to recognize facial expressions of emotion. The sample consisted of 80 children and adults with mental retardation and a control group of 80 nonhandicapped children matched on mental age and gender. Ekman and Friesen's normed photographs of the six basic emotions (anger, disgust, fear, happiness, sadness, and surprise) were used in a recognition task of facial expressions. Subjects were individually read two-sentence stories identifying a specific emotion, presented with a randomized array of the six photographs of the basic facial expressions of emotion, and then asked to select the photograph that depicted the emotion identified in the story. This procedure was repeated with 24 different stories, with each of the six basic emotions being represented four times. Results showed that, as a group, individuals with mental retardation were not as proficient as their mental-age-matched nonhandicapped control subjects at recognizing facial expressions of emotion. Although adults with mild mental retardation were more proficient at this task than those with moderate mental retardation, this finding was not true for children. There was a modest difference between the children with moderate mental retardation and their nonhandicapped matched controls in their ability to recognize facial expression of disgust.     

The understanding and use of interpersonal gestures by autistic and Down's syndrome children

Autistic adolescents with mild, moderate, and severe degrees of mental retardation, Down's syndrome adolescents, and clinically normal 4-, 5-, and 6-year-old children were compared in their ability to understand a set of simple instrumental gestures. Almost all gestures were perfectly understood, that is, correctly responded to, by normal children from age 5 onwards, and by all the handicapped groups, regardless of diagnosis or degree of retardation. However, the ability to initiate such gestures on verbal request was generally less good, especially in the less able autistic groups. The same subjects were unobtrusively observed in the playground and during mealtime at their schools. Peer interactions were least frequent in the autistic subjects, regardless of degree of mental retardation. However, relative to interaction frequency, the autistic group used nonverbal instrumental gestures as a means of communication to the same extent as the other groups. Unlike Down's syndrome adolescents, or normal preschool children, no autistic adolescent ever used expressive gestures.Institute of Education, MRC Developmental Psychology ProjectOur particular thanks are due to the Dedisham School for Autistic Children and to many special schools in Croydon and Herefordshire.     

Efficacy of a pre-retirement planning intervention for ageing individuals with mental retardation

ABSTRACT. This report describes a study designed to identify variables related to attitudes toward retirement among adults with mental retardation. Seventy-five adults with mild to moderate mental retardation were compared to a group of age peers drawn from the employee population of a regional centre in a Southern American state. It was found that a general satisfaction with life and feelings of preparedness for retirement were strongly related to attitudes toward retirement among both groups. Other common corelates that were significantly related among both groups included financial preparedness, health perceptions, orientation to work and commitment to work. Also observed was a high correlation between life satisfaction and orientation to work and commitment to work among both groups. The authors also found that pre-retirement planning did prove to be a generally effective means of teaching older adults with mental retardation the options that are available during their retirement years, but did not seem to change their attitudes toward retirement or life satisfaction. The auhors conclude that retirement has positive connotations for most individuals with mental retardation, particularly if they feel that they are prepared for it.     

Implicit memory in aging adults with mental retardation with and without Down syndrome

Effects of age and IQ on implicit memory in adults with mild or moderate mental retardation with and without Down syndrome were examined. When the etiologically defined groups were equated on age (and IQ), an age-associated difference in implicit memory performance was not evident. When data were reanalyzed, including only participants with unspecified mental retardation from a broader age range, we found a significant but small age-related difference and a significant but small IQ-related effect on implicit memory. In summary, although implicit memory showed an age-associated difference and IQ-associated variation in adults with mental retardation, these effects were relatively small. Data support existing theories proposing the relative invariance of implicit processes across a range of individual differences in age and intelligence.     

Topiramate in children and adolescents with epilepsy and mental retardation: a prospective study on behavior and cognitive effects

The aim of the present study was to assess the behavioral and cognitive effects following treatment with topiramate in children and adolescents with epilepsy with mild to profound mental retardation. The study group comprised 29 children, 16 males and 13 females, aged 3 to 19 years, affected by partial (4) and generalized (25) crypto/symptomatic epilepsy and mental retardation (7 mild, 5 moderate, 15 severe, 2 profound), who were administered topiramate (TPM) as add-on therapy to their baseline antiepileptic treatment. At baseline, 3 months, 6 months, and 12 months, parents or caregivers of each patient were administered a questionnaire based on the Holmfrid Quality of Life Inventory. After a 3-month follow-up, the add-on topiramate caused overall mild to moderate cognitive/behavioral worsening in about 70% of children and adolescents with mental retardation and epilepsy. After 6 and 12 months of follow-up, global worsening persisted in 31 and 20.1% of cases, respectively. In conclusion, this trial confirms that TPM can have significant adverse cognitive and behavioral side effects, even in mentally disabled children and adolescents.     

Caring for adults with mental retardation: survey of family practice residency program directors

In their core curriculum guidelines, the Society of Teachers of Family Medicine has recognized the importance of training family physicians in caring for persons with mental retardation. We mailed surveys to all family practice residency directors in the United States, questioning them about experiences and methods used to teach residents about health care needs of adults with mental retardation and the importance of this education. We found that 84% of programs provide residents with one or more experiences, and 60% instruct residents in this area. Most directors ranked this education as very important or important. There was no relationship between type or age of residency program and likelihood that residents were educated about mental retardation. The importance of this education is discussed.     

Occupational and leisure preferences of older adults with mental retardation

This article presents baseline information on the occupational and leisure preferences of a statewide sample of older adults with mild to moderate mental retardation who reside in the State of Oregon, USA. Data were collected through an interview with the older person with mental retardation and through a questionnaire sent to a knowledgeable other who was matched to each older adult. Findings are presented on the types of occupational and leisure activities that were participated in by these older adults, on the level of satisfaction experienced with these activities, and on their preferences for continued participation as they grow older. Results indicate an overwhelming desire for continued participation in occupational and leisure pursuits that are personally meaningful. Strong preferences are expressed for greater variety, frequency, and choice in these activities. Implications of the findings for designing retirement options for older adults with mental retardation are discussed.     

Normal ageing in adults with Down's syndrome: a longitudinal study

The ubiquitous presence of the neuropathoiogy of Alzheimer disease (AD) in individuals with Down's syndrome (DS) over 40 years of age suggests that this group of people will exhibit a high prevalence of dementia of the Alzheimer type (DAT) as they age. The present study indicates that there is a clear discrepancy between the presumed presence of AD neuropathoiogy and the clinical expression of DAT among older people with DS. In the first 6 years of a longitudinal study, the present authors compared 91 adults (31–63 years of age) with DS and mild or moderate mental retardation to 64 adults (3 l –76 years of age) with other forms of mental retardation (MR) on yearly measures of mental status, short-and long-term memory, speeded psychomotor function, and visuospatial organization. The results indicated that, over repeated testing on the verbal long–term memory test, younger participants with DS showed small increases in their scores, while older participants with DS showed very slight decreases. Overall performance scores on this test and a speeded psychomotor task were poorer for both diagnostic groups in individuals aged SO years and older. The magnitude and type of these selective changes in performance were consistent with performance profiles observed in older healthy adults without mental retardation on tests measuring similar cognitive functions. Only four out of the 91 people with DS in the present sample showed changes in funaioning that have led to a diagnosis of possible DAT. and in these individuals, alternative causes of performance declines were concurrently present (e.g, thyroid dysfunction). These findings indicate that some age–associated changes in funaioning are related to ‘normal’ but probably precocious ageing among adults with DS. Furthermore, these findings suggest that adults with DS and mild or moderate mental retardation may be at lower risk for dementia during their fourth and fifth decades of life than previous studies have suggested.     

A clinical study of cerebral palsy in Shiga; 1977-1986--II. Severity of the disability and complications in various types of cerebral palsy]

The severity of the disability and complications was evaluated at 6 years of age in 202 cases of cerebral palsy (CP) in Shiga Prefecture (69 with spastic diplegia, 62 with tetraplegia, 33 with hemiplegia, 23 with the dyskinetic type and 15 with the ataxic type) born between April 1977 and March 1987. The degree of gross motor disability differed among the clinical types, being mild in 45%, moderate in 17%, and severe in 39%. Gross motor disability was generally correlated with mental retardation, with some exceptions. Some non-ambulatory cases exhibited a normal or subnormal mentality, and most of such cases had been preterm infants with spastic diplegia. Most cases with mild gross motor disability and severe or moderate mental retardation had been term infants. Forty-eight percent suffered from epilepsy (25% in spastic diplegia, 86% in tetraplegia, 45% in hemiplegia, 39% in the dyskinetic type and 13% in the ataxic type). Microcephaly was noted in 35% (66% in tetraplegia and about 20% in other types).     

Auditory brain stem responses(ABR) in developmentally retarded infants and children

We recorded ABR in 65 infants and children with developmental retardation and analyzed the relationship between the incidence of abnormal findings and the degree of mental retardation, which was confirmed by follow-up observation and formal intelligence tests. There were 3 cases for whom no response could be obtained. In the 21 retardates with specific causes of retardation or complications, peripheral abnormalities were noted in 13 (62%) and central abnormalities in 11 (52%). The 41 cases of idiopathic retardates were divided into three categories according to the degree of mental retardation. Of 15 cases who were ascertained to be normal or borderline retardates, 3 (20%) showed some abnormal findings. Of 13 cases who were proven to be suffering from mild to moderate mental retardation, 5 (38%) showed abnormal findings. Of 13 cases who were confirmed to be suffering from severe to profound mental retardation, 5 (39%) showed central abnormalities. The high incidence of ABR abnormalities in retardates compared to in normal controls suggests that ABR measurement is a useful tool for evaluating infants who have possible developmental delay. It seems that disturbance of acoustic information processing can disturb language acquisition, and such a consideration is necessary for the education of children who show significant elevation of the response threshold.     

Effects of topiramate in adults with Prader-Willi syndrome

Prader-Willi syndrome is a multisystem neurogenetic obesity disorder with behavioral manifestations, including hyperphagia, compulsive behavior, self-injury, and mild to moderate mental retardation. In an 8-week open-label study, we evaluated adjunctive therapy with the anticonvulsant topiramate in 8 adults with Prader-Willi syndrome. Appetite was measured by a 1-hour access to food four times throughout the study and quantified with a visual analogue scale. Topiramate did not significantly change calories consumed, Body Mass Index, or increase self-reported appetite. In addition, there were no significant changes in compulsions. Surprisingly, topiramate treatment resulted in a clinically significant improvement in the self-injury (i.e., skin-picking) that is characteristic of this syndrome. Potential benefits of topiramate for self-injury should be evaluated further in controlled trials.     

Enhancing the ability of adults with mental retardation to recognize facial expressions of emotion.

The ability to recognize accurately and respond appropriately to facial expressions of emotion is essential for interpersonal interaction. Individuals with mental retardation typically are deficient in these skills. The ability of 7 adults, 1 with severe and 6 with moderate mental retardation, to recognize facial expressions of emotion correctly was assessed. Then, they were taught this skill using a combination of a discrimination training procedure for differentiating facial movements, directed rehearsal, and Ekman and Friesen's "flashing photograph" technique. Their average increase in accuracy over baseline was at least 30% during the course of the training and over 50% during the last 5 days of the training phase. Further, these individuals were able to generalize their skills from posed photographs to videotaped role plays and were able to maintain their enhanced skills during the 8 to 9 months following the termination of training. This is the first study to show that individuals with mental retardation can be taught skills that enhance their ability to recognize facial expressions of emotion.     

Influence of mental retardation severity and respondent characteristics on self-reported attitudes toward mental retardation and eugenics

Eugenics refers to the investigation of means of social control to improve the mental or physical qualities of future generations. The present study investigated whether the self-reported attitudes toward mental retardation and eugenics of a sample of 572 respondents would vary as a function of (1) severity of the mental retardation attitude referent; and (2) respondent sociode-mographic characteristics. Among the respondents, 380 were health and human service providers (66% upper division undergraduate students and 34% graduate level professionals) and 192 were upper division undergraduate students majoring in fields other than health and human services. The results supported these conclusions: (1) psychometric characteristics of the scales used to measure attitudes were adequate; (2) increasing mental retardation severity was related to increasing endorsement of eugenic principles, independent of global attitudes toward people with mental retardation; and (3) respondent education was related to the expression of eugenic attitudes toward mild mental retardation, while familiarity with people with mental retardation was related to the expression of eugenic attitudes toward moderate and profound mental retardation.