bluuebunny/crossref_metadata_2025 huggingface dataset:
Endotoxin-induced lung injury in α-galactosylceramide-sensitized mice is caused by failure of interleukin-4 production in lung natural killer T cells
Authors: Dagvadorj J , Tumurkhuu G , Naiki Y , Noman A S M , Iftakhar-E-Khuda I , Badamtseren B , Komatsu T , Koide N , Yoshida T , Yokochi T
Published: July 2010

Administration of bacterial lipopolysaccharide (LPS) known as endotoxin into α-galactosylceramide (α-GalCer)-sensitized mice causes severe lung lesions but few hepatic lesions in lethal shock, and interferon (IFN)-γ is suggested to play a pivotal role in preparation of the lung lesions. In order to clarify the mechanism of how α-GalCer sensitization causes lung lesions exclusively in mice, we examined the differential responsiveness of lungs and livers to α-GalCer sensitization. Although lung and liver natural killer T (NK T) cells both produced IFN-γ in response to α-GalCer, IFN-γ signalling was triggered only in the lungs of α-GalCer-sensitized mice. Lung NK T cells did not produce interleukin (IL)-4 in response to α-GalCer and it did not induce the expression of suppressor of cytokine signalling 1 (SOCS1) in the lungs. Conversely, IL-4 produced by liver NK T cells led to the expression of SOCS1 in the livers of the mice. Neutralization of IL-4 reduced SOCS1 expression in the livers and exacerbated LPS-induced hepatic lesions. IL-10 was produced by liver NK T cells but not lung NK T cells. However, IL-10 was produced constitutively by alveolar epithelial cells in normal lung. Lung NK T cells and liver NK T cells might express CD8 and CD4, respectively. Based on the fact that IL-4 inhibited IFN-γ signalling in the livers of α-GalCer-sensitized mice via SOCS1 expression and signal transducer and activator of transcription 1 (STAT-1) activation, no inhibition of the IFN-γ signalling in the lungs caused LPS-induced lung lesions in α-GalCer-sensitized mice. The detailed mechanism of development of the lung lesions in α-GalCer-sensitized mice is discussed.
Chrousos syndrome: a seminal report, a phylogenetic enigma and the clinical implications of glucocorticoid signalling changes
Authors: Charmandari Evangelia , Kino Tomoshige
Published: July 2010

Eur J Clin Invest 2010; 40 (10): 932–942

Background  Glucocorticoids regulate a broad spectrum of physiologic functions and play important roles in resting and stress homeostasis. Their actions are mediated by the nuclear glucocorticoid receptor (GR).

Design  Using a patient as a stimulus, we reviewed briefly the area of Primary Generalized Glucocorticoid Resistance in man and nonhuman primates.

Results  In man, Primary Generalized Glucocorticoid Resistance is a rare sporadic or familial syndrome characterized by target‐tissue insensitivity to glucocorticoids and compensatory elevations in adrenocorticotropic hormone (ACTH), leading to increased secretion of cortisol and adrenal steroids with mineralocorticoid and/or androgenic activity, and causing hypermineralocorticoidism and hyperandrogenism without Cushing stigmata. The presentation, diagnosis and therapy of this condition are summarized. Many or, most likely, all New World primates have markedly elevated cortisol and ACTH, and resistance to dexamethasone suppression, without any pathology. These primates in fact have ‘pan‐steroid/sterol’ resistance, including all five steroid hormones and 1,25‐dihydroxy‐vitamin D. In humans, the molecular basis of Primary Generalized Glucocorticoid Resistance has been mainly ascribed to recent mutations in the GR gene, which impair glucocorticoid signal transduction. In contrast, in the primates, steroid/sterol signalling systems have adapted under yet unknown selective pressures or genetic drift over many million years. Of course, other molecules of the signaling pathways may also be involved in both states. There are now a host of human states associated with tissue‐specific pathologic glucocorticoid target tissue changes. These include allergic, autoimmune, inflammatory and lymphoproliferative disorders.

Conclusions  In recognition of Professor George P. Chrousos’ extensive ground‐breaking research in this field, and for the sake of brevity, we propose that ‘Chrousos syndrome’ is used instead of ‘Primary Generalized Familial or Sporadic Glucocorticoid Resistance’.
The role of climate, habitat, and species co‐occurrence as drivers of change in small mammal distributions over the past century
Authors: RUBIDGE EMILY M. , MONAHAN WILLIAM B. , PARRA JUAN L. , CAMERON SUSAN E. , BRASHARES JUSTIN S.
Published: July 2010

Species distribution models are commonly used to predict species responses to climate change. However, their usefulness in conservation planning and policy is controversial because they are difficult to validate across time and space. Here we capitalize on small mammal surveys repeated over a century in Yosemite National Park, USA, to assess accuracy of model predictions. Historical (1900–1940) climate, vegetation, and species occurrence data were used to develop single‐ and multi‐species multivariate adaptive regression spline distribution models for three species of chipmunk. Models were projected onto the current (1980–2007) environmental surface and then tested against modern field resurveys of each species. We evaluated models both within and between time periods and found that even with the inclusion of biotic predictors, climate alone is the dominant predictor explaining the distribution of the study species within a time period. However, climate was not consistently an adequate predictor of the distributional change observed in all three species across time. For two of the three species, climate alone or climate and vegetation models showed good predictive performance across time. The stability of the distribution from the past to present observed in the third species, however, was not predicted by our modeling approach. Our results demonstrate that correlative distribution models are useful in understanding species' potential responses to environmental change, but also show how changes in species‐environment correlations through time can limit the predictive performance of models.
Inhibitor development in haemophilia A: the role of von Willebrand factor/factor VIII concentrates
Authors: GOUDEMAND J.
Published: July 2010

Summary.  The presence of inhibitors that neutralize the function of factor VIII (FVIII) decreases the haemostatic efficacy of replacement clotting factor concentrate and increases morbidity among patients with haemophilia A. Certain genetic and environmental variables have been linked to a higher incidence of inhibitors. Conversely, the presence of von Willebrand factor (VWF) in some plasma‐derived FVIII products may provide some measure of protection against inhibitor development, although the evidence is not conclusive. Clinical trials are needed to resolve this issue and determine the appropriate role of VWF‐containing FVIII concentrates in the treatment of haemophilia A patients.
A DOUBLE BLIND STUDY OF VITAMIN B6 IN DOWN'S SYNDROME INFANTS. PART 2—CORTICAL AUDITORY EVOKED POTENTIALS
Authors: Frager Joseph , Barnet Ann , Weiss Ira , Coleman Mary
Published: July 2010

Cortical auditory evoked potentials (CAEPs) were recorded at 1 and at 3 years of age in 19 children with Down's syndrome participating in a double blind trial of vitamin B6 and placebo begun in early infancy and continued for 3 years. CAEPs have previously been shown to have abnormally high amplitude in Down's syndrome patients. The CAEPs of the patients in the B6‐treated and placebo groups were compared. Only minor effects were found in the CAEPs recorded at 1 year of age. At 3 years of age, however, comparison of the B6‐treated group and the placebo group revealed significant differences in both amplitudes and latencies of CAEP components. Peak to peak amplitudes of prominent components were significantly lower in B6‐treated patients than in their placebo controls. Amplitude correlated in some cases with whole blood serotonin levels. Latencies for several prominent evoked peaks were significantly longer in B6‐treated patients.
Klinefelter syndrome and associated Fragile‐X syndrome
Authors: PUESCHEL S. M. , O'BRIEN M. M. , PADRE‐MENDOZA T.
Published: July 2010

ABSTRACT. During screening of male individuals for Fragile‐X syndrome in a residential facility for persons with mental retardation, the authors found a 21‐year‐old profoundly retarded man who displayed facial features and behaviour suggestive of Fragile‐X syndrome. The chromosome analysis revealed 47, fra(X)(q27)fra(X)(q27)Y. His physically and intellectually normal sister had 14% of X chromosomes with a fragile site. Her two sons, who were subsequently examined, were found to have Fragile‐X syndrome.

Thus, the identification of Fragile‐X syndrome in the proband during the screening process of a large institution led to the investigation of the proband's family and the subsequent diagnosis of Fragile‐X syndrome in the proband's two nephews. The ascertainment of the two affected boys permitted prompt introduction of early intervention and special education services. Genetic counselling of other at‐risk family members was carried out.
The Fragile‐X syndrome in twin sisters
Authors: COWIE V. A. , SINGH K. RAMESHWARI , WHEATER R. , BIRD J.
Published: July 2010

ABSTRACT. Two mentally handicapped dizygotic twin sisters were found to possess the Fragile‐X lesion. They showed different levels of cognitive deficit. The hypothesis that the level of cognitive function in female carriers of the Fragile‐X lesion may be influenced by Lyonization is discussed. The mother of the twins was schizophrenic. Although she was an obligate carrier her psychosis was thought not to be causally connected with the Fragile‐X status.
The elderly mentally handicapped in hospital: a clinical study
Authors: DAY K. A.
Published: July 2010

ABSTRACT. In a study of 99 long‐stay hospital residents aged 65 years and over, two thirds were functioning in the moderately to mildly handicapped range, two thirds were under the age of 75 years and there was an overall female to male ratio of 2:1. As in the general population, mobility problems, a tendency to falls and fractures, cardiorespiratory disease, deteriorating eyesight, and hearing and urinary incontinence were commonly found; all increased in frequency with age. A fifth exhibited psychiatric disorder: the commonest conditions, as expected, were the psychoses and dementia. It is suggested that retirement provision for the elderly mentally handicapped should be made within the mental handicap services and that the small group with significant geriatric problems would be most appropriately cared for within the generic geriatric services. A plea is made for evaluative studies before large scale implementation of resettlement programmes for the elderly mentally handicapped in hospital.
Negative ion effects on hemispheric processing and selective attention in the mentally retarded
Authors: MORTON L. L. , KERSHNER J. R.
Published: July 2010

ABSTRACT. The effect of high concentrations of negative air ions on a dichotic digits task was investigated in 19 mentally retarded individuals. Subjects were assigned randomly to an ion or to an unmodified‐air placebo condition under double‐blind testing. Left and right ears were precued for report order and this order was reversed for the second set of trials. Negative ions promoted greater left hemisphere lateralization on the first set of trials, and enhanced recall when switching to the opposite channels, in the second set of trials. However, the ion‐induced REA occurred at the expense of selective attention to the left channel and superior left ear recall after the right ear first condition is an anomalous dichotic listening pattern. Such paradoxical results suggest that negative ions are not necessarily beneficial or detrimental to processing. Negative ions may increase arousal, in this case amplifying a time‐phased, information processing disorder in the retarded characterized by excessive right hemisphere inhibition during early processing of receptive speech and diffuse interhemispheric excitatory activation during later processing.
Digital dermatoglyphics of Turner's syndrome
Authors: RIGNELL A.
Published: July 2010

ABSTRACT. The proposition that linger print variability between individuals might be reduced by the absence of an X‐diromosome in Turner's syndrome was rejected. In the present study of 58 XO patients, aged 15–50 years, relatives of several cases, unrelated female control samples and three unrelated male samples were investigated. The higher mean value of the TRC among patients supported the hypothesis forwarded by Pcnruse that an added X‐ or Y‐chromosome reduces the TRC and a missing one increasing it. The figures do not speak against the hypothesis that genes affecting the TRC are located on the X‐chromosome.

A summary of the major dermatoglyphic investigations in Turner's syndrome is presented.
Coffin‐Lowry syndrome and schizophrenia: a family report
Authors: COLLACOTT R. A. , WARRINGTON J. S. , YOUNG I. D.
Published: July 2010

ABSTRACT. A family is reported in which the mother and 4 of her 6 children are affected by a constellation of abnormalities including mental handicap, abnormal facies, short stature, soft fleshy hands with sapering fingers and skeletal abnormalities. The family is believed to represent a further group of individuals with Coffin‐Lowry syndrome. Additionally, one affected daughter has symptoms suggestive of schizophrenia and one affected son has severe sensorineural deafness.
Neurogenic atherosclerosis in mentally retarded persons
Authors: CHANEY R. H.
Published: July 2010

ABSTRACT. The question is raised whether arteriosclerosis (AS) may be induced in man by chronic central nervous system injury. In an institutionalized population of mentally retarded children, more AS than expected was observed. Of 1173 residents with detailed clinical and autopsy records over 40 years, 78 of them, aged 1–21 years, showed mild to moderate coronary or aorta AS. Thirty control subjects without AS, matched for sex, age and IQ, were studied in comparison. In 65 of the probands, the condition could not be explained by predisposing diseases or known risk factors. There were more lesions in those with convulsive disorder; this was felt to reflect arterial changes due to neuronal‐sympathoadrenal hyperactivity. In addition, more disease occurred in physically restricted patients; this appeared related to the stress of handicap and inability to respond to psychosocial needs. It is concluded that certain areas of brain defect predispose to atherogenesis. Down's syndrome provides a different set of influences on AS, yet the frequency of AS in these patients was similar to that of non‐Down's.
Research on mental handicap, 1957–1987: a selective review
Authors: CLARKE A. D. B. , CLARKE A. M.
Published: July 2010

ABSTRACT. Some of the major advances in research over the last 30 years are outlined. While primarily offering an account of the behavioural sciences as applied to this field, some attention is devoted to major biomedical findings which have revolutionized our potential for preventive action. Mental handicap research has been a powerful influence in changing practices.
Leaving home: the experience of people with a mental handicap
Authors: CATTERMOLE M. , JAHODA A. , MARKOVA I.
Published: July 2010

ABSTRACT. Open‐ended interviews were conducted with 12 people with a mental handicap who had left parental homes in order to live as tenants in community residential hostels. Parents and hostel staff were also interviewed. The nature of life in the family home, and the tenant's role in the move, reasons for leaving home and expectations for the future were explored. It was found that social life, autonomy and opportunities to develop self‐help skills had been limited at home. The tenants who had the widest social experience and the greatest use of self‐help skills at home were the most active in changing their situation. Tenants hoped to gain more freedom by leaving home, although they were also in agreement with parents and staff about the value of learning new skills.
Marriage in a young adult mentally retarded population
Authors: KOLLER H. , RICHARDSON S. A. , KATZ M.
Published: July 2010

ABSTRACT. Marriage was examined in a total population of mentally retarded young adults born in a 5‐year period. No one with severe retardation had married. Among the mildly retarded, significantly fewer young people had married by age 22 than in a nonretarded comparison population, and the retarded young people who had married had significantly higher IQs than the remainder of the mildly retarded population. The retarded females had significantly more problems in their marriages than nonretarded comparisons. Nevertheless, the marriages of almost half the mildly retarded young women appeared to be working out well. The retarded young men were not significantly different from nonretarded comparisons. Marriages in which both partners were retarded had many problems.
Stigma and the self‐concept of people with a mild mental handicap
Authors: JAHODA A. , MARKOVA I. , CATTERMOLE M.
Published: July 2010

ABSTRACT. Twelve people with a mild mental handicap, their mothers and members of staff of Adult Training Centres were interviewed to explore their views on the subjects of stigma and handicap, and to establish the facts about the social life and autonomy of people with a mental handicap. It was found that all participants with a mental handicap were aware of the stigma attached to them. A minority of three conceived of themselves as ‘essentially different’ from non‐handicapped people, while the majority of nine conceived of themselves as ‘essentially the same’. In contrast, the majority of mothers viewed their sons and daughters as ‘essentially different’ from non‐handicapped people. These findings do not support the claim of the social constructionist theory of the self that people's self‐concepts are primarily determined by the ways in which they are treated by the significant others. Rather, people with a mental handicap are aware of their own agency and clearly express their socio‐emotional needs.
Prediction of mortality in community and institutional settings*
Authors: EYMAN R. K. , BORTHWICK‐DUFFY S. A. , CALL T. L. , WHITE J. F.
Published: July 2010

ABSTRACT. This study attempted to re‐evaluate predictors of mortality based on a large sample of mentally retarded individuals. Additionally, mortality rates were compared among several types of placements including institutions after controlling for the most salient predictors of death. The findings indicated that non‐mobility and the need for medical support add to the risk of an early death for these children, and are significant indicators of mortality, regardless of level of retardation and placement.
Parent's perception of social and sexual functions in adolescents with Down's syndrome
Authors: PUESCHEL S. M. , SCOLA P. S.
Published: July 2010

ABSTRACT. The authors investigated parental perceptions of social interactions, interest in the opposite sex, sexual functions and issues concerning sex education in young persons with Down's syndrome. The evaluation of the data revealed that more than half of the study population showed interest in the opposite sex and are attending social gatherings. Many of the youngsters had expressed a desire to get married, however, only a few had an interest in sexual relationships. Masturbation was observed in 40% of the young men and in 22% of the young women. About half of the parents feel that their children should be sterilized or should have other forms of birth control if they would be able to reproduce. Only a limited number of adolescents had sex education. More parents who have girls with Down's syndrome than those who have boys are worried that their child may be taken advantage of sexually.
The relationship of depression to social skills and intellectual functioning in mentally retarded adults
Authors: HELSEL W. J. , MATSON J. L.
Published: July 2010

ABSTRACT. Ninety‐nine mild to severely mentally retarded adults were evaluated on a variety of measures. The battery included the Peabody Picture Vocabulary Test Revised (PPVT), the Psychopathology Instrument for Mentally Retarded Adults, the Social Performance Survey Schedule, Hamilton Rating Scale of Depression, the Beck Depression Inventory and Zung Self‐Rating Depression Scale. Depression and social skills measures correlated significantly with each other on self‐report and informant reports. Receptive vocabulary did not significantly correlate with depression or social skills measures, irrespective of self or other report format. Additionally, demographic variables such as intellectual level were not significant. The PPVT mean scores differed significantly by level of mental retardation as might be expected. The significance of these data and their implication for further study are reviewed.
Adaptation of the Zung self‐rating anxiety scale for people with a mental handicap*
Authors: LINDSAY W. R. , MICHIE A. M.
Published: July 2010

ABSTRACT. There are few instances of studies which elicit mentally handicapped subjects’ views of their own anxiety. This may be because the assessments available are inappropriate and poorly understood by the client group. The present study sets out to adapt the Zung self‐rating anxiety scale for use with mentally handicapped subjects Items were rephrased to ensure understanding, although a small degree of standardization may have been lost in this process. Similarly, response presentations were altered to ensure a reliable understanding on behalf of subjects. It was found that a simple response concerning presence or absence of the anxiety symptom was most reliable.
Referral of mentally handicapped patients to the psychiatrist: a community study
Authors: GHAZIUDDIN M.
Published: July 2010

ABSTRACT. In a retrospective study, 98 consecutive psychiatric referrals of mentally handicapped persons living in the community were examined. By far the commonest cause of referral was behaviour disturbance, followed by requests for disposal, placement or respite care. The referring agents were mostly general practitioners and social workers. Factors affecting the pattern of referral such as degree of mental retardation and life events are discussed.
Hospital to community: a pilot rehabilitation project for elderly mentally retarded
Authors: SINGH T. HARI , BALASUBRAMANIAM S. S.
Published: July 2010

ABSTRACT. Four mentally handicapped patients, three of whom were aged 75 years plus, with a mean age of 67 years (in 1981) and a total institutional life of 160 years, have been successfully rehabilitated in a group home in an active community environment since August 1981. The process of preparing them for independent living, the social services and community nursing support need, the qualitative change in their life style are described. The success is defined as a fuller community integrated living, free of institutional rigidity and freedom to do what they like with some support and using all the community services as used by other people.
The use of drugs for physical conditions in adults with mental retardation
Authors: SEARLE G. F. , BERELOWITZ G. J. , WRIGHT E. C.
Published: July 2010

ABSTRACT. A survey of the drugs given for physical complaints in two mental handicap hospitals is described. Thirty‐six per cent of 537 adults in hospital A and 43% of 944 adults in hospital B received medications and, of those who did, over hall in each hospital received only one drug. The hospital populations differed significantly but both showed a significant increase in total drug usage with increasing age in both sexes, higher among females in every group. This increase was greatest with C.V.S. drug usage, but it did not reach significance for the three most frequently prescribed groups, which were gastrointestinal drugs (to 13% of the total patients), vitamins and nutritional supplements (11%), dermatological (10%) and cardiovascular (10%) drugs. Menial level was significantly indirectly related to usage of gastrointestinal drugs, drugs for anaemia, and vitamins and nutritional supplements, and directly to usage of cardiovascular drugs.
Post mortem findings in a patient with 46,XX,fra(2)(q13)
Authors: SMITH A. , SULLIVAN J. , EVANS W. A.
Published: July 2010

ABSTRACT. A severely mentally retarded girl who suffered from grand mal seizures, myoclonic jerking and ataxia died at 18 years of age. Chromosome studies, before and after death revealed a fragile site on chromosome 2q13, the expression of which was greater in peripheral blood than in fibroblasts on two occasions. Post mortem findings did not reveal any abnormality which could be attributed to this fragile site.
The view from Walter's window: social environment and the communicative competence of adults with a mental handicap
Authors: VAN DER GAAG A. D.
Published: July 2010

ABSTRACT. This paper reports on a study of the communicative competence of adults with a mental handicap living in hospital and community settings. One hundred and twenty subjects, half from hospital settings and half from community settings, were assessed on the Communication Assessment profile (CASP). Significant differences were found between the hospital and community groups on pragmatic competence. The communication environment was shown to be an important determinant of pragmatic skills. The implications of this finding are discussed.
A behavioural intervention for Gilles de la Tourette syndrome in a severely mentally handicapped girl
Authors: ZARKOWSKA E. , CRAWLEY B. , LOCKE J.
Published: July 2010

ABSTRACT. This paper describes an attempt to treat Gilles de la Tourette syndrome (GTS) in a severely mentally handicapped girl, using a cuc‐controlled relaxation technique. Response to treatment followed a pattern frequently found in the general clinical literature. Namely tic frequency decreased during relaxation sessions but there was no generalization of effects outside of relaxation sessions. Pharmacological intervention, using clonidine, also did not reduce tic frequency. However, pimozide proved immediately effective in suppressing tics and this improvement was maintained at follow‐up. Medication side effects were noted and implications for long‐term treatment of GTS are discussed.
Cystathioninuria in Down's syndrome
Authors: HESTNES A. , BORUD O. , LUNDE H. , GJESSING L.
Published: July 2010

ABSTRACT. Secondary Cystathioninuria is associated with various pathological conditions (Gjessing, 1963; Gjessing & Mauritzen, 1965; Endres & Wuttge, 1978). In many cases, Cystathioninuria has been associated with mental retardation (Harris el al., 1959; Robb et al., 1984). As far as the authors know, Cystathioninuria has not previously been described in Down's syndrome. In 1981, in the authors' institution for the mentally retarded, all patients with Down's syndrome were screened with regard to aminoaciduria, using thin layer chromatography. In the course of this process, a case of Cystathioninuria was discovered. The results are presented in detail.
The social reception of mainstreamed children in the regular classroom
Authors: GILLMORE J. L. , FARINA A.
Published: July 2010

ABSTRACT. The purpose of the present study was to learn how a mainstreamed mentally retarded or emotionally disturbed child is received in a regular classroom by the other pupils in the class. Each of 36 fifth grade boys interacted with one of three male confederates and 36 eighth grade boys interacted with one of three other male confederates. In each case the confederate was of the same age and grade as the subjects. He was presented to the subject as an ordinary child, as a mentally retarded child, or as an emotionally disturbed child who might soon attend their school and the subject was asked to estimate how the confederate would fit into the school. The confederate was always blind to condition and, after the interaction, the subject was questioned about the confederate by an experimenter also blind to condition. Behavioural measures taken from the sound recordings of the interaction showed that the subjects in the mentally retarded and emotionally disturbed conditions behaved in a less friendly and more anxious manner than in the control condition. They also wanted more social distance from the ‘retarded’ and the ‘disturbed’ confederate and expected their peers to be more rejecting toward them relative to the control condition. Essentially the same results were obtained for fifth and eighth grade boys and little difference was found between the two types of mental handicaps. The implication of the study for deinstitutionalization are discussed.
The prevalence of taurodontism in Down's syndrome
Authors: BELL J. , CIVIL C. R. , TOWNSEND G. C. , BROWN R. H.
Published: July 2010

ABSTRACT. Taurodontism, a condition in which the pulp chambers of teeth are elongated, was observed in extracted lower molars of 12 out of 33 (36.4%) individuals with Down's syndrome (DS). It is suggested that this high prevalence is associated with a delayed ingrowth and fusion of the epithelial flaps of the developing root sheath and that taurodontism represents one of several characteristic morphological dental features in DS that result from decreased mitotic activity of cells in developing tooth germs.
Epilepsy in school children with intellectual impairments in Sheffield: the size and nature of the problem and the implications for service provision
Authors: SHEPHERD C. , HOSKING G.
Published: July 2010

ABSTRACT. This study analyses the prevalence and nature of epilepsy in all children between the ages of 5 and 16 years of age in the City of Sheffield with mild, moderate or severe intellectual impairments. An overall figure of 18% was obtained with a range of 7% for those with mild to moderate intellectual impairments to 67% for those with severe intellectual impairments and a physical disability. The severity of the epilepsy similarly varied with the most severe being in those with severe intellectual impairments and physical disabilities.
Eye abnormalities in the mentally handicapped
Authors: AITCHISON C. , EASTY D. L. , JANCAR J.
Published: July 2010

ABSTRACT. In 1987, a survey of eye abnormalities was undertaken of the mentally handicapped patients in Stoke Park Hospital, Bristol. The total population was 367 patients (262 females and 105 males). Of the 367 patients reviewed, 218 were found to have one or more eye anomalies (including the Down's Syndrome group), which shows that more than half the patients suffered from eye disorders (59%). The prevalence of the following anomalies is presented in detail: strabismus, refractive errors and cataracts. Corneal anomalies, nystagmus, retinopathy, glaucoma and other eye pathologies associated with various syndromes are noted. Eye anomalies in 31 patients suffering from Down's syndrome in the sample are recorded. Preventative measures to ameliorate the defects of eyesight, particularly in the aging population, relevance of regular eye examination and medical or surgical treatment of eye disorders are discussed.
Recent trends in incidence, morbidity and survival in Down's syndrome
Authors: McGROTHER C. W. , MARSHALL B.
Published: July 2010

ABSTRACT. The numbers of young adults with Down's syndrome is currently increasing due to improvements in survival and a period of increased incident numbers in the 1960s. Deaths from infection have shown the largest decline. Forty‐one per cent of survivors suffer from one or more serious morbid conditions, principally congenital heart disease. These changes are adding to the pressures upon local authorities for day and residential care facilities.
A population‐based study of mild mental handicap in children: preliminary analysis of obstetric associations
Authors: RAO J. MURTI
Published: July 2010

ABSTRACT. One hundred and twenty‐nine children in the 8‐ to 12‐year‐old age group in Cardiff were ascertained to have mild mental deficiency. Detailed obstetric data were obtained which indicated that nearly 69% of the children were associated with adverse obstetric factors. Fifty‐five per cent had the combined pathogenetic risk factors of pre‐, peri‐ and neonatal adversities. Forty‐two per cent of the cases had non‐optimal perinatal factors and 45% were considered to have prenatal causation. There were four children with chromosomal anomalies. The various adverse obstetric influences are discussed separately.
The use of functional analyses to test causes of self‐injurious behaviour: rationale, current status and future directions
Authors: REP A. C. , SINGH N. N. , OLINGER E. , OLSON D. R.
Published: July 2010

ABSTRACT. Self‐injurious behaviour (SIB) is a relatively common phenomenon among severely retarded persons and involves various repetitious behaviours resulting in tissue damage. Perhaps because of the damage it does, the behaviour has generated a considerable amount of applied research and discussion, and much of this research has involved attempts to reduce SIB through the manipulation of antecedents or consequences. The purpose of this paper was to determine the extent to which those conducting this research used a functional analysis of SIB to determine why the behaviour was occurring and subsequently matched treatment to one of these reasons. Results showed that only a small proportion of the studies reported analyses that would allow the experimenter to determine reasons for the self‐injurious behaviour. The discussion centres on why functional analyses are not conducted in ways that would lend treatment to be based on hypotheses of why SIB occurs.
Cerebral metabolic pattern in young adult Down's syndrome subjects: altered intercorrelations between regional rates of glucose utilization
Authors: HORWITZ B. , SCHAPIRO M. B. , GRADY C. L. , RAPOPORT S. I.
Published: July 2010

ABSTRACT. Correlations between regional cerebral metabolic rates for glucose determined by pisitron emission tomography with [18F]‐2‐fluoro‐2‐deoxy‐D‐glucose, have heen used to indicate functional associations between pairs of brain regions. This method was applied to data from 14 healthy adult subjects with trisomy 21 Down's syndrome tape < 34 years) and from 24 age‐matched healthy controls. Correlations were obtained between ratios of regional‐to‐global resting glucose utilization. In comparison to age‐matched controls, the Down's svndrome group had many smaller correlations for region‐pairs wilhin and between the frontal and parietal lubes, some correlations were large and negative in the Down's syndrome group, but large and positive in the controls. One region so affected was the inferior frontal gyrus thai includes Broca's area. This result is consistent with ihe relatively greater language impairment in Down's syndrome subjects. The thalamus also had smaller correlations with temporal and occipital regions in the Down's syndrome group compared: to controls. These results suggest that Down's syndrome is accompanied by a functional disruption of neural circuits associated with directed attention.
Rating management difficulty: a study into the prevalence and severity of difficult behaviour displayed by residents in a large residential hospital for the mentally handicapped
Authors: TUTTON C. , WYNNE‐WILLSON S. , PIACHAUD J.
Published: July 2010

ABSTRACT. This survey assessed the prevalence and severity of difficult behaviour displayed by residents in a residential hospital for the mentally handicapped as reported by the direct‐care staff. Of the hospital residents, 30.2% were reported as having at least one problematic behaviour. The men were generally more difficult than the women and those on the disturbed wards more difficult than those on residential wards. Men and women exhibited the same types of behaviours in both disturbed and residential wards. However, withdrawn/uncooperative behaviour, stealing and inappropriate sexual behaviour were more prevalent on disturbed wards; withdrawn and inappropriate sexual behaviour being exhibited by more men than women on disturbed wards. The factors most likely associated with being on a disturbed ward were sex and age; young men presenting with most management difficulty. Approximately 12% of the hospital population were defined as presenting with a ‘severe’ management difficulty. Difficult behaviour is the main reason for admission to and remaining in hospital. The concept of management difficulty and the implications on service provision are discussed.
Psychotropic drugs and mental retardation: 2. A comparison of psychoactive drug use before and after discharge from hospital to community
Authors: THINN K. , CLARKE D. J. , CORBETT J. A.
Published: July 2010

ABSTRACT. The use of psychoactive medication was examined among 64 people with mental retardation who moved from two hospitals to the community as part of a plan of resettlement. There was no significant difference between the overall use of antipsychotic drugs 2 years before discharge, on the date of discharge and after at least 6 months (mean 25 months) of community living, although changes in drugs and doses prescribed had taken place. About 39% of people were receiving antipsychotics, 32% receiving antiepileptics, 7% receiving antidepressants and 8% receiving benzodiazepines.
The effects of experience on attribution of a stereotyped personality to children with Down's syndrome
Authors: WISHART J. G. , JOHNSTON F. H.
Published: July 2010

ABSTRACT. A questionnaire in the form of a bipolar rating scale was constructed using 26 personality attributes drawn from the literature on personality of children with Down's syndrome (DS). This was administered to nine groups of adults with differing levels of experience and contact with children with DS. Adults who had frequent contact proved to be significantly less likely to rate the personality of children with DS as stereotypical than those with little contact. The implications of acceptance of the stereotype are discussed in relation to integration and to development of the mother‐child relationship.
Inhalation of a drawing pin: an unusual hazard of smoking in the mentally handicapped
Authors: LIGHTLEY J. , TYRER S.
Published: July 2010

ABSTRACT. The case of a mildly handicapped man who inhaled a drawing pin is described. Early chest radiology is recommended in unexplained respiratory symptoms in mental handicap.
Elderly residents in Dutch mental deficiency institutions*
Authors: MAASKANT M. A. , HAVEMAN M. J.
Published: July 2010

ABSTRACT. In the period 1986–1988, a cross‐sectional study was carried out in 21 mental deficiency institutions in two provinces of the Netherlands. One of the key‐questions of this study was whether there are differences between the younger and older adult mentally handicapped in Dutch mental deficiency institutions, in prevalences of diseases and sensory handicaps, mobility, motor skills and social functioning. In this article, some results of the study are presented.
Musical aptitudes, musical interests and mental retardation
Authors: Miller L. K.
Published: July 2010

A modified version of the Bentley scales of musical aptitude was given to a sample of mild and moderately retarded adults chosen on the basis of alleged musical interest or experience. Several comparison groups were also given the assessment battery. The musical nominees generally performed more accurately than both matched retarded subjects with no particular musical interests and a group of normal children matched on (Wechsler) vocabulary scores. The musical nominees showed especially high performance on the subtest assessing voice analysis in chords.
Paradox, reprimand and extinction in adults with mental handicap
Authors: WOOD V. E. , CHAMOVE A. S.
Published: July 2010

To assess the efficacy of paradoxical directives, levels of challenging behaviour during 2 weeks of paradox, reprimand and extinction were compared with baseline levels in four adults with mental handicaps attending a day centre. Paradox was the most effective procedure for reducing the frequency and severity of challenging behaviour by an average of over 70% by the end of 2 weeks and up lo 90% m certain subjects; extinction was least effective. Paradox was most effective with more defiant subjects, when staff rated treatment success as low., when improvement using extinction and reprimand was poorest, and in reducing aggressive behaviour. The present authors suggest the overjustification effect offers an explsnation for the effects of paradox.
Moyamoya disease in Down's syndrome: a report of two cases
Authors: VICARI S. , ALBERTINI G.
Published: July 2010

The case of two children with Down's syndrome and multiple cerebral infarction on CT scan are described. Angiography revealed advanced moyamoya disease.
On the association of moyamoya disease with Down's syndrome
Authors: BERG J. M. , ARMSTRONG D.
Published: July 2010

The occurrence of moyamoya disease in a child with Down's syndrome is reported. Ten previously published instances of this association are briefly reviewed, with comments on its possible significance.
Age and functional abilities of people with a mental handicap: evidence from the Wessex mental handicap register
Authors: MOSS S. C.
Published: July 2010

ABSTRACT. Funclional behaviours in relation to age were investigated using data from approximately 3000 people on the Wessex Mental handicap Register. The main functional data on the register, the National Development Team's 24‐item questionnaire, were cluster and factor analysed to derive a series of client‐defining variables. Age‐trends were analysed in relation to self and commiiniiy care skills (activities of daily living and instrumental activities of daily living), problem behaviours, chronic medical conditions, mobility and sensory abilities. Generally, it was found that differential mortality leads to an older population In gtwd health and with a high level of functional skills. As with ihe general population, however, people over 70 years old show increasing prevalence of age‐related infirmities.
Erotomania and Fregoli‐like state in Down's syndrome: dynamic and developmental aspects
Authors: COLLACOTT R. A. , NAPIER E. M.
Published: July 2010

ABSTRACT. The present authors report the case of a 42‐year‐old female with Down's syndrome who developed a misidentification syndrome, erotomanic delusions towards her Bible instructor, and increasing grandiosity following the death of her father. The authors believe that her symptoms may be formulated in developmental and dynamic terms.
Mental retardation and WAIS‐R difference scores
Authors: ATKINSON L.
Published: July 2010

ABSTRACT. This article explores the psychometric properties (reliabilities, standard deviations and measurement errors) of Wechsler Adult Intelligence Scale‐Revised (Wechsler, 1981) subtest difference scores. The sample consisted of 290 subjects with IQ <80. Results demonstrated less than satisfactory difference score reliability and disproportionate measurement error. Nevertheless, neither property was so inadequate as to render cautious profile interpretation impossible. The tabled values can help clinicians working with developmentally delayed clients interpret differences between subtest scores based on statistically reliable discrepancies.
Scatter variability in the WAIS‐R profiles of mentally retarded adults
Authors: JR C. E. WATKINS , CAMPBELL V. L. , REINBERG J. , HIMMELL C. D.
Published: July 2010

ABSTRACT. 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.
Divalproex‐responsive rapid cycling bipolar disorder in a patient with Down's syndrome: implications for the Down's syndrome‐mania hypothesis
Authors: SOVNER R.
Published: July 2010

ABSTRACT. A case of rapid cycling bipolar disorder in a person with Down's syndrome, responsive to divalproex sodium, a valproic acid derivative, is presented. This case and three other reported cases of mania in association with Down's syndrome suggest that this chromosomal disorder does not preclude the development of mania.
Tentative findings of a study of the technology needs and use patterns of persons with mental retardation
Authors: PARETTE H. P. , VANBIERVLIET A.
Published: July 2010

ABSTRACT. An examination of the technology use patterns and needs of 680 persons with mental retardation was conducted as part of a grant application process in response to P. L. 100–407, the Technology‐Related Assistance for Individuals with Disabilities Act of 1988. Important trends which were deemed valuable for state technology planning purposes were revealed which have implications for other states desiring to develop comprehensive technology systems. Responses obtained from participants indicated that needs for technology were evident in all areas of life functioning, with computer technology being reported as the area of greatest need. Most respondents reported spending less than $1000 for their technology and more than half expressed that a credit plan would have been helpful in purchasing needed technology. Transportation services was reported to be an area of need for many persons participating in the study. More than half the participants indicated the need for more information relating to technology.
Teachers' attitudes towards plastic surgery in children with Down's syndrome
Authors: SAVIOLO‐NEGRIN N. , CRISTANTE F.
Published: July 2010

ABSTRACT. The problem of whether to improve on the physical appearance of children with Down's syndrome by means of plastic surgery is a much debated question which involves bio‐ethical and ideological concepts. In this research, the opinions of a group (n=132) of Italian elementary and junior school teachers are investigated on the basis of a questionnaire. Data are analysed through the application of log‐linear models, and parameter estimates are calculated for each response category and interactions between response categories and types of teachers. Results show a greater acceptance of children with Down's syndrome as they are by people who have daily experience of them and who can get to know them better from all points of view. On the other hand, plastic surgery for children with Down's syndrome is considered with more favour by people who are less psychologically involved with them.
Subgroups in autism: are there behavioural phenotypes typical of underlying medical conditions?
Authors: GILLBERG C.
Published: July 2010

ABSTRACT. Fifty‐nine cases with infantile autism/autistic disorder were subclassified according to associated medical condition (fragile‐X, tuberous sclerosis, neurofibromatosis, hypo‐melanosis of Ito, Moebius syndrome, Rett syndrome, and a ‘new’ syndrome associated with a marker chromosome). It was concluded that, even within a group of cases fitting currently accepted criteria for autism, there is considerable variation in symptom profile depending on the exact type of associated medical condition.
The test‐retest reliability and stability of the WAIS‐R in a sample of mentally retarded adults
Authors: WATKINS C. E. , CAMPBELL V. L.
Published: July 2010

ABSTRACT. Fifty mentally retarded adults were administered the Wechsler Adult Intelligence Scale‐Revised (WAIS‐R) on two separate occasions, with the mean amount of time between testings being 2 years, 8 months. The data were examined in an effort to study the test‐retest reliability and stability of the WAIS‐R with the mentally retarded. Based on correlational, t‐value and percentage of scale score change information, the authors concluded that the WAIS‐R IQs appeared to possess good test‐retest reliability and stability over an approximate 2–5 year period for the present sample of mentally retarded adults.