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PubMed1992

A new X linked syndrome with mental retardation and craniofacial dysmorphism?

We present a syndrome manifested in two half brothers and their two maternal aunts which is characterised in the two boys by severe mental retardation and craniofacial dysmorphism (broad, coarse features and marked plagiocephaly with flattened occiput), and in the aunts merely by moderate mental retardation without dysmorphic features. The brothers do not seem to fall into any previously described X linked syndrome with mental retardation.

Abnormalities, Multiple↗
PubMed1991

Current developments in the understanding of mental retardation. Part II: Psychopathology.

During recent years there has been greater recognition of the impressive degree of psychiatric disturbance that affects those with mental retardation. An increasing number of systematic studies are focusing on the prevalence, risk factors, and clinical characteristics of psychiatric disturbance within this population. In addition, traditional neuropsychiatric assessment and treatment approaches are being evaluated, and new approaches are being developed. Such efforts will undoubtedly improve the ability to effectively diagnose and treat mentally retarded individuals suffering from psychiatric disorders. This paper is the second of two reviews that explore several recent developments in biological, phenomenological, and psychopathological aspects of mental retardation.

Adolescent↗
PubMed1990

Validity of self-report of adaptive behavior skills by adults with mental retardation.

The validity of self-reports of 48 adults with mental retardation of daily living competency using the recently revised Vineland Adaptive Behavior Scales (Sparrow, Balla, & Cicchetti, 1984) was investigated. Self-report was compared to standard Vineland interviews of subjects' program counselors and to demonstrated competency in a real-life setting. On domains measuring adaptive skills, the subject and counselor interviews yielded highly consistent results. On the optional Maladaptive Behavior domain, however, the subjects with mental retardation underreported problematic behavior. Response validity and implications for use of self-report for program planning and placement decisions were discussed.

Activities of Daily Living↗
PubMed1981

Keratoconus and blindness in 469 institutionalised subjects with Down syndrome and other causes of mental retardation.

The records of 469 institutionalised mentally retarded subjects were examined. Keratoconus and blindness were present in 8 per cent and 4 per cent of the ninety-one subjects with Down syndrome. Only one case of keratoconus was found among the 378 subjects with mental deficiency of other aetiology. The incidence of strabismus, nystagmus and cataract was also increased in subjects with Down syndrome, especially in females. Cases of acute keratoconus associated with Down syndrome reported in the world literature are summarised. A total of about forty cases has ben reported between the ages of nine and fifty-three years. Sight has been preserved equally well after both conservative management and keratoplasty in eyes with and without cataracts.

Adolescent↗
PubMedDate not supplied

The Draw-a-Person task in persons with mental retardation: what does it measure?

As a widely used, easy-to-administer, and nonthreatening task, the Draw-a-Person (DAP) holds particular promise as a nonverbal index of intelligence in persons with mental retardation. The DAPs of 108 adolescents and adults with mental retardation were reliably scored using Naglieri's cognitive and emotional disturbance scoring systems. Contrary to expectations, visual-motor skills emerged as the best predictor of DAP cognitive scores. Intelligence was correlated with DAP scores, but had considerably less predictive value than visual-motor skills. DAP emotional indicator scores were only modestly associated with social adaptation; even less support was found linking DAP emotional indicators to psychopathology. Findings point to considerable caution in using the DAP as an index of intelligence or as a screen for adjustment problems or specific psychopathology. Although related to intelligence, this popular task seems predominantly to measure visual-motor development in adults with mental retardation.

Adolescent↗
PubMed1993

Agenesis of the corpus callosum with mental retardation and osseous lesions.

We report on a patient with agenesis of corpus callosum, mental retardation, and unusual hitherto undescribed bone changes. The latter include multiple Wormian bones, thin ribs, short, straight, laterally tapering clavicles, small iliac bodies, high iliac angles, triangular areas of sclerosis in the iliac bones, minimal metaphyseal irregularity, striated trabecular pattern in some metaphyses, granular ossification pattern of the patellae, hypoplastic distal phalanges, minimal flatness of phalangeal epiphyses, and retarded bone age. This patient represents a new mental retardation syndrome with agenesis of corpus callosum and unusual bone changes.

Abnormalities, Multiple↗
PubMed1994

Characteristics of community-based individuals with mental retardation and aggressive behavioral disorders.

A cohort of 199 individuals with mental retardation referred for behavioral and psychiatric crisis intervention services was studied to determine attributes differentiating physically aggressive behavior from other behavioral problems. Individuals with aggressive and nonaggressive behavior had similar neurological histories and current medical status and similar levels of seizure disorders and CNS abnormalities. Aggressive individuals more often had psychiatric diagnoses of organic brain syndrome, but frequencies of this diagnosis in each group were small. Current aggression was predicted by gender, level of mental retardation, and history of previous institutional placement; the strongest predictor was history of aggression. These data suggest a complex equation to describe social inadequacy involving interactions between CNS functioning and developmental cognitive and social variables that are only partially defined at this time. Further work to characterize this interaction almost certainly must include a prospective longitudinal analysis of social and developmental functions early in life.

Activities of Daily Living↗
PubMed1978

Effects of within-stimulus and extra-stimulus prompting of letter discrimination by mentally retarded persons.

Two different prompting procedures for teaching letter discrimination to mentally retarded persons were compared. Each of 24 severely retarded subjects received training on six alphabet letters, three by extra-stimulus prompting (adding to topographically different cue, a pointing finger) and three by within-stimulus prompting (accentuating critical features of the stimuli themselves). Letter discrimination was tested prior to, immediately following, and 2 weeks after training. A significantly greater proportion of letters trained by within-stimulus prompting was learned (p less than .001), thereby replicating past research with autistic children. Since mentally retarded persons are overselective and attend to few cues in a stimulus array, within-stimulus prompting may be more effective than between-stimulus prompting.

Adult↗
PubMed1984

Survey of bruxism in an institutionalized mentally retarded population.

Bruxism was assessed in a population of 358 institutionalized mentally retarded individuals using two independent indexes: toothwear and staff reports. Bruxism based upon toothwear was found in 58%. Bruxism identified by staff reports was found in 41% of the population. No sex differences were observed. Three bruxer subgroups were identified: daytime only (17%), night only (8%), and day and night (16%). Older residents were more likely to grind their teeth than were younger residents. These results, when compared with other reports, suggest that bruxism is more likely to occur in the institutionalized mentally retarded population than in the nonretarded population.

Adolescent↗
PubMed1993

[Hyperlipoproteinemia in mentally retarded children].

Screening revealed a significantly higher incidence of hyperlipoproteinaemia in mentally retarded children in an Institute for Social Welfare than in the healthy child population (P 1%). In the aetiology participate most markedly secondary influences ensuing in particular from complications of the basic diagnosis of oligophrenia. It is mainly a question of the action of antiepileptics, neuroleptics, the influence of viral hepatic infections and other endogenous and exogenous factors. Early diagnosis of hyperlipoproteinaemia is possible so far only by biochemical methods. It is essential for genetic counselling, for prevention of deterioration as regards mental retardation, restriction of pharmacotherapy and later for a reduced risk of development of cardiovascular disease.

Adolescent↗
PubMed1996

Essential characteristics of self-determined behavior of individuals with mental retardation.

Despite increased emphasis on self-determination for individuals with mental retardation, only a few theoretical models have been formulated that specify measurable characteristics for the promotion and evaluation of this outcome. We propose that self-determination refers to acting as the primary causal agent in one's life and making choices and decisions regarding one's quality of life free from undue external influence or interference. Self-determined behavior is autonomous, self-regulated, based on psychological empowerment, and self-realizing. We evaluated this definition by asking participants with mental retardation to complete various instruments that measured self-determined behavior and these essential characteristics. Discriminant function analysis indicated that measures of essential characteristics predicted differences between groups based on exhibition of self-determined behavior.

Activities of Daily Living↗
PubMed1995

Refractive state and correction of refractive errors among mentally retarded adults in a central institution.

In a population of 212 mentally retarded, retinoscopy could be performed in 342 eyes in 182 patients. Of these, 334 eyes were phakic. Hypermetropia of +5 or more was encountered in 20 eyes (6.0%), while 26 (7.8%) had a myopia of -5 or more. The most striking finding was a high number of severe astigmatism, cylindrical power of -3.25 or more being present in 29 eyes (8.7%). Glasses were initially prescribed to 88 patients. Six months after the prescription, 59.1% used their glasses regularly. Aspects of glass prescription to mentally retarded are discussed in the paper.

Adolescent↗
PubMed1979

Disease pattern among 942 mentally retarded persons in a Danish county.

An analytical description is presented of 942 mentally retarded persons from the county of West Zealand. The prevalence of mental retardation (i.e. persons who were registered with an IQ below 75) was 0.36%. Almost half of the patients were living in institutions. Of the patients 43% were women and 57% men. Important aetiological factors are listed. In almost one third of the patients, similar cases were present among the closest relatives; 30% of the patients were born as the fourth or subsequent child in the family; one sixth of the mothers were more than 35 years old at the birth of the handicapped child. Twenty-two percent of the patients with IQs below 50 had Down's syndrome. Seventeen percent of the patients had epilepsy had 11% cerebral palsy. In the central institution all patients had psychiatric or somatic complications.

Adolescent↗
PubMed2004

Premotor and motor reaction time of educable mentally retarded youths in a Taekwondo program.

This study investigated the educational effect of a Taekwondo program on premotor and motor reaction time in high school students who were mentally retarded but educable. Subjects were voluntarily recruited from the Special High School for the Mentally Retarded in Busan, South Korea. Subjects were divided into a trained group (n = 10) and a control group (n = 10) with balanced ages, physical conditions, and IQ. Subjects took part in pretests after practicing a Taekwondo motion 10 times with a signal presented randomly between 2 and 8 sec. after "Ready!" The motion was raising the right knee powerfully enough to crush a pine board. Subjects in the trained group took a Taekwondo course three times a week for 7 mo. This program was designed for the cognitive and physical characteristics of mentally retarded persons. After 7 mo., all subjects underwent posttests like the pretests. A four-channel EMG analyzing device (LAXTHA Inc., 2001) recorded EMG activity. Analysis of co-variance analyzed the difference of mean pre- and posttests for the two groups. For premotor RT, mean performance of the groups showed no significant difference, but significant improvement in mean motor RT by the trained group (p <.01) was noted.

Adolescent↗
PubMedDate not supplied

New X-linked mental retardation disorder with Dandy-Walker malformation, basal ganglia disease, and seizures.

We report on a 4 generation family of individuals with an X-linked form of mental retardation involving 9 affected males and 5 obligate carrier females. Key manifestations include severe mental retardation, early hypotonia with progression to spasticity and contractures, choreoathetosis, seizures, presence of a long, narrow face with coarse features, cystic enlargement of the fourth ventricle with cerebellar hypoplasia (Dandy-Walker malformation), and iron accumulation in the basal ganglia with neuroaxonal dystrophy similar to Hallervorden-Spatz disease. Of the 5 known heterozygotes, 3 are dull intellectually, and one of the 3 developed a "presenile dementia." At autopsy she had iron deposition and neuroaxonal dystrophy in the basal ganglia and atrophy of the cerebral cortex. Although the clinical findings among relatives are variable, we conclude that this is a distinct, previously unrecognized X-linked mental retardation syndrome.

Abnormalities, Multiple↗
PubMed1988

[Organization of the medico-social rehabilitation of mentally retarded adolescents in specialized pediatric boarding homes].

The authors present socio-hygienic characteristics of 1137 adolescents from 68 boarding homes for mentally-retarded children and clinical characteristics of 102 of these adolescents. The latter type of characterization includes the description of the degree of imbecility and attendant psychopathological disturbances. On the basis of the developed model of rehabilitation (organization of differential rehabilitation regimens, individual programme of communal and occupational adaptation of mentally retarded children, the organization of their training to acquire professional skills in specially created conditions of an industrial enterprise) the authors show a positive dynamics of attendant psychopathological disorders. It has been established that a certain proportion of adolescents with marked mental retardation may acquire professional skills and actively participate in socially useful production.

Adolescent↗
PubMed1985

Skin potential responses of mentally retarded children during nocturnal sleep.

Skin potential responses (SPRs) of 38 mentally retarded children were studied during their nocturnal sleep. One subject exhibited no SPRs; 19 of the 37 subjects showed more SPRs (Type A); 11, no predominant ones (Type B); and 7, fewer SPRs (Type C) during quiet sleep (no rapid eye movements, NREM sleep) than in active sleep (REM sleep). Types B and C tended to be observed more frequently for subjects with abnormal clinical EEGs than for those with normal clinical EEGs. Because investigators have established that nonretarded subjects of 3 months and over exhibit Type A, SPRs may be used as an additional assessment for diagnosis of mental retardation in early infancy.

Child↗
PubMed1982

[Autosomal anomalies in a specially selected group of children with mental retardation without signs of Down's syndrome].

Pathogenetic examinations of 96 mentally retarded children with multiple somatic anomalies and developmental defects (but without Down's disease) were carried out. Changes of the caryotype were discovered in 36 children (37.5%). In 21 children (22.0%) there were true autosomal aberrations 1/4 of which were tri- and monosomies, mainly in the form of mosaicism, and 3/4 were non-balanced structural reconstructions of the autosomes. In about 1/3 of the cases the structural anomalies were inherited by the children from their phenotypically healthy parents who had balanced translocations or inversions. In 15 children (15.6%) chromosomal variations were discovered. No differences between the clinical manifestations of the mental retardation in children with true autosomal aberrations and chromosomal variations were noted.

Abnormalities, Multiple↗