A performance-based lottery to improve residential care and training by institutional staff. (1/2787)

Two experiments were conducted on four units of a residential facility for the multiply-handicapped retarded in an attempt to improve daily care and training services. Experiment I compared the effects of two procedures in maintaining the work performance of attendants, using an A-B design on two units. One procedure consisted of implementing specific staff-resident assignments, the other consisted of allowing attendants who had met performance criteria to be eligible for a weekly lottery in which they could win the opportunity to rearrange their days off for the following week. Results showed that the lottery was a more effective procedure as measured by the per cent of time attendants engaged in predefined target behaviors, and by their frequency of task completion in several areas of resident care. Experiment II replicated and extended these results to the area of work quality on two additional units, using a multiple-baseline design. The performance lottery was found to be an effective econimical procedure that could be implemented by supervisory staff on a large scale.  (+info)

The effects of social punishment on noncompliance: a comparison with timeout and positive practice. (2/2787)

The effects of social punishment, positive practice, and timeout on the noncompliant behavior of four mentally retarded children were assessed in a multitreatment withdrawal design. When programmed, the experimental procedure occurred contigent on non-compliance to experimenter-issued commands. Commands were given at 55-sec intervals throughout each experimental session. The results showed (1) lower levels of noncompliance with social punishment than with the positive-practice or timeout conditions, and (2) that relatively few applications of social punishment were required to obtain this effect. The advantages of social punishment over other punishment procedures, considerations to be made before using it, and the various aspects of the procedure that contribute to its effectiveness were discussed.  (+info)

A new X linked neurodegenerative syndrome with mental retardation, blindness, convulsions, spasticity, mild hypomyelination, and early death maps to the pericentromeric region. (3/2787)

We report on a family with an X linked neurodegenerative disorder consisting of mental retardation, blindness, convulsions, spasticity, and early death. Neuropathological examination showed mild hypomyelination. By linkage analysis, the underlying genetic defect could be assigned to the pericentromeric region of the X chromosome with a maximum lod score of 3.30 at theta=0.0 for the DXS1204 locus with DXS337 and PGK1P1 as flanking markers.  (+info)

Insulin dependent diabetes mellitus (IDDM) and autoimmune thyroiditis in a boy with a ring chromosome 18: additional evidence of autoimmunity or IDDM gene(s) on chromosome 18. (4/2787)

A 4 year 3 month old boy with insulin dependent diabetes mellitus (IDDM), autoimmune thyroiditis, slight mental retardation, facial dysmorphism, and a de novo ring chromosome 18 (deletion 18q22.3-18qter) is described. This unique association of defects could represent a chance association. Alternatively, the clinical features could be the result of the chromosomal aberration. If so, one could speculate that a gene or genes on chromosome 18 might act as a suppressor or activator of the autoimmune process by itself or in concert with other IDDM loci.  (+info)

Dilemmas in counselling females with the fragile X syndrome. (5/2787)

The dilemmas in counselling a mildly retarded female with the fragile X syndrome and her retarded partner are presented. The fragile X syndrome is an X linked mental retardation disorder that affects males and, often less severely, females. Affected females have an increased risk of having affected offspring. The counselling of this couple was complicated by their impaired comprehension which subsequently impaired their thinking on the different options. The woman became pregnant and underwent CVS, which showed an affected male fetus. The pregnancy was terminated. Whether nondirective counselling for this couple was the appropriate method is discussed and the importance of a system oriented approach, through involving relatives, is stressed.  (+info)

The Dyggve-Melchior-Clausen syndrome. (6/2787)

Two new cases of Dyggve-Melchior-Clausen syndrome are described; they belong to the fourth family from Lebanon in which this disease has been recognized. There is no genealogical linkage between these four families. A particular feature in these cases is a striking rhizomelic shortness of the arms especially in one case. Clinical and radiological findings, progression of the skeletal changes are studied, along with the review of the cases in the literature. Cytological and biochemical data indicate that the DMC syndrome is not a mucopolysaccharidosis.  (+info)

Quantitative morphological studies of developing human cerebellar cortex in various disease states. (7/2787)

A quantitative morphological assessment was carried out of the cellularity and staining properties of the cells of the layers of the human cerebellar cortex, both in the normal child and in 41 children suffering from a series of disorders including mental retardation. A computerized image analyser and highly standardized procedures were used. All of the cases of mental retardation and some cases with congenital cardiac anomalies showed abnormal cell concentrations and staining properties. 3 cases of 'cot death' also showed abnormal results. These findings are presented as a new measurable aspect of brain disease, and as a indication for further study.  (+info)

Severe mental retardation in a boy with partial trisomy 10q and partial monosomy 2q. (8/2787)

A severely mentally subnormal child with many physical stigmata was shown to have the karyotype 46,XY,-2,+der(2),t(2;10)(q31;q24)pat. Full evaluation of this patient's karyotype depended on the family studies. It was shown that a balanced translocation t(2,10) was present in 4 normal males in 3 generations.  (+info)