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PubMed1986

Spastic paresis, glaucoma and mental retardation--a probable autosomal recessive syndrome?

A syndrome of spastic paresis, mental retardation and glaucoma has been described only once previously (Heijbel & Jagell 1981). We describe three brothers, products of a marriage between first cousins once-removed, who appear to have the same syndrome. The brothers are not dysmorphic but they have slowly progressive spastic paresis, moderate mental retardation and glaucoma with secondary cataracts. Documentation of a second consanguineous kindred with this triad of features supports the view that this is a distinct entity with an autosomal recessive mode of inheritance.

Consanguinity↗

PubMed2000

Research on mental retardation: an agenda for the future.

This article summarizes the results of a study carried out on 12 scientific journals that deal with research on mental retardation. The purpose was to analyze the type of research currently being published. Data shows that, although most of research on mental retardation from 1991 to June, 1999 agrees with the multidimensional system proposed by the AAMR in 1992, research still tends to focus on a psychopathological model when considering people with mental retardation. We conclude by offering several suggestions on the need for a collaborative approach between researchers and professionals and the benefits of developing a supportive culture for research.

Adolescent↗

PubMed1998

A case of probable autosomal recessive ectodermal dysplasia with corkscrew hairs and mental retardation in a family with tuberous sclerosis.

We describe a woman with a probable autosomal recessive ectodermal dysplasia with corkscrew hairs and mental retardation in a family with tuberous sclerosis. Other findings included syndactyly, typical facies, dental abnormalities, dermatoglyphic hypoplasia, epidermal ridge sweat pore count slightly below normal, and keratosis pilaris. Clinical studies and genetic analysis excluded the diagnosis of tuberous sclerosis in our patient. We conclude that she has ectodermal dysplasia associated with mental retardation. This association has been described previously; it suggests the possible interrelationship of a community of ectodermal dysplasia syndromes with a distinctive structural hair abnormality (pili torti et canaliculi), variable midfacial malformations, limb defects, and other features such as mental retardation. The similarity of our patient to that described by Whiting et al. and Abramovits-Ackerman et al. suggests the autonomy of this syndrome.

Adult↗

PubMed2000

Screening adolescents with mental retardation for depression.

This study investigated the reliability and validity of the Children's Depression Inventory (CDI) using 27 adolescents with mild mental retardation attending schools in a large metropolitan area. Cronbach's alpha was 0.79 and the test/retest correlation was 0.90 at 10 days to 2 weeks, indicating adequate reliability. Scores on the self-report CDI were compared with scores on an observer-completed inventory of psychopathology filled out by teachers and other staff, the Reiss Scales for Children's Dual Diagnosis. For female students, statistically significant negative associations were found between scores on the CDI and scores on the Reiss Scales and its depression subscale. Screening adolescents with mental retardation for depression should be an important part of Individualized Educational Plans for school nurses. School staff report should not be the only method of screening for depression in adolescents with mild mental retardation. School nurses may find the CDI useful as a self-report screening tool for depression.

Adolescent↗

PubMed1991

Cataract, hypertrichosis, and mental retardation (CAHMR): a new autosomal recessive syndrome.

We have studied 2 Egyptian sibs (the offspring of normal first cousins) with congenital cataract, hypertrichosis, mental retardation, and normal chromosomes. Review showed that the condition of our patients was not similar to any previously reported entity. POSSUM lists 84 syndromes with any of the above 3 main traits. Two disorders with cataract and mental retardation, Martsolf syndrome and Mollica-Pavone-Anterer syndrome, have overlapping manifestations and therefore are particularly differentiated from our cases. We suggest that the association of congenital cataract, hypertrichosis, and mental retardation observed in this report represents a new autosomal recessive syndrome.

Cataract↗

PubMed1997

Arch fingerprints, hypotonia, and areflexia associated with X linked mental retardation.

A syndrome with distinctive facies, poor muscle tone, absent deep tendon reflexes, tapered fingers, excessive fingerprint arches, genu valgum and mild-moderate mental retardation has occurred in four males in two generations of a white family of European ancestry. The facies are characterised by square configuration, tented upper lip, and thickening of the helices, upper eyelids, and alae nasi. At birth and at maturity, growth (head circumference, height, weight) of affected males is comparable to or greater than unaffected male sibs. Moderate impairment of cognitive function was documented (IQ scores between 40-51). Carriers show no heterozygote manifestations. This X linked condition appears to be different from other syndromes with mental retardation, although there are certain similarities with the alpha thalassaemia-mental retardation syndrome (ATR-X). Linkage analysis found tight linkage to DXS1166 and DXS995 in Xq13 and Xq21 respectively.

Abnormalities, Multiple↗

PubMed1977

Effects of the label "mentally retarded" on causal explanations for success and failure outcomes.

Previous research has been inconclusive in demonstrating the effects of the label "mentally retarded" on the reactions and evaluations of others toward the labeled person. The present study derived predictions from attribution theory regarding the effects of the label on the perceptions of observers. Specifically, we hypothesized that the presence of the label would reduce the likelihood that observers would credit successful task outcomes to the labeled person's ability but enhance the likelihood that observers would credit unsuccessful task outcomes to the labeled person's ability. Such effects were not anticipated in the absence of the label. A factorial design varying Labels (mentally retarded us. no label), Task Outcomes (success v. failure), and Sex of Target Person was employed. Dependent variables measured observers' assessments of the causes of the target person's outcomes. The results strongly confirmed the hypothesis by showing that ability, effort, and task difficulty are all perceived differently for labeled than for nonlabeled target persons. The results were discussed in terms of implications for future research and proper use of the label "mentally retarded."

Achievement↗

PubMed1983

Dermatoglyphic and palmar-crease alterations as indicators of early intra-uterine insult in mental retardation.

A comparative study of unusual dermatoglyphic and palmar patterns revealed significant differences between the frequencies of certain patterns among 200 congenitally affected mentally retarded children and 500 normal controls. A scoring method demonstrating the significance of eight unusual patterns as non-specific indicators of early intra-uterine fetal insult was devised. 10 per cent of the children previously classified as idiopathically mentally retarded were shown to have been exposed to early intra-uterine insult. Dermatoglyphic and palmar-crease analysis should be included as a routine investigation for children with mental retardation of unknown cause.

Abnormalities, Multiple↗

PubMed1988

Job histories in open employment of a population of young adults with mental retardation: I.

Job histories were obtained for a population of young adults with mental retardation. No one with IQ less than 50 had been in open employment. Persons with mild mental retardation (n = 100) who received no adult services were compared to peers who were not retarded (n = 52) who left school without academic qualifications on a variety of job measures (e.g., unemployment, time out of the labor force, job turnover, level of job skill, and take-home pay). Among the 54 subjects with IQs of 50 or more who received adult services, approximately half had some open employment. Our results provide a less optimistic picture than that given by reviewers of previous research.

Adult↗

PubMed1979

The dimension of adaptive behavior in mental retardation research: an analysis of recent practices.

The dimension of adaptive behavior has been accepted by many professionals as an important component of identification in mental retardation since the definitions of Heber (1961) and Grossman (1973). In order to investigate the impact of this more comprehensive concept of mental retardation on research practices, we analyzed the use of adaptive behavior in the selection and description of subjects. This investigation was based in research articles published in the Americal Journal of Mental Deficiency from 1974 through 1978. In a significant percentage of the articles reviewed, the researchers had not attended to adaptive behavior and used only measures of intellectual functioning for descriptive purposes. These results were discussed in light of the validity of research on mental retardation. Recommendations were included for future research practices.

Adaptation, Psychological↗

PubMed1997

Acquiescence in quality-of-life interviews with adults who have mental retardation.

Acquiescence was studied in a group of 616 persons with mental retardation. When an item-reversal technique with four pairs of oppositely worded questions was used, 11.1% to 36.3% of respondents answered acquiescently. The rates of acquiescence were much lower than we expected on the basis of the results of Sigelman, Budd, Spanhel, and Schoenrock (1981). No significant correlations between acquiescence and level of mental retardation were found. Women acquiesced more than men did. A significant relation was found between acquiescence and gender combination of an interviewee and an interviewer.

Adolescent↗

PubMed1995

Composite acupuncture treatment of mental retardation in children.

128 children of mental retardation were diagnosed in accordance with the diagnostic standards proposed by WHO in 1985. The patients were treated compositely with acupuncture, auriculo-acupoint pellet pressure and herbal plasters on acupoints, bringing about improved mental developments in intelligence quotient (IQ) and social adaptation behaviour (SAB), as evidenced by recognized intelligence tests for children.

Acupuncture Points↗

PubMed1998

Abnormal frequency mismatch negativity in mentally retarded children and in children with developmental dysphasia.

The auditory event-related potential waveform termed "mismatch negativity" was examined in 12 mentally retarded children with delayed development of speech and language (aged 5-8 years) and in 13 children with developmental dysphasia (aged 5-9 years). The mismatch negativity waves were elicited with pure sine-wave tone stimuli using the oddball paradigm. We measured the peak latency and peak amplitude of mismatch negativity responses to frequency (500/553 Hz) difference. The mismatch negativity patterns were compared with those of 10 children with normal development of linguistic skills (aged 5-9 years). In both the mentally retarded and dysphasic groups, the peak amplitude of the frequency mismatch negativity was significantly attenuated when compared with the control group, but no significant difference was observed between the mentally retarded and dysphasic groups. Attenuated frequency mismatch negativity was related to impairment of linguistic skills irrespective of the child's cognitive skills. Because the mismatch negativity response reflects central auditory processing and is modal specific for auditory stimuli, this change-specific response can serve as an objective tool to elucidate central auditory deficits in children.

Aphasia↗

PubMed1993

Evaluation of an adaptive behaviour classification for mentally retarded adults.

Adaptive behaviour was assessed in a total of 119 mentally retarded adults by means of the Disability Assessment Schedule (DAS). According to a newly developed classification scheme, the level of handicap was graded on a four-step scale according to the subject's score in adaptive behaviour on the DAS; this was highly correlated both to the Coloured Progressive Matrices (CPM)-IQ (r = 0.75) and the Columbia Mental Maturity (CMM) Scale (r = 0.77). The DAS classification was associated with relevant neuropsychiatric variables. The findings indicate that the DAS classification represents a practicable way for assessing the degree of handicap in mentally retarded adults and could be especially valuable in cases where intelligence testing proves impossible, as was the case for 45% (CPM) and 39% (CMM) of the present sample.

Adaptation, Psychological↗

PubMed1980

Cross-sectional studies of grammatical morphemes in autistic and mentally retarded children.

The frequency of occurrence of functors in obligatory contexts was studied in verbal autistic and mentally retarded children matched for nonverbal mental age, and the percentages of correct use of functors were rank-ordered. The grammatical complexity of their language was also described using a transformational grammar. The data were compared to those obtained in a normal group matched for mental age and to the data presented by Brown (1973) and deVilliers and deVilliers (1973) in younger children. The autistic subjects omitted functors frequently and independently of the grammatical complexity of their language. The rank ordering of morphemes was consistent within both the autistic and mentally retarded groups but showed no correlation between the two groups or to the rank ordering described by deVilliers and deVilliers. It is suggested that functors in autistic subjects may develop in an atypical but consistent order and that this may be due to specific semantic deficits, particularly in the areas of person and time deixis.

Autistic Disorder↗

PubMed1990

[Correlations between vocal qualities and mental retardation].

This research is intended to verify the existence of vocal spectrographic alterations, with particular reference to the values of Fundamental Frequency, in patients with different levels of Mental Retardation. The results show the existence of a direct correspondence between the values of the Fundamental Frequency and the level of Mental Retardation: the spectrographic characteristics appear to be more altered in subjects with severe Mental Retardation. The spectrographics patterns are characterized by the presence of bi-phonation particularly at the onset time and at the end, and by a noise signal on constant frequency. The shifts of Fundamental Frequency and voice breakage are rarely detected.

Adolescent↗

PubMed1987

Adrenocortical function and depressive illness in mentally retarded patients.

The authors administered a 1-mg dexamethasone suppression test (DST) to 85 institutionalized adults with mild to profound mental retardation after screening to exclude false-positive nonsuppression. Thirty-one (36%) of these subjects had baseline hypercortisolemia, which was significantly correlated with age, symptoms, and "modified" DSM-III criteria for major depressive disorder. Twenty (24%) of the 85 subjects were nonsuppressors (5 micrograms/dl) after testing; nonsuppression was significantly related to age, female sex, level of retardation, symptoms, and "modified" DSM-III criteria for major depressive disorder (sensitivity 41%, specificity 81%). First-order partial correlations maintained significant relationships between age and severity of retardation but not sex. Mental retardation itself did not appear to invalidate the DST.

Adolescent↗

PubMed1981

[The intelligence structure of the mental retardates: an examination by means of the WAIS (author's transl)].

A comparison was conducted, with the purpose of investigating the intelligence structure of the mental retardates, on the results of an administration of Wechsler Adult Intelligence Scale (WAIS) to the groups of both normal and mentally retarded persons. Intercorrelations among the WAIS subtest scores for each group were separately analyzed by the method of principal component analysis. Three common factors were found for the two groups: (1) the factor of general intelligence, (2) the factor separating verbal subtests from all other subtests, and (3) the factor separating numerical subtests from all other subtests. There were no clear difference in intelligence structure between the mental retardates and the normals.

Humans↗