bluuebunny/crossref_metadata_2025 huggingface dataset:
Cerebrospinal fluid monoamines in Down's syndrome adults at different ages
Authors: SCHAPIRO M. B. , KAY A. D. , MAY C. , RYKER A. K. , HAXBY J. V. , KAUFMAN S. , MILSTIEN S. , RAPOPORT S. I.
Published: July 2010

ABSTRACT. Markers of monoamine metabolism in lumbar cerebrospinal fluid (CSF) andplasma were determined in nine young, healthy adults with trisomy 21 Down'ssyndrome (DS), 21–34‐years‐old, and in three DS subjects over 45 years, twoof whom were demented, as well as in two groups of age‐matched controls. Test scores of general intelligence, visuospatial ability, visual discrimination and verbal intelligence were reduced significantly in the old as compared to the young DS subjects. Dementia in DS was evident from a history of mental deterioration, disorientation and hallucinations. In the young DS adults, as compared to the controls, CSF 5‐HIAA and norepinephrine were significantly elevated but plasma levels were unchanged. HVA, MHPG and biopterin did not differ between the DS groups and age‐matched controls, or with relation to age in the DS or control subjects. These results suggest an increased turnover of monoamines in young adults with DS but that alterations in monoamine metabolism are unrelated to the cognitive decline with age in DS.
Cystathioninuria in a profoundly mentally retarded woman with spastic tetraplegia, deafness and abnormal liver function tests
Authors: HESTNES A. , BORUD O. , LUNDE H. , GJESSING L. R.
Published: July 2010

ABSTRACT. This article describes a profoundly mentally retarded woman with spastic tetraplegia, deafness and abnormal liver function tests with a secondary Cystathioninuria. The Cystathioninuria could be corrected with vitamin B6 supplementation.
Development, disintegration and dementia
Authors: CORBETT J.
Published: July 2010

ABSTRACT. It has been argued that there is an important group of conditions, seen for the first time in late infancy and early childhood, which are both remarkably persistent and pervasive in their influence on cognitive and social development. They combine features of childhood autism, mental retardation and cognitive deterioration and the term ‘disintegrative’ most precisely characterizes their impact on development. The term ‘psychosis’ is only usually appropriate in describing the severity of the psychiatric symptoms. In order to avoid confusion with the psychoses or dementia of adult life, the term ‘distmtegrative disorder of development’ may be preferred. While specific pathology is being identified in an increasing number of cases, the combined use of psychiatric anphysical diagonostic catagories parhaps best serves to draw attention to this important group of disorders. It is likely that, with increasing knowledge, the need for such an interim diagnostic category may become unnecessary.
The biochemical approach to mental handicap: 30 years of achievements and disappointments
Authors: STERN J.
Published: July 2010

ABSTRACT. In the past half century, biochemistry has emerged as an independent discipline that provides essential and fundamental information for medical science. It has an increasingly important role in various branches of medicine and the biochemist is frequently called upon to apply his expertise to the solution of clinical problems, amongst them the role of biochemical disturbances in the aetiology of disorders associated with mental handicap, and the investigation of factors affecting their severity and clinical course. The expectation has been that the understanding of biochemical defects will lead promptly to the suggestion of remedies.
Aggregated early intervention effects for Down's syndrome persons: patterning and longevity of benefits
Authors: GIBSON D. , HARRIS A.
Published: July 2010

ABSTRACT. Pooled findings from 21 early intervention demonstration studies for Down's syndrome infants and children yield consistency of short‐term benefits in the growth of liner motor skills, simple social repertoire and DQ/IQ scores, hut conflicting evidence in support or not of benefits in the gross motor, linguistic and cognitive/ academic domains. Support for the tenacity of gains, on follow‐up to the early years of primary schooling, is disappointing. It is recommended that: (1) intervention programmers view the key working assumptions and ideological positions governing present practices more critically; (2) intervention curricula reflect the unique biological and behavioural properties of the syndrome, taking into account individual differences which are independent of etiological label; and (3) care delivery systems be based more fully on multidisciplinary collaboration, especially between the health sciences and education fields.
Hepatitis B in a hospital for the mentally subnormal in South Wales
Authors: McGREGOR M. A. , COWIE V. A. , WASSEF K. , VEASEY D. , MUNRO J.
Published: July 2010

ABSTRACT. The resident population in a long‐stay hospital for the mentally handicapped was surveyed in order to assess evidence for past and present infection with the Hepatitis B virus. The authors found a 0.5% prevalence of carriage of Hepatitis Bc Antigen and do not recommend mass vaccination of staff and residents in this hospital.
Carpal tunnel syndrome in children with mucopolysaccharidoses: needs for surgical tendons and median nerve release
Authors: PRONICKA E. , TYLKI‐SZYMANSKA A. , KWAST O. , CHMIELIK J. , MACIEJKO D. , CEDRO A.
Published: July 2010

ABSTRACT. This report analyzes the clinical and electrophysiological results of the carpal tunnel release operation in mentally retarded children with mucopolysaccharidoses and discusses the needs for early surgical intervention.
A computer‐aided programme for promoting unsupervized activities for multihandicapped adolescents
Authors: LANCIONI G. E. , OLIVA D.
Published: July 2010

ABSTRACT. The present study was aimed at developing a computer‐aided programme for promoting performance of unsupervized activities with two multihandicapped adolescents. Simple household and occupational tasks already familiar to the subjects were selected as activities. Both subjects discriminated the pictorial representations of ihosc activities. In order to control for the effects of the computer‐aided programme, the subjects were also exposed to a card programme. Data indicate that the computer‐aided programme was successful with both subjects. Advantages and limitations of this programme are discussed.
The effects of a direct‐mail informational campaign on attitudes of industrial managers toward the mentally retarded population
Authors: RUSSELL T. , AYER F. E.
Published: July 2010

ABSTRACT. This study attempted to determine the effects of a direct‐mail campaign on the attitudes of managers and presidents of industries toward the mentally retarded. The participants in the study were 99 managers and presidents selected from a total population of 4290 within the State of Alabama, USA. Random sampling techniques were utilized to select and assign managers and presidents into an experimental group (n=50) and a control group (n=49), giving a total usable sample size of 99. The size of this sample was adequate to insure that, in 19 out of 20 cases, the sample mean was within 0.50 points of the population mean on the response scale according to Elliot (1980). The pre‐test of the attitude scale was administered to the experimental and control groups by mail. A post‐test was administered to the control and experimental groups one week after the mailing of the final pamphlet. The major finding of this study revealed positive attitude gains from pre‐test to post‐test for the experimental group following the direct‐mail campaign. No significant difference was noted for the control group from pre‐test and post‐test. Pre‐test scores of the experimental and control groups were not significantly different; however, post‐test scores between these groups were significantly different. The results of this research contribute further information on a direct‐mail approach for changing attitudes of managers and presidents of industries toward mentally retarded persons.
Analysis of blood cell populations, plasma zinc and natural killer cell activity in young children with Down's syndrome*
Authors: NOBLE R. L. , WARREN R. P.
Published: July 2010

ABSTRACT. Down's syndrome (DS) is, associated with respiratory infections, lymphoblastic leukemia and a mortality rate that is elevated particularly during the first 5 years of life. Unfortunately, most studies on immune function in DS have been performed on adults or older children, the results of which may be not relevant to a primary immune defect in DS since maturation of the immune system and compensatory mechanisms may mask to a certain degree primary immune defects which are associated with this syndrome. In the present study, several immune and hematologic parameters wert studied in DS children under 6 years of age and in age‐matched controls. The children with DS exhibited a reduced number of lymphocytes while the total number of white blood cells was normal. In addition, the children with DS had reduced numbers of circulating OKT4+ cells and a significantly depressed ratio of OKT4+ to OKT8+ cells, but their overall proportion of T‐cells was normal. Normal NK activity was observed and Cells from DS children were no more sensitive to the augmenting effects of alpha interferon than cells from control children. Hematocrit levels were slightly elevated in the DS group while plasma zinc levels appeared to be normal in this group.
WAIS‐R short forms with mentally retarded adults: a note of caution
Authors: WATKINS C. E. , HIMMELL C. D. , POLK N. E. , REINBERG J. A.
Published: July 2010

ABSTRACT. In summary, the authors found that both selected‐subtest and selected‐items short forms were highly correlated with the WAIS‐R data. Nevertheless, neither method met the t‐test and ≤3‐point IQ criteria. Therefore, the short forms examined here do not appear to be valid measures in assessing the intellectual functioning of mentally retarded adults.
A comparison of two behaviour reduction procedures: traditional extinction alone and interpolated reinforcement followed by extinction
Authors: SCHMID T. L.
Published: July 2010

ABSTRACT. Extinction responding of children with severe retardation was compared when one component of a multiple schedule (VR‐20/VR‐20) was interpolated with one session of additional reinforcement. Either variable ratio 10 (VR‐10), variable ratio 5 (VR‐5) or continuous reinforcement (CRF) were interpolated in one component prior to extinction in both. Interpolated reinforcement resulted in an extinction process at least as efficient as traditional methods. Total responding in the VR‐10 and VR‐5 components was less than in the comparable unchanged components. Results support and extend research with animals and humans. Results also suggest the feasibility of using such a procedure to gain control of behaviours that are controlled by intermittent schedules of reinforcement, are under multiple or inconsistent stimulus control or occur infrequently.
Aortic regurgitation and mitral valve prolapse with Down's syndrome: a case‐control study
Authors: GOLDHABER S. Z. , BROWN W. D. , ROBERTSON N. , RUBIN I. L. , SUTTON M. G. ST JOHN
Published: July 2010

ABSTRACT. This case‐control study was designed to determine whether aortic regurgitation (AR) and mitral valve prolapse (MVP) are specifically associated with Down's syndrome, or occur in a comparably retarded population without Down's syndrome. The 92 control subjects resided at the same institution as the 131 cases, and had mental retardation attributable to low birth weight or perinatal asphyxia. Mean (±SD) ages of the cases and the control group were 41 × 11 years compared with 40 × 13 years, respectively. The relative risk of AR among the cases (those with Down's syndrome) was 2.9 (95% confidence interval, 0.65–13.2; P=0.16) and that of MVP was 3.5 (95% confidence interval, 1.2–10.2; P=0.02). The results showed that these two valvular abnormalities are specifically associated with Down's syndrome in adults.
Psychological findings in three children with ring 15 chromosome
Authors: BORGHGRAEF M. , FRYNS J. P. , VAN DEN BERGHE H.
Published: July 2010

ABSTRACT. In this paper, the authors present a description of the clinical and psychological data of three girls with ring chromosome 15, These children were carefully observed and tested on their mental level and school skills, and the results are compared with the findings in the literature. In general, patients with ring 15 present a mild to moderate mental retardation, wish some language difficulties at early age, Observation of behaviour and social skills, present these patients as cordial, willing and with good social integration, despite their severe growth failure.
The development of a psychosocial behaviour scale for the assessment of mentally handicapped people
Authors: ESPIE C. A. , MONTGOMERY J. M. , GILLIES J. B.
Published: July 2010

ABSTRACT. The development of a new behaviour assessment scale is described. The scale focuses particularly upon disturbed behaviours which may be indicative of an ‘hysterical response tendency’. Factor analysis of the scale yielded a relatively ‘pure’ structure and the sub‐scales generated thereby provide a useful descriptive profile and help to define areas for a more detailed appraisal. Applications of the scale are discussed.
The significance of Atlanto‐axial instability in Down's syndrome
Authors: GUNASINGHAM V. , AKUFFO E. O.
Published: July 2010

ABSTRACT. Allanto‐axial instability has been a cause for concern amongst those with Down's syndrome who take pan in ‘high risk’ sports. The authors recommend radiological screening of people with Down's syndrome to help identify those with unstable and symptomatic Atlanto‐axial joints and suggest advice on management.
Teaching mentally handicapped adults how to use a cafeteria
Authors: BATY F. J. , MICHIE A. M. , LINDSAY W. R.
Published: July 2010

ABSTRACT. This study taught three moderately to severely mentally handicapped women skills to enable them to make use of cafeterias. While some work has been carried out in this area (e.g. Marholin et al., 1979; Van den Pol et al., 1981; Desai, 1983), it has not established whether hospital‐based individuals with this level of handicap and no previous experience of cafeterias can be taught how to use them. A role‐playing procedure was used during training with in vivo assessments being carried out before and after training. An in vivo generalization assessment was also conducted. Training was found to be successful with the skills taught transferring to the real life situation and subjects' improved performance, in the majority of the skill areas, generalizing to a ‘new’ cafeteria which had a completely different system of use.
Plasma amino acids in patients with senile dementia and in subjects with Down's syndrome at an age vulnerable to Alzheimer changes
Authors: WATKINS S. E. , THOMAS D. E. , CLIFFORD M. , TIDMARSH S. F. , SWEENEY A. E. , AH‐SING E. , DICKERSON J. W. T. , COWIE V. A. , SHAW D. M.
Published: July 2010

ABSTRACT.Plasma concentration of tryptophan, tyrosine, leucine and thiamine were reduced in senile dementia of Alzheimer type. In Down's syndrome, where Alzheimer‐type histology appears consistently at an early age, there was a definite type of abnormality, raised concentrations of isoleucine, leucine, phenylalanine and cystine. Because of the competition between amino acids for transfer into the brain, either or both of these types of change could lie in the aetiological chain underlying the development of Alzheimer pathology. Alternatively, these patterns could reflect the requirements of aberrant central/peripheral protein turnover.
William's syndrome in adulthood: a case study focusing on psychological and psychiatric aspects
Authors: BRADLEY E. A. , UDWIN O.
Published: July 2010

ABSTRACT. The present authors report on the history and the psychiaitric, psychological and social status of a 43‐year‐old man recently identified as having William's syndrome.
Episodic rage and aggression attributed to frontal lobe seizures
Authors: GEDYE A.
Published: July 2010

ABSTRACT. Episodic rage and aggression in mentally handicapped people have typically been very difficult to eliminate or even reduce. Behaviour modification programmes designed to reduce extremely aggressive outbursts are based on various theories which presume these behaviours are volitional. This paper considers the possibility that these behaviours are involuntary and probably due to frontal lobe dysfunction. Twenty extremely aggressive cases are reported which present the clinical picture of frontal lobe seizures. Specific eye, face, trunk and limb movements plus vocalizations are extracted from the neurologic literature on frontal lobe seizures. A similarity with Tourette syndrome is noted.
Defining fragility of the elderly severely mentally handicapped according to mortality risk, morbidity, motor handicaps and social functioning*
Authors: HAVEMAN M. J. , MAASKANT M. A.
Published: July 2010

ABSTRACT. The main aim of this study was to find a cut‐off point in the age‐distribution at which the physical fragility of the severe mentally handicapped residents increases. The following criteria for age‐related fragility were used: mortality risk, morbidity rates of chronic diseases, motor problems and social functioning. The age‐specific mortality risks were computed by means of data of ihe National Case register in the period 1978–1983. The morbidity‐rates, rates of motor handicaps and handicaps of social functioning were calculated from a random sample of 550 severe mentallv handicapped in 100 institutions and sheltered homes. The results of this cross sectional study showed a high prevalence of diseases and handicaps, and a high mortality‐rale until the age of 30, mainly determined by pre‐ and perinatal birth defects. The most healthy residents were found according to our criteria in the 30–50 year age group. A significant increase in health problems of ‘the elderly’ were observed only after the age of about 50 years, as indicated by a high prevalence of diseases and sensory handicaps, mortality risk, motor problems and problems with basis personal skills. The linguistic skills of elderly persons were significantly better in comparison with ihe younger residents.
Meeting the emotional needs of handicapped people: a psycho‐dynamic approach
Authors: PRANKISH P.
Published: July 2010

ABSTRACT. Emotional development is considered from a theoretical standpoint of normal development and applied to people with mental handicaps. Individual eases are described and the treatment they received. Implications for care models, education and further research are discussed.
The psychometric properties of the Social Training and Achievement Record: II. Reliabilities and concurrent validities
Authors: OLURIN J. , STURMEY P.
Published: July 2010

ABSTRACT. Three studies are reported which investigated the test‐retest and inter‐rater reliabilities of the Social Training and Achievement Record as well as concurrent validities with intelligence. Reliabilities of total and sub‐scale scores as well as individual items are reported. Test‐retest reliabilities over the period of one month were good for total scores, subscale scores and individual items. Inter‐rater reliabilities were more modest. At times, inter‐rater reliabilities of a small proportion of items were only at chance levels. Concurrent validities with Raven's Matrices and the Weschler Adult Intelligence Scale were comparable with other measures of adaptive behaviour. The role of direct observation in the assessment of adaptive behaviour, in particular with respect to individual skills being selected for teaching, is discussed.
Overview of a research programme designed to address key issues in the planning and delivery of services for people with mental handicap
Authors: GRIFFIN J.
Published: July 2010

ABSTRACT. This paper describes the programme of mental handicap research funded by the UK Department of Health, which addresses issues of policy, planning and service delivery of key interest to all involved professionals. The author is manager of the research programme and the research discussed covers the provision of good community residential care, hospital contraction, costs, other services in the community, supporting children with mental handicaps at home with their families, and the special needs of certain groups.
Verbalization of appropriate and inappropriate cues in discrimination reversal learning by moderately mentally retarded children
Authors: UMETANI T. , KITAO S. , KATADA A.
Published: July 2010

ABSTRACT. Effects of verbalization on discrimination reversal learning for moderately mentally retarded children were examined under three training conditions. Predicted results indicated that verbalization of both appropriate and inappropriate cues within relevant dimension significantly facilitated reversal shift performance, as compared to verbalization of only appropriate cues or non‐verbalization. Results were interpreted to be due to the fact that verbalization of both appropriate and inappropriate cues actually served to utilize attentional or verbal mediators in terms of combining appropriate with inappropriate cues within relevant stimulus dimension.
Considerations in the use of psychotropic drugs in elderly mentally retarded persons
Authors: AMAN M. G.
Published: July 2010

ABSTRACT. Elderly people tend to be large consumers of medication in general and psychotropic drugs in particular. Sedative/hypnotics, anxiolytics, antidepressants, and neuroleptic drugs tend to be the most commonly used psychotropic medications among elderly people who are not developmentally disabled. These drugs are probably commonly employed among elderly mentally retarded people as well; although the rank order of use probably differs. A number of physiological changes which accompany greater age are described and the impact on pharmacokinetic variables outlined. Other factors associated with aging, which influence drug actions, are also outlined. The net effect appears to be that most drugs are more potent, more toxic, or longer lasting among the elderly. Data are also presented on tardive dyskinesia insofar as aging is concerned. This is followed by overall conclusions and some suggested guidelines for the use of these drugs in older mentally retarded persons.
Gene map of mental retardation
Authors: WAHLSTRÖM J.
Published: July 2010

ABSTRACT. A gene map based on a compilation of genetic disorders involving mental retardation is presented. The presentation is divided into three parts; the first part describes 69 genes assigned to an autosome, the second part consists of the 73 genes assigned to the X‐chromosome, and the third part consists of genetic disorders without any known chromosome location.
Moving out: an opportunity for friendship and broadening social horizons?
Authors: JAHODA A. , CATTERMOLE M. , MARKOVA I.
Published: July 2010

ABSTRACT. The study investigated the changes in social activities and social network of people with mild learning difficulties who moved from family home and from a long‐stay hospital to live more independently in the cummunity, Twenty‐five people with a learning difficulty, 12 mothers and 33 members of stuff participated in this siudy based on interviews before and alter the move to the community. It was found that people with a learning difficulty participated in more comrminity‐bascd activities after the move but they had very few opportunities to meet non‐handicapped people, Their segregation remained a main source of dissatisfaction with their social life.
Degenerative changes in the cervical spine in Down's syndrome
Authors: TANGERUD Å. , HESTNES A. , SAND T. , SUNNDALSFOLL S.
Published: July 2010

ABSTRACT. It is well established that there is an increased frequency of atlanto‐axial instability in Down's syndrome. In two out of the 27 patients with Down's syndrome who were examined, there was an atlanto‐axial dislocation, bus without any corresponding neurological symptoms. However, scrutiny of me X‐rays revealed many cases with marked degenerative changes, particularly in the upper part of the cervical spine. The degenerative changes in Down's syndrome patients were compared with those in age‐ and sex‐matched patients examined because of cerviealgia. Patients examined because of acute neck traumatas constituted a second control group. I he results indicate that there is a significant increase in degenerative changes in the upper part of the cervical spine in Down's syndrome.
The social effects of stereotyped behaviour
Authors: JONES R. S. P. , WINT D. , ELLIS N. C.
Published: July 2010

ABSTRACT. A survey of the altitudes of adolescents towards stereotyped behaviour was carried out. Two hundred and five students attending two secondary schools in a rural market town were randomly divided into four groups, Each group was shown a videotaped recording of an actress engaging in routinehousehold tasks. Two groups saw the actress displaying stereotyped behaviour. Half the sample weretold the actress was a university student and half were told that she was a person with a mental handicap. Subjects then completed a questionnaire which asked about their perceptions of the personin the video. Results showed that more negative responses were given to a person displaying stereotyped behaviour irrespective of label. The sex of the respondent and prior contact with mental handicap were significant variables. It is suggested that the public's perception of stereotypy should be a relevant factor in the decision in reduce the behaviour.
Psychotropic drugs and mental retardation: 1. Disabilities and the prescription of drugs for behaviour and for epilepsy in three residential settings
Authors: CLARKE D. J. , KELLEY S. , THINN K. , CORBETT J. A.
Published: July 2010

ABSTRACT. The Birmingham Special Needs Register, a cumputerized database, was used to examine the disabilities of, and the use of psychoactive (psychotropic and anliepileptic) drugs among 1825 people over the age of 20 with mental retardation. People living in three different types of residential setting (hospitals, community residential facilities and family homes) were compared. The prevalence of physical disability, impaired communication and incontinence was highest among people resident in hospital, followed by those living with their families. People resident in community residential facilities were ihe least disabled group. Psychotropic drues prescribed to alter behaviour were used most frequently in hospitals (prescribed for 40.2% of people), followed by community residential (acuities (19.3%). Use was lowest in family homes (10.1%). Most hospital residents who received medication to alter behaviour did not have a diagnosed psychiatric disorder. The reported prevalence of behaviour disorders among the three populations was not significantly different. Drugs for epilepsy were prescribed for 26.2% of hospital residents, 9.3% of people in community residential facilities and 18.5% of people living with their families.
Evaluation of a parent training programme for teaching mentally retarded children age‐appropriate restaurant skills: a preliminary investigation
Authors: ALVEY G. L. , AESCHLEMAN S. R.
Published: July 2010

ABSTRACT. A programme designed to train parents to teach their mentally retarded children age‐appropriate restaurant skills was developed and evaluated. After participating in a brief training programme, three mothers attempted to facilitate greater independence in their developmentally delayed children during meals at a fast‐food restaurant. The programme was evaluated by assessing the mothers' and children's performance in restaurants using a multiple‐probe design. The results indicated that the parents' teaching interactions were influenced by the training programme and that their children's restaurant skills were enhanced. The results were discussed in the context of the normalization principle and deinstitutionalization movement.
Lowe syndrome: clinical and neuropathological studies of an adult case
Authors: GIANNAKOPOULOS P. , BOURAS C. , VALLET P. , CONSTANTINIDIS J.
Published: July 2010

ABSTRACT. A 23‐year‐old male with clinically diagnosed Lowe syndrome had bilateral cataracts, glaucoma, pendulous nystagmus, severe mental and growth retardation, hypotonia, areflexia, joints hyperextensibility, proteinuria, aminoaciduria, and metabolic acidosis. There was also severe epileptic activity (Lennox‐Gastaut syndrome). The neuropathological examination revealed a marked cerebellar atrophy and central chromatolysis in the cerebral cortex. These observations do not confirm the hypothesis of dysmyelination as formulated in previous studies. The reported case rather suggests the existence of a dynamic process starting as a stilt‐undefined metabolic abnormality that, in turn, causes various and inconsistent lesions at the microscopic level.
Ecobehavioural analysis of stereotypic and adaptive behaviours: activities as setting events
Authors: REPP A. C. , SINGH N. N. , KARSH K. G. , DEITZ D. E. D.
Published: July 2010

ABSTRACT. The effects of activities and task demands on the rale of stereotvpie and adaptive behaviour were analysed within the conceptual framework of setting events. Data on 12 menially retarded subjects were collected in real time using portable microcomputers during six activities: leisure, pre‐vocational training, gym, academic instruction, home living, and lunch. Results showed that stereotypic and adaptive responses occurred in all settings, with the two behaviours occurring simultaneously across both subjects and activities some of the time. Micro‐level analysis showed that teacher covert task demand provided the setting evenl for most of the stereotypic and adaptive responding during each activity. The importance of ecobehavioural analysis in understanding the nature of stereotypy and adaptive behaviour, anil the contribution of such an analysis to appropriate and effective intervention were highlighted.
The Motivation Assessment Scale: inter‐rater reliability and internal consistency in a British sample
Authors: NEWTON J. T. , STURMEY P.
Published: July 2010

ABSTRACT. Inter‐rater reliability of the Motivation Assessment Scale (MAS) was assessed for independent ratings of 12 adolescents and adults with severe/profound mental handicap. Correlations of item and subscale scores across raters were low. The internal consistency of subscales and total scores of the MAS was high. It is suggested that future research using the MAS should include an analysis of the reliability of this measure and features of the raters and subjects which might limit the robustness of the MASJ
AIDS: education and adults with a mental handicap
Authors: ROBERTSON P. E. , BHATE S. R. , BHATE M. S.
Published: July 2010

ABSTRACT. At present, there is a lot of concern about the acquired immune deficiency syndrome (AIDS). Efforts are being made to educate the pubUc about AIDS, so that individuals can take precautions against acquiring or transmitting the disease. Many people with a mental handicap may not benefit from the current education campaign because of limitations in their general understanding and poor or non‐existent reading skills. Two cases are presented to illustrate the difficulties encountered in educating people with a mental handicap about
The psychometric properties of the Behaviour Assessment Record (the BAR schedule)
Authors: GATHERER A. J. H. , STURMEY P.
Published: July 2010

ABSTRACT.Two hundred and forty‐seven completed Behaviour Assessment Records (BAR) for people with learning difficulties living in hospital, community residential facilities or the family home were subjected to item analysis and factor analysis. The instrument as a whole proved to be highly internally consistent, although the corrected item‐total correlation coefficients suggest the redundancy of some items on individual sub‐scales. Factor analysis produced a two‐factor solution accounting for 32–9 and 10–8% of the variance. These factors were namedPersonal self‐careandUse of public amenities.Summative scales based upon these factors were highly internally consistent. Comparisons are made between the internal consistency and factor structure of the BAR and those of similar measures of adaptive behaviour, such as the Social Training Achievement Record (Sturmeyet al., 1988), the Pathways to Independence Checklist (Walsh & McConkey, 1989), and the Adaptive Behavior Scale (Nihira, 1969a, b, 1976).
Biochemical changes and catecholamine responses in Down's syndrome adolescents in relation to incremental maximal exercise
Authors: EBERHARD Y. , ETERRADOSSI J. , THERMINARIAS A.
Published: July 2010

ABSTRACT. The aim of this study was to determine biological responses in Down's syndrome subjects for an incremental exercise lasting 10 min. After a training programme specially adapted for children and adolescents with mental handicaps, 11 healthy Down's syndrome subjects, seven boys and four girls aged from 15 to 20 years, performed a progressive exercise until exhaustion on an ergometric bicycle. The results were compared with those taken from the literature for similar aged normal subjects. The results in our series of Down's syndrome subjects showed: (a) no differences in haematologic parameters, except for a high concentration of uric acid at rest which did not increase after the test; (b) a lower blood lactate level than in maximal exercise for this age range; (c) a late mobilization of FFA; and (d) a slightly lower maximal value of catecholamines. These results may suggest a reduced sympathetic response to maximal exercise.
Hormonal and biochemical disturbances in Down's syndrome
Authors: HESTNES A. , STOVNER L. J. , HUSØY Ø. , FØLLING I. , FOUGNER K. J. , SJAASTAD O.
Published: July 2010

ABSTRACT.Clinical and laboratory endocrine variables in 29 adult institutionalized patients with Down's syndrome were compared with those of matched controls consisting of other mentally retarded patients from the same institution. Of the clinical variables, testes volume and body height were significantly lower in patients with Down's syndrome than in control patients. The thyroid function tests documented a higher average TSH level in Down's syndrome than in other mentally retarded patients. However, there was no clear‐cut correlation between TSH and thyroid hormone levels. The data indicate that there is a tendency towards primary thyroid dysfunction in Down's syndrome. In addition, there is some evidence indicating a relative failure of TSH secretion. In male patients, estradiol was elevated compared to controls. FSH and LH also seemed slightly higher in the study group, but the differences only reached statistical significance when patients on chronic medication were omitted. Prolactin was significantly greater in the Down's syndrome patients than in the controls, both over the entire sample and in the subgroup of men with Down's syndrome, withP‐values of around 0.001. The elevation of prolactin was not due to medication and did not correlate to thyroid function or difficulties during blood sampling. In females, the difference was not statistically significant. Laboratory tests that may be associated with endocrine disease or might indicate disease which could influence the endocrine status, were also included in this study. Compared with the controls, ESR, creatinine and uric acid levels were higher in Down's syndrome patients, while albumin was lower, all with P‐values lower than 0.001. Vitamin B12was moderately lower in Down's syndrome patients than in controls (P<0.05).
Self‐reinforcement in mildly mentally retarded adults: effects of motivational orientation and instructional demand
Authors: SWITZKY H. N. , HAYWOOD H. C.
Published: July 2010

ABSTRACT. The effects of external (environmental) and internal (cognitive) selfinfluences of self‐regulatory behaviour were investigated in 60 mildly mentally retarded adults. External conditions were three demand conditions (stringent, variable, and lenient). The‘internal’variable was an individual differences one, motivational orientation: one‐half of the subjects were relatively intrinsically motivated and the other half were relatively exrrinsically motivated. Both external and internal self‐influences affected performance on a motor/attention task. Subjects in the stringent‐demand condition worked harder, set higher performance standards and arranged leaner schedules of self‐reinforcement than did subjects in the lenient demand condition. Intrinsically motivated subjects worked harder, set higher performance standards, and arranged leaner schedules of self‐reinforcement than did extrinsically motivated subjects over all demand conditions. Furthermore, intrinsically motivated subjects chose higher performance standards than had been demonstrated to them in the lenient‐demand condition, and also arranged leaner schedules of self reinforcement over all demand conditions than had been demonstrated to them, compared to extrinsically motivated subjects. Internal self‐system characteristics appear to interact reciprocally with external demand characteristics to reveal substantial individual differences in patterns of self‐reward behaviour.
Growth hormone deficiency in Down's syndrome children
Authors: CASTELLS S. , TORRADO C. , BASTIAN W. , WISNIEWSKI K. E.
Published: July 2010

ABSTRACT. Down's syndrome (DS) children have been reported to have severe postnatal growth arrest and microcephaly. To determine if growth hormone (GH) deficiency plays a role in growth retardation in DS, 20 children were studied. The subjects (13 boys, 7 girls) were aged between 15 months and 13.9 years, had a height SDS ranging from − 1.19 to − 5.48, weight SDS ranging from − 0.21 to −4.58, head circumference SDS ranging from − 0.40 to − 6.6, and a skeletal age ranging from 0.9 to 4.6 SD below the mean for normal children of same age and sex. GH was evaluated by levodopa (125 mg up to 15 kg, and 250 mg between 15–30 kg), clonidine (0.15 mg m−2) stimulation tests and hGH secretory patterns by the integrated 24 h. GH concentration (IC‐GH) using a constant withdrawal pump with continuous blood collection every 30 min. The serum concentrations were: TSH, 0.7–8.0 mIU ml−1 (0.2–5.5); T4, 6.6–14.3 μg dl−1 (5–12); T3, 95–254 ng dl−1 (85–185); LH, <2.0–8.3 mlU ml−1 (<3); FSH, <1.3–7.2 mIU ml−1 (<3); testosterone, <30 ng dl−1 (5–35); estradiol, <5 ng dl−1 (<5–25); prolactin, 35.7–2.9 (F: 5–25; m 5–15); and somatomedin‐C (Sm‐C), 0.14–1.98 U ml−1 (0.08–5.90) (normal values in brackets). Peak serum GH after levodopa and clonidine was found to be below 10 ng ml−1 for both stimulatory tests in seven out of the 20 children studied. Twelve children showed a disparity between levodopa and clonidine testing. Of the 12 children, peak serum GH after levodopa was found to be below 10 ng ml−1 in five children; and peak serum GH after clonidine was found to be below 10 ng ml−1 in six. One child had a clonidine peak increase in serum GH concentration exactly 10 ng ml−1, but had a 12 h IC‐GH of 1.5 ng ml−1 (N<3.2). Two children with peak GH after clonidine above 10 ng ml−1 had a 24 h IC‐GH of 0.7 and 1.3 ng ml−1. A fourth child who had peak GH concentrations above 10 ng ml−1 with levodopa and clonidine had a 12 h IC‐GH of 0.5 ng ml−1. These data suggest that DS children have marked growth retardation, associated with a reduced serum GH response to levodopa and clonidine stimulation tests, or disparity in responses to the stimulatory tests and low 24 h IC‐GH.
Vaccination against hepatitis B in preschool children with Down's syndrome
Authors: VAJRO P. , LETTERA P. , FONTANELLA A. , SBREGLIA C. , MANZILLO E. , SARTORIO R. , DEL GIUDICE E.
Published: July 2010

Twenty‐nine healthy HBsAg‐ and HBsAb‐negative children with Down's syndrome who were living at home (mean age 42 months; 19 M, 10 F) were vaccinated against hepatitis B virus either with recombinant DNA or plasma‐derived vaccine. Both groups of children responded well to the vaccination schedules, with HBsAb seroconversion rates close to 100%. Vaccination against hepatitis B in preschool children with Down's syndrome is effective in spite of the existing abnormalities of the immune function.
Communication abilities in a case of Rett syndrome
Authors: WOODYATT G. C. , OZANNE A. E.
Published: July 2010

ABSTRACT. The conununicalion skills in a 4‐year‐old girl with Rett syndrome (RS) prior to, during and after the characteristic regression period are presented. The case study serves to provide a detailed description of the typical communication skills seen in this population, even though this child must be considered an atypical case of RS. The case study emphasizes the need for the assessment and treatment of communication skills in cases with RS to focus on the intentionality and functionality ol the child's communication.
Living in hospital and hostel: the pattern of interactions of people with learning difficulties
Authors: MARKOVA I. , JAHODA A. , CATTERMOLE M. , WOODWARD D.
Published: July 2010

ABSTRACT. This study compared interactions between the staff and residents living in hospital wards and in community‐based hostels. Twenty‐four people with moderate to severe learning difficulties participated in this study. Interactions were categorized according to who was the initiator and recipient, their purpose, attitude of the recipient, duration, and place. It was found that the hospital and hotel residents had virtually no interactions with people outwith the establishment in which they lived. The hostel appeared to offer the residents a sociable environment with more interpersonal interactions and more positive attitudes towards the interactants than the hospital. Interactions in both kinds of setting were very short, thus giving residents little chance to develop communicative skills. It is suggested that a more personal approach, such as joint activities between residents and staff, and living in small groups in ordinary housing, should be the first priorities in the effort to improve the pattern of social interactions of people with moderate to severe learning difficulties.
Anger, depression and self‐concept in adults with mental retardation
Authors: BENSON B. A. , IVINS J.
Published: July 2010

ABSTRACT. Self‐report measures of anger, depression and self‐concept that were designed for non‐retarded children were adapted for adults with mental retardation. The measures were administered to 130 adults with mental retardation who lived in the community. Informants rated the subjects on shortened forms of the three emotional indices. Informant self‐concept ratings were negatively correlated with ratings of anger and depression. On the self‐report measures, subjects who reported low self‐concept also reported high levels of depression. Mildly mentally retarded subjects were more likely to report anger than severe/moderately retarded subjects. Informant and self‐report measures were significantly correlated for self‐concept and depression, but not for anger. The results provide preliminary normative data on indices of emotional adjustment for community‐based adults with mental retardation.
The relationship between attitude to disabled siblings and ratings of behavioural competency
Authors: WILSON C. J. , McGILLIVRAY J. A. , ZETLIN A. G.
Published: July 2010

ABSTRACT. The impact of maturation factors and Auctioning level on the relationship between disabled and non‐disabled siblings was examined. Two groups of adults, distinguished by level of functioning of their mentally retarded sibling, completed a Schaefer Sibling Behavior Inventory (SBI) and participated in a semi‐structured interview. The latter explored a number of dimensions of the relationship including degree of warmth, contact and involvement. Judgements about involvement and comfort as remembered from the past and judgements about the present were obtained to derive hypotheses about possible changes with time. Data from the SBI indicated that significantly more competent siblings tended to be involved in relationships with a higher degree of reciprocity. In contrast, measures of attitude derived from interview data did not appear to relate to the functioning level of the sibling in any systematic manner. The results suggest that level of discomfort with peers may decrease significantly over the years with neither level of functioning nor gender being important variables. The extent to which non‐disabled individuals expressed a preparedness to participate in the life of their disabled sibling may relate to the life‐stage of the two. Males in particular anticipated increased future involvement. Although higher‐functioning siblings were described as more active in the relationship, this did not appear to influence perceived positiveness of regard. The implications of these results are discussed briefly.
Evaluation of a screening instrument for dementia in ageing mentally retarded persons
Authors: EVENHUIS H. M.
Published: July 2010

ABSTRACT. The value of a standardized hetero‐anamnestic questionnaire, the Dementia Questionnaire for Mentally Retarded Persons (DMR), as a screening instrument for diagnosis of early dementia, has been evaluated during a longitudinal follow‐up of 139 institutionalized ageing mentally retarded subjects. By means of decision analysis, provisional criteria have been derived for a diagnosis, based on absolute DMR scores and on scorechanges through time. For different intellectual levels, separate criteria are required for interpretation of absolute scores, but longitudinal scorechanges might be independent of the intellectual level. The DMR was only evaluated for the mildly and moderately retarded intelligence categories, because of the small number of dementing persons among the severely and profoundly retarded subjects. Causes of felsepositivity and false‐negativity are considered.
Cognitive profiles in adolescents with mental retardation
Authors: VICARI S. , ALBERTINI G. , CALTAGIRONE C.
Published: July 2010

ABSTRACT. Cognitive profiles of performance were obtained from a selected group of adolescent subjects with mental retardation (MR) (n=32; mean age; 15.4 years, S.D.=3.7) by means of an ad hoc neuropsychological battery. On the basis of each subject's IQ, the experimental sample was divided into three homogeneous subgroups (severely, moderately and mildly retarded) and cognitive performances obtained in the banery tests were compared. Subsequently, in order to clarify the qualitative aspects of MR, the cognitive patterns of subjects with the same IQ and chronological age were examined. Altogether, from a neuropsychotogical point of view, the results of this study seem to support the hypothesis that MR is a heterogeneous condition of cognitive deficits (some abilities are better preserved than others) and indicate that a set of multiple tests exploring single cognitive functions is needed in order to describe cognitive profiles in MR.
Autism in Down's syndrome: presentation and diagnosis
Authors: GHAZIUDDIN M. , TSAI L. Y. , GHAZIUDDIN N.
Published: July 2010

ABSTRACT. Although autism is said to occur rarely with Down's syndrome, it may be more common in those persons with Down's syndrome who also show superimposed behavioural problems. In this brief report, the authors explore this possibility. They describe three patients with Down's syndrome who were referred for behavioural reasons and were found to have coexisting autism. They propose that a systematic study of the association of these two conditions may have implications on research and clinical practice.