bluuebunny/crossref_metadata_2025 huggingface dataset:
Appraised significance of intellectual disability for parents of children in three age cohorts: exploring the stress process
Authors: BAXTER C.
Published: July 2010

The conceptualization of stress as a process involving stressors, mediators and manifestations of stress has opened up a way of more systematically investigating the impact of perceptions on stress experienced by parents of children with intellectua disabilities. Results obtained at the end of the first part of a two‐stage panel design study indicate that appraised significance perceptions of parents are potentially exacerbating mediators of parental stress. Findings of a multiple regression analysis were interpreted as being consistent with the proposition that the parent‐appraised significance of the child's disability for their parental role becomes increasingly salient as an exacerbating mediator of parental stress during the later childhood to early adolescent years. An attempt will be made to test this proposition when the children in the 3–5 year old cohort moves into the 10–12 year‐old age category.
Increases in knowledge following a course of sex education for people with intellectual disabilities
Authors: LINDSAY W. R. , BELLSHAW E. , CULROSS G. , STAINES C. , MICHIE A.
Published: July 2010

Although sex education programmes are thought to be useful in reachitig people with intellectual disabilities, tbere is very little evidence tbat the material taught is retained by clients. This paper reports data whicb has been collected routinely on a sex education programme. Forty‐six subjects were assessed on their level of sexual knowledge in seven areas: parts of the body, masturbation, male puberty, female puberty, intercourse, pregnancy and childbirth, and birth control and venereal disease. They were retested after a 9‐month sex education programme and tested again at a 3‐month followup. A control group of 14 subjects were tested on two occasions, 4 months apart. There were significant and substantial increases in sexual knowledge on all areas for the experimental group. The control group showed no corresponding increases in knowledge.
Differences in purine metabolism in patients with Down's syndrome
Authors: PEETERS M. A. , MEGARBANE A. , CATTANEO F. , RETHORE M.‐O. , LEJEUNE J.
Published: July 2010

Three enzymes intervening in de novo purine synthesis, as well as cystathionine B‐synthetase, have been mapped to chromosome 21. In order to gain a better understanding of purine synthesis anomalies in Down's syndrome, the present authors studied the variations in mitotic index of lymphocyte cultures to which various inhibitors or metabolites of purine synthesis had been added. In spite of common gene dosage effects, unexpected and highly significant differences were noted between Down's syndrome patients without complications and those presenting with additional psychotic features. In Down's syndrome patients without complications, a highly significant decrease in mitotic index was noted in the presence of exogenous inosine. A significant decrease in the presence of adenosine and guanosine was also noted. These findings are in keeping with the expected metabolic repercussions of genes mapped to chromosome 21. In Down's syndrome patients with psychotic complications, the in vitro reactions were quite different. A paradoxal increase in mitotic index was noted in the presence of inosine and of adenosine, but the response to guanosine did not differ from that observed in normal controls. These findings were unexpected and seem to indicate that, in spite of the gene dosage effect, psychotic Down's syndrome patients are unable to compensate abnormal purine synthesis and resulting imbalances. Furthermore, a marked difference in purine metabolic reactions was noted between Down's syndrome patients receiving supplemental folic/folinic acid and those on no therapy. This suggests that some modulation of the gene dosage effect may be possible.
Psychometric analysis in children with mental retardation due to perinatal hypoxia treated with fibroblast growth factor (FGF) and showing improvement in mental development
Authors: AGUILAR L. C. , ISLAS A. , ROSIQUE P. , HERNANDEZ B. , PORTILLO E. , HERRERA J. M. , CORTES R. , CRUZ S. , ALFARO F. , MARTIN R. , CANTU J. M.
Published: July 2010

Basic fibroblast growth factor (bFGF) has shown a neuroirophic effect in the neurons of several CNS areas. In vivo, it contributes to restore neurochcmical and morphological deficits in different rodent models of brain damage, including rats with brain damage induced by hypoxia/ischemia when FGF was intramuscularly (i.m.) administered. Toxicological and immunological studies performed in rats, mice and volunteers showed no evidence of side‐effects. Bovine FGF was i.m. administered in children with mental retardation caused by perinatal hypoxia, aged 1–15 years, at dosages of 0–4 or 0–28 μ.g kg−1, once or twice a month, over 7–12 months. Group A [n= 12; 6 treated (T), 6 controls (Ct)], group B (n= 16; 8 T, 8 Ct) and group C (n = 67; 45 T, 22 Ct) were evaluated with the P. A. R. scale, the WISC‐RM and the Gesell scale, respectively. Development increased significantly in treated children from groups A (P < 0.02) and C (P < 0.001), and IQrose by more than 10 points (P < 0.001) in group B patients.
The origin of excessive daytime sleepiness in the Prader‐Willi syndrome
Authors: HELBING‐ZWANENBURG B. , KAMPHUISEN H. A. C. , MOURTAZAEV M. S.
Published: July 2010

The polygraphically recorded sleep‐wake continuum of 21 Prader‐Willi syndrome (PWS) patients was compared with that of 19 normal people. In the Prader‐Willi group, excessive daytime sleepiness (EDS) is found in 95% of subjects, and rapid eye movement (REM) sleep disorders occur in 52%. These two features were significantly different from the normal group of subjects. No indications were found for the presence of the apnoea syndrome. The REM sleep disorders are: sleep onset rapid eye movements (SOREM), REM sleep in naps, many arousals during REM sleep, and a significant decrease in total REM sleep. These disturbances in the Prader‐Willi group, combined with the presence of EDS and sometimes of cataplexy, are likely to be expressions of a narcoleptic syndrome although this was not sustained by the HI‐A‐DR2 expression above normal. The quality of life of PWS subjects can be improved in some cases by treating them as narcoieptic patients.
Complete recovery of cytochrome oxidase and superoxide dismutase activities in the brain of brindled mice receiving copper therapy
Authors: YOSHIMURA N. , HATAYAMA I. , SATO K. , NISHIMURA M.
Published: July 2010

To elucidate the roles played by copper‐containing en2yines in the brain degeneration associated with Menkes disease, the brains of brindled mouse hemizygotes (BMs) were studied histochemically and biochemically before and after copper therapy. Light and electron microscopic histochemistry revealed that, while neuronal mitochondria in BM brains demonstrate only a weak diaminobenzidine reaction for cytochrome oxidase, these exhibit strong activity after therapy and in control mice. Biochemical assays of enzyme activity revealed only 30% of the normal level before a single subcutaneous application of 50 |jLg of CuCb, whereas neuronal mitochondria of BMs surviving 8 months after the copper therapy displayed essentially no difference from the controls. Similar results were also gained for superoxide dismutase activity, although the reduction was less marked. The present findings provide direct support for decreased activities of copper‐containing enzymes being responsible for the mitochondrial abnormalities and brain degeneration associated with Menkes disease.
Development, behaviour and seizures in 300 cases of tuberous sclerosis
Authors: HUNT A.
Published: July 2010

Three hundred cases of tuberous sclerosis were surveyed by postal questionnaire, including 240 cases with learning difficulties. People who have tuberous sclerosis and severe learning disabilities also have a higher frequency of epileptic seizures than people with learning difficulties from all causes. Pervasive developmental disorder and hyperactive behaviour are behavioural features of the disorder. Sleep problems and the level of vigilant care required because of seizures or behaviour have implications on the level of service needed to support people with tuberous sclerosis in the community.
Service use among school leavers with severe learning difficulties: the views of carers
Authors: INEICHEN B.
Published: July 2010

School leaving is a time when difficult decisions have to be taken. Difficulties are likely to be particularly complex when the school leaver has severe learning difficulties. Carers of 61 young people living in one East London Health District were interviewed about their child's experience of service use at around the time of school leaving. The timing of school leaving appears to have been well handled, and the experience of those who went on to further education was positive. The school social work service was greatly appreciated. On the debit side, many informants were critical of the quality of short‐term residential care. The commonest concern was for the prompt availability of day care. As in the few other studies of this subject, the impression that services appeared uncoordinated was given by carers.
Asperger's syndrome: three cases and a discussion
Authors: SIMBLETT G. J. , WILSON D. N.
Published: July 2010

The recently revived academic interest in and controversy over the subject of Asperger's syndrome is briefly discussed. Gillberg & Gillberg's operational criteria for its definition are used to isolate three cases of the syndrome presenting over a single year to a mental handicap service. The cases are described in detail. Common themes and associations with the available literature are discussed. In all three cases, the struggle for clarity in the syndrome's nature or specificity in the literature is also reflected in aspects of their clinical management.
An analogue assessment of hand stereotypies in two cases of Rett syndrome
Authors: WEHMEYER M. , BOURLAND G. , INGRAM D.
Published: July 2010

Stereotype hand movements are one behavioural manifestation of Rett syndrome, a neurodegenerative disorder resulting in severe mental retardation. They are neuropathological in origin, and constitute a barrier to the acquisition and exhibition of more developmentally appropriate behaviours, as well as posing a health risk for some individuals. The present study used an analogue assessment procedure, in which experimental conditions were constructed varying in the consequences delivered contingent upon the exhibition behaviour, to examine environmental correlates to the moment‐to‐moment expression of these behaviours. Results are discussed regarding their implications for intervention and research into the aetiology of stereotypic behaviours.
Normalized growth velocity in children with Down's syndrome during growth hormone therapy
Authors: ANNERÉN G. , GUSTAFSSON J. , SARA V. R. , TUVEMO T.
Published: July 2010

Between 6 months and 3 years of age, growth velocity in children with Down's syndrome (DS) is markedly reduced in comparison to that of healthy children. However, after 3 years of age, it is almost normal. Thus, growth retardation becomes pronounced during the period when growth hormone (GH) starts to regulate growth. The present authors report the long‐term effects of GH‐therapy in 16 children with DS, who are being treated for 3 years from the age of 6–9 months. The treatment, Genotropin®, 0–1 U kg−1 BW day−1, was started at a mean age of7.4 (6.9) months. The results after 12 (n= 16), 24 (n= 12) and 30 (n = 8) months are presented. The mean height standard deviation score, SDS (range; Swedish standard), before therapy was ‐ 1.8 (−0.5 to −3.1) and the mean head circumference was −1.2 (−0.4 to −3.5). After 12, 24 and 30 months, the mean height SDS were −1.1 (−0.8 to −1.9), −0.9 (0 to −1.5) and −0.9 (0.1 to ‐ 1.5) and the mean head circumference SDS were −1.1 (0 to −2.5), ‐ M (0 to −2.2) and −1.2 (−0.5 to −2.0), respectively. During hGH‐treatment, the children with DS thus gained height during the first year, and then followed the growth rate of healthy Swedish children. When compared to growth charts for children with DS the mean height of these children started at the fiftieth centile and reached the ninety‐fifth centile after 24 months of treatment. Head circumference only slightly increased during the therapy, and not to the same extent as height. This indicates that small head circumference in DS is not only an effect of growth retardation, but also due to microcephaly.
A longitudinal study of cognitive skills and communication behaviours in children with Rett syndrome
Authors: WOODYATT G. C. , OZANNE A. E.
Published: July 2010

Changes in the cognitive, communicative and interactive development of a group of six girls with Rett syndrome (three younger and three older) were documented over a 3‐year period. All six maintained a profound level of intellectual performance and a preintentional level of communication in which caregivers assigned meaning to the girls' limited behaviours. However, marked individual variation was noted in both cognitive and social interaction skills. The study supported the notion of an increased perception of social interactiveness by the caregivers over time. The development of means‐end behaviour, in particular, seems closely linked with the measured Increase in behaviours inferred as communicative. However, the results also show that factors such as cogniiive level, physical status and educational intervention may be related to this perception.
Diagnosis and treatment of psychiatric and behavioural disorders in mentally retarded individuals: the state of the art
Authors: DOšen A.
Published: July 2010

It is now well known that a higher proportion of people with mental retardation show behavioural and psychiatric disorders compared to their non‐mentally retarded counterparts. However, the exact relationship between psychiatric illness and behavioural disorder in this population is far from clear. There are problems of using the standard diagnostic and classification categories in this population, particularly in those who are severely and profoundly mentally retarded. Recently, there have been many novel approaches to this diagnostic dilemma, including ‘developmental approach’ and ‘psychodynamic approach’. In the Netherlands, the so‐called ‘developmentaldynamic’ approach has been used in the diagnosis and treatment of behavioural and psychiatric disorders in the mentally retarded. There have also been many treatment approaches, including drug therapy, behavioural treatment, psychotherapy, cognitive and social learning. Also the Dutch, Swiss and German traditions have extensive experience in the directive pedagogical treatment of the mentally retarded.
Mental health services for people with mental retardation: a framework for the future
Authors: Day K. A.
Published: July 2010

The mental health needs of mentally retarded people cannot be met satisfactorily with generic mental health services. Specialized services are required. A number of different service models have evolved which require evaluation. They include subregional units, community‐based services with a small admission facility, integrated services and specialist teams. Staff working in specialist mental health services must be appropriately trained.
Initial encounters between people with a mild mental handicap and psychiatrists: an investigation of a method of evaluating interview skills
Authors: DUCKWORTH M. S. , RADHAKRISHNAN G. , NOLAN M. E. , FRASER AND W. I.
Published: July 2010

ABSTRACT. This study repons the results of an initial study into the ability of psychiatrists to interview people with a mild mental handicap who are accompanied by a caregiver. Initial interviews by 12 psychiatrists of different ages (30–57 years) and differing levels of experience with this client group were recorded on video tape. Three of the recordings were used to train four judges who were familiar with examining clinical interactions, though not between doctors and patients. The judges used a specially designed rating scale. The remaining nine interviews were evaluated independendy by the four judges. There was a correlation of 0.65 (Kendall's coefficient of concordance, W) for the judges' evaluations using the rating scale for the nine interviewers. However, there was greater agreement between judges as to who were the best and worst interviewers (W=0.86). Some of the aspects of the interviews which contributed to the variability in the judgments are discussed.
Fluoxetine treatment of depression and associated self‐injury in two adults with mental retardation
Authors: SOVNER R. , FOX C. J. , LOWRY M. J. , LOWRY M. A.
Published: July 2010

ABSTRACT. The use of fluoxeiine to treat chronic depression and associated self‐injurious behaviour (SIB) in a woman with severe mental retardation and a man with profound mental retardation is reported. In the first case, behavioural monitoring of treatment response revealed a dramatic decrease in SIB and a normalization of the woman's sleep disturbance. In the second case, SIB and the use of mechanical restraint decreased substantially. In both cases, anecdotal reports also indicated a diminution of other depressive symptoms. These cases highlight the need to consider an affective disorder as a cause of SIB in persons with severe and profound developmental disabilities. The behaviour monitoring system proved to be a practical aid in the diagnosis of depression and evaluation of antidepressant treatment in individuals who were incapable of self‐report.
Autopsy findings in patients with mental handicap
Authors: COLE G. , NEAL J. W. , FRASER W. I. , COWIE V. A.
Published: July 2010

Abstract. Autopsies were carried out on 60 mentally handicapped patients and the brain was examined in detail in all cases. The clinical records were studied and correlated with the pathological findings, A variety of pathological changes were found in the central nervous system, and there was a high incidence of microcephaly and gross abnormalities among the patients with epilepsy and neurological dysfunction. The incidence of Alzheimer's disease was very high among patients with Down's syndrome, but in those patients without Down's syndrome, the incidence appeared to be much the same as in the general population. The most common cause of death was respiratory disease, followed by cardiovascular disease. There was a high incidence of volvulus among the group with epilepsy. The findings are discussed, and reference made to the long‐term care of the mentally handicapped. The study demonstrates the importance of the autopsy in terms of furthering knowledge in the field of mental handicap.
Environmental and temperament assessments of children with Down's syndrome
Authors: PUESCHEL S. M. , MYERS B. A.
Published: July 2010

ABSTRACT. This study was designed to investigate the family environment and the temperament of children with Down's syndrome (DS). Parents of 40 children with DS completed the Family Environmental Scale (FES). They also were asked to fill out the Temperament Assessment Battery for Children (TABC) for both the child with DS and the nearest same‐sex sibling. A similar questionnaire of the TABC (teacher form) was sent to the children's teachers. The results of these investigations revealed that there were high scores in categories cohesion, expressiveness, achievement, moral/religious emphasis, organization and control on the FES, indicating that family members in the study cohort are relationship oriented, provide support for one another, emphasize ethical values and are able to express their feelings. Comparing TABC results between children with DS with their siblings, significant differences were observed in such categories as adaptability, approach/withdrawal and persistence. Control children were scoring higher in the adaptability and persistence categories, whereas children with DS achieved significantly higher scores than the control children in the approachiwithdrataal category.
The need for assessment of sensory functioning in ageing people with mental handicap
Authors: VAN SCHROJENSTEIN LANTMAN‐DE VALK H. M. J. , HAVEMAN M. J. , MAASKANT M. A. , KESSELS A. G. H. , URLINGS H. F. J. , STURMANS F.
Published: July 2010

ABSTRACT. Within the framework of a study on the ageing process of people with mental handicap in the Netherlands, information about visual and hearing impairments in 1583 people with mental handicap living in group homes or institutions was obtained from their physicians by means of a written questionnaire. Of the people with Down's syndrome (DS) who were older than 50 years of age, 46% had a visual impairment, whereas approximately 13% of subjects with other causes of mental handicap at the same age experienced similar visual impairment. Hearing loss in this age group was reported in 28% of people with DS, but only in 8% of subjects with other causes of mental handicap. The most common eye condition was cataracts, and the most frequent cause of hearing impairment was infection. In people with severe and profound mental handicap of all ages, sensory impairments were more frequent than in persons with mild or moderate mental handicap. Glasses or hearing aids were rarely used by people with severe or profound mental handicap. Assessment of visual and hearing impairments in people with mental handicap seemed clearly indicated, especially in those aged 50 years and older, in those with DS, and in those with severe or profound mental handicap.
Care dependence and services planning
Authors: MAASKANT M. A. , KESSELS A. G. H. , FREDERIKS M. A. , HAVEMAN M. J. , VAN SCHROJENSTEIN LANTMAN DE VALK H. M. J. , URLINGS H. F. J. , STURMANS F.
Published: July 2010

ABSTRACT. For policy purposes as well as for the provision of individual care, it is relevant to know which individual characteristics have an impact upon the level of care dependence. For policy purposes, it is preferable to predict the level of care dependence as efficiently as possible. This study of residents of institutions and group homes for people with mental handicap showed that profoundly mentally handicapped residents are almost all totally dependent upon care: additional information about the individual characteristics of this group is superfluous. The results of logistic regression analyses for residents with severe or mild mental handicap showed that age, gender and aetiological diagnosis (Down's syndrome or other) do not have significant predictive power. For the level of care dependence, adding the score on Activities of Daily Life (ADL) to the various models improved the predictive power. It depends on the level of mental handicap and on the level of care dependence which one wants to predict which other individual characteristics have to be included in order to improve predictive‐power.
Polydipsia amongst adults with a learning disability in an institution
Authors: DEB S. , BRAMBLE D. , DRYBALA G. , BOYLE A. , BRUCE J.
Published: July 2010

ABSTRACT. A hospital‐based adult learning disabled population (n = 371) was screened for polydipsia with the help of a purpose‐designed questionnaire. Polydipsia was defined as excessive drinking of more than 31 of non‐alcoholic fluid over a 24‐h period. Altogether, 23 (6.2%) subjects were found to have polydipsia. The polydipsic group was compared with the whole hospital population on variables such as age and IQ distribution. A matched group of 23 individuals without a history of polydipsia was drawn from the same hospital population. The polydipsic and the matched group were compared using various biochemical and psychological measures. Thirty‐five per cent of polydipsic patients, compared to 13% of the matched group, showed evidence of compensated hyponatraemia. This difference was not significant. There was no significant difference between the polydipsic and the matched group in the frequency of psychiatric illness, behavioural problems or autism. There also was no significant difference in the IQ levels of the polydipsic patients and the total hospital population. Polydipsia in this population is largely seen as part of an abnormal behavioural repertoire without any evidence of possible organic cause, except unidentified diabetes mellitus. Klein Levin syndrome and pica were represented in the polydipsic group, but not amongst the matched group.
A survey of aggressive behaviour among a population of persons with intellectual disability in Queensland
Authors: SJGAFOOS J. , ELKINS J. , KERR M. , ATTWOOD T.
Published: July 2010

ABSTRACT. Asurvey was conducted on aggressive behaviour within a population of 2412 persons with intellectual disability in Queensland, Australia. Two hundred and sixty‐one individuals were identified who engaged in at least one form of aggressive behaviour, yielding an overall prevalence of 11 %. The relative prevalence of aggressive behaviour was higher among institutionalized persons (35%) when compared to those living in group homes (17%) or other community‐based facilities (3%). The aggressive behaviour sample included a higher percentage of males (64%). Most were described as functioning in the severe/profound (54%) or moderate (31%) range of intellectual disability with one‐third having no intelligible speech. Two‐thirds of the sample received medication for their challenging behaviour, while only one‐third had behavioural programmes. Eighty per cent engaged in three or more forms of aggression. Many also displayed self‐injury (34%) or properly destruction (30%). Surveys of aggressive behaviour may prove useful for coordinating services.
The association between Coffin‐Lowry syndrome and psychosis: a family study
Authors: SIVAGAMASUNDARI U. , FERNANDO H. , JARDINE P. , RAO J. M. , LUNT P. , JAYEWARDENE S. L. W.
Published: July 2010

ABSTRACT. This paper discusses a family presenting with features of Coffin‐Lowry syndrome, namely abnormal facies, skeletal abnormalities and mental handicap. Two of the mildly affected females had psychotic illness with predominant depressive features, and all the severely affected males had profound sensor‐ineural deafness.
Behaviour problems in Angelman syndrome
Authors: Summers J. A. , Allison D. B. , Lynch P. S. , Sandier L.
Published: July 2010

Angelman syndrome (AS) is a genetic disorder that is associated with a deletion on chromosome 15, and is characterized by abnormalities or impairments in neurological, motor and intellectual functioning. While behaviour problems have been reported iri clients with AS, relatively little is known about their developmental course and outcome. In this study, data on the nature and prevalence of behaviour problems among clients with AS were gathered from two sources: (i) a review of published case reports; and (2) parent responses to a survey of behaviour problems in a small (n= 11) sample of children with AS. Data from both sources showed that behaviour problems were present in males and females of all ages, and included language deficits, excessive laughter, hyperactivity, short attention span, problems with eating and sleeping, aggression, noncompliance, mouthing of objects, tantrums, and repetitive and stereotyped behaviour. Identification and treatment of severe behaviour problems in clients with AS may improve their adaptive functioning.
Urine fetish in a man with learning disabilities
Authors: Collacott R. A. , Cooper S.‐A.
Published: July 2010

Urine fetishes are uncommon. A 30‐year‐old man with leaming disabilities and a fetish for observing women urinate is described, and the possible dynamic aspects of his paraphilia are discussed.
Measurement of attitudes of trainee professionals to people with disabilities
Authors: Beckwith J. B. , Matthews J. M.
Published: July 2010

The importance of measuring attitudes of trainee professionals to people with disabilities was addressed. A questionnaire which included the Scale of Attitudes towed Disabled Persons (SADP) and the Intellectual Disability Misconceptions Scale (IDMS) was completed by first and final year tertiary students enrolled in a 3‐year course on intellectual disability. The attitude measures did not discriminate between the two groups of students, psychometric properties were poor and the factor structure of the SADP did not replicate previous findings. As expected, there was some convergence between the two attitude measures, and relationships to subject variables were consistent with previous research. Recommendations for subsequent research were discussed.
Personality and trait disturbances in an adult mental retardation population: significance for psychiatric management*
Authors: Goldberg B. , Gitta M. Z. , Puddephatt A.
Published: July 2010

The authors demonstrate the high prevalence of personality disorders in 384 intellectually impaired individuals examined in both community and institutional settings in southwestern Ontario, Canada. The relationship of specific clusters of personality disorders to later onset of psychiatric disorders in intellectually impaired individuals is suggested. New approaches to the management of personality disorders are noted utilizing clinical case studies. Differences and similarities in nomenclature in American and International diagnostic classifications are illustrated in this study. The use of a detailed longitudinal and cross‐sectional collection of biological, social and standardized personality schedules is demonstrated as feasible in this special population.
Care dependence and individual care provision
Authors: Maaskant M. A. , Kessels A. G. H. , Frederiks C. M. A. , Haveman M. J. , van Schrojenstein Lantman‐de Valk H. M. J. , Urlings H. F. J. , Sturmans F.
Published: July 2010

For policy purposes as well as for the provision of individual care, it Is relevant to know which individual characteristics have impact upon the level of care dependence. For purposes of individual care provision, characteristics which can be influenced and which also have an important impact upon the care dependence are of interest. This study showed that the profoundly mentally handicapped are almost all totally dependent upon care, and therefore additional information about individual characteristics is superfluous. The results of logistic regression analyses showed a statistically significant and important impact of ADL‐functions. Exactly which other characteristics are relevant to consider depends upon the level of care dependence and the level of mental handicap.
Eating disorders in adults with intellectual disability
Authors: Gravestock S.
Published: July 2010

There is an increasing focus on the nutrition of people with intellectual disability (ID), but less interest in the range of eating disorders (EDs) that they may exhibit and the bio‐psycho‐social impact of these conditions. Despite diagnostic and methodological difficulties, psychopathology and ED research studies suggest that 3–42% of institutionalized adults with ID and 1–19% of adults with ID in the community have diagnosable EDs. Weight surveys indicate that 2–35% of adults with ID are obese and 5–43% are significantly underweight, but the contribution of diagnosable EDs is unknown. Such data and case reports suggest that EDs are associated with considerable physical, behavioural, psychiatric and social comorbidity. Review papers have focused on the aetiology and treatment of pica, rumination, regurgitation, psychogenic vomiting and food faddiness/refusal. Emerging clinical issues are the development of appropriate diagnostic criteria, multimodal assessment and clinically effective treatment approaches. Key service issues include staff training to improve awareness, addressing comorbidity and access issues, and maintaining support for adults with ID and EDs, and their carers. Research should confirm the multifaceted aetiology and comorbidity of EDs. Then multicomponent assessment and treatment models for EDs can be developed and evaluated.
Experiences of staff in dealing with client sexuality in services for teenagers and adults with intellectual disability
Authors: McConkey R. , Ryan D.
Published: July 2010

Previous studies have focused on staff attitudes to sexuality rather than their experience of dealing with sexual incidents. A self‐completion questionnaire was devised in which seven scenarios were proposed relating to client sexuality (e.g. public masturbation and accusation of sexual abuse). Staff noted whether or not they had encountered this type of incident, and if so, how confident they felt (or would feel) in dealing with it, and whether or not they would enlist the help of colleagues. Questionnaires were sent to all statutory, voluntary and private service providers (including schools) within one community health and social services trust area in Northern Ireland, and 150 staff responded. Around two‐thirds of staff reported having dealt with at least one of the seven incidents listed. Staff with previous experience of the incident listed, felt that they could deal with it more confidently in future, as did staff working in residential services rather than day services. The latter group of staff were more likely to enlist the help of colleagues than were residential staff. Nearly 50% of staff identified more training and clear policy guidelines as the two means of increasing their confidence in dealing with issues of client sexuality. These results highlight the need for staff training that spans agencies and results in common approaches to client sexuality. Suggestions for further research are noted.
Neuropsychological test battery in the follow‐up of patients with juvenile neuronal ceroid lipofuscinosis
Authors: Lamminranta S. , Åberg L. E. , Autti T. , Moren R. , Laine T. , Kaukoranta J. , Santavuori P.
Published: July 2010

The aim of the present study was to develop a neuropsychological test battery for patients with juvenile neuronal ceroid lipofuscinosis (JNCL) and to study the development of cognitive functions during the first 5 years after diagnosis. Fourteen patients with JNCL entered the study. Nine patients were homozygous for the major mutation, whereas five were compound heterozygotes. All patients were studied annually with a special neuropsychological test battery (NEPSY) adapted from Luria's neuropsychological test, and modified for the visually handicapped; the Wechsler Intelligence Scale for Children – Revised (WISC‐R) was also included. The neurological examinations were scored. Furthermore, 1.OT magnetic resonance imaging scan was performed at the beginning of follow‐up and after a mean of 5 years. A decline in verbal IQ (WISC‐R) during the follow‐up period was found in all subjects except one compound heterozygous male. Short‐term memory and digit memory span were already impaired at an early stage of the disease. Orientation to time was found to decline more than orientation to person and place. Motor speed usually became impaired after 10 years of age. Spatial orientation was impaired only in the patients homozygous for the major mutation. The test battery was found to be reliable and easy to use, and offered valuable information on the progress of the disease. It also provided important guidelines for rehabilitation.
Consistency, context and confidence in judgements of affective communication in adults with profound intellectual and multiple disabilities
Authors: Hogg J. , Reeves D. , Roberts J. , Mudford O. C.
Published: July 2010

Twenty‐four service providers rated 12 video samples of four service users with whom they were familiar for affective behaviour (i.e. ‘like’/‘dislike’) and confidence (i.e. ‘certain’/’uncertain’) in their judgement. Each video sample had been recorded as part of a stimulus preference assessment during which a wide range of specific stimuli were presented to each service user. Each video sample was presented twice in a counterbalanced design either with contextual information, i.e. what the presented stimulus was (C) or without such information, i.e. context free (CF). The observers showed considerable individual variation in their judgements, largely uninfluenced by the availability or otherwise of contextual information. However, as a group, observers significantly distinguished between video samples with regard to affective communication (determined through multiple analyses of variance) and the pattern of judgements, i.e. the relative judgement of positive or negative affect, from one sample to another. This showed a good level of consistency between observers (determined through principal components analysis). The impact of contextual information was not apparent for all video samples. However, contextual information significantly influenced judgements in four samples, typically making them more extreme; for example, a response indicative of positive affect in the CF situation became more positive when contextual information was provided, indicating that the stimulus was one that the participant was thought to like.
Electrophysiology and intelligence: the electrophysiology of intellectual functions in intellectual disability
Authors: Martín‐Loeches M. , Muñoz‐Ruata J. , Martínez‐Lebrusant L. , Gómez‐Jarabo G.
Published: July 2010

The electroencephalograms (EEGs) of 127 subjects with intellectual disability were analysed with regarding to their correlation to intellectual functions in order to further understand the relationships between EEG and intelligence. The EEG frequency spectrum was subdivided into 15, 2‐Hz‐wide bands and was recorded from electrodes F1, Fpz, Fp2, F7, F3, Fz, F4, F8, T3, C3, Cz, C4, T4, T5, P3, Pz, P4, T6, O1, Oz and O2. Patterns of acorrelation showed several similarities when compared to other analogous studies with normal subjects. However, the typical finding in the present study was a high number of correlations involving the frontal lobes, mainly the prefrontal portions, which is at variance with the patterns of normal subjects. Frontal lobes, especially the prefrontal regions, seem to be affected, regardless of its aetiology, in subjects with intellectual disability.
A general practice‐based prevalence study of epilepsy among adults with intellectual disabilities and of its association with psychiatric disorder, behaviour disturbance and carer stress
Authors: Matthews T. , Weston N. , Baxter H. , Felce D. , Kerr M.
Published: July 2010

Background  Although the elevated occurrence of epilepsy in people with intellectual disabilities (ID) is well recognized, the nature of seizures and their association with psychopathology and carer strain are less clearly understood. The aims were to determine the prevalence and features of epilepsy in a community‐based population of adults with ID, and to explore whether the presence of epilepsy was associated with greater psychopathology or carer strain.

Methods  Data were collected on the age, gender, place of residence, adaptive and challenging behaviour, social abilities and psychiatric status of 318 adults from 40 general practices, together with the degree of malaise and strain of family carers. For participants with epilepsy, a nurse collected information on seizures, investigations, treatment and carer concerns by interview. Association between epilepsy and psychiatric morbidity, challenging behaviour and caregiver malaise or strain, was explored by comparing those with epilepsy with a comparison group matched on adaptive behaviour.

Results  Fifty‐eight participants (18%) had epilepsy: 26% were seizure free, but 34% had extremely poorly controlled seizures. Earlier onset and seizure frequency were associated with adaptive behaviour. Carer concerns were related to seizure frequency and a history of injury. There were no significant differences in psychopathology, carer malaise or caregiver strain between the matched epilepsy and non‐epilepsy groups.

Conclusions  This study supports the high occurrence and chronicity of epilepsy among people with ID. While psychopathology and carer strain is common within this population, underlying disability‐related factors appear to be more important than the presence of epilepsy per se.
Glucocorticoid Ultradian Rhythmicity Directs Cyclical Gene Pulsing of the Clock Gene Period 1 in Rat Hippocampus
Authors: Conway‐Campbell B. L . , Sarabdjitsingh R. A. , McKenna M. A. , Pooley J. R. , Kershaw Y. M. , Meijer O. C. , De Kloet E. R. , Lightman S. L .
Published: July 2010

In vivo glucocorticoid (GC) secretion exhibits a distinctive ultradian rhythmicity. The lipophilic hormone can rapidly diffuse into cells, although only the pulse peak is of sufficient amplitude to activate the low affinity glucocorticoid receptor (GR). Discrete pulses readily access brain regions such as the hippocampus where GR expression is enriched and known to regulate neuronal function, including memory and learning processes. In the present study, we have tested the hypothesis that GR brain targets are responsive to ultradian GC rhythmicity. We have used adrenalectomised rats replaced with pulses of corticosterone to determine the transcriptional effects of ultradian pulses in the hippocampus. Confocal microscopy confirmed that each GC pulse results in transient GR nuclear localisation in hippocampal CA1 neurones. Concomitant GR activation and DNA binding was demonstrated by synthetic glucocorticoid response element oligonucleotide binding, and verified for the Clock gene Period 1 promoter region by chromatin immunoprecipitation assays. Strikingly each GC pulse induced a ‘burst’ of transcription of Period 1 measured by heterogeneous nuclear RNA quantitative polymerase chain reaction. The net effect of pulsatile GC exposure on accumulation of the mature transcript was also assessed, revealing a plateau of mRNA levels throughout the time course of pulsatile exposure, indicating the pulse timing works optimally for steady state Per1 expression. The plateau dropped to baseline within 120 min of the final pulse, indicating a relatively short half‐life for hippocampal Per1. The significance of this strict temporal control is that any perturbation to the pulse frequency or duration would have rapid quantitative effects on the levels of Per1. This in turn could affect hippocampal function, especially circadian related memory and learning processes.
Atypical odontalgia – pathophysiology and clinical management
Authors: BAAD‐HANSEN L.
Published: July 2010

Summary  Atypical odontalgia (AO) is a chronic form of dental pain without signs of pathology. Several hypotheses have been put forward regarding the pathophysiology. AO has been proposed to be psychogenic, vascular, neuropathic or idiopathic. The scientific evidence supporting or rejecting these hypotheses are reviewed in this paper. At this time, the best supported hypothesis is that AO is a neuropathic pain condition. Relevant differential diagnoses, such as odontogenic pain, sinusitis, trigeminal neuralgia among others, are presented and the evidence regarding possible management strategies is reviewed. A treatment algorithm for AO is proposed based on the rather scarce scientific evidence available and inspired by a similar treatment algorithm for peripheral neuropathic pain. The proposed strategy involves an interdisciplinary approach including patient education, psychological counselling, topical and systemic medication and, importantly, avoidance of invasive treatments like surgery and endodontics. Two illustrative cases are presented.
Caudal epidural injection of lidocaine, tramadol, and lidocaine–tramadol for epidural anesthesia in cattle
Authors: BIGHAM A. S. , HABIBIAN S. , GHASEMIAN F. , LAYEGHI S.
Published: July 2010

Bigham, A. S., Habibian, S., Ghasemian, F., Layeghi, S. Caudal epidural injection of lidocaine, tramadol, and lidocaine–tramadol for epidural anesthesia in Cattle.J. vet. Pharmacol. Therap.33, 439–443.

Caudal epidural anesthesia is commonly utilized in veterinary medicine to allow diagnostic, obstetrical, and surgical intervention, in the perineal region of large animal. The aim of this study is to directly compare the time of onset and duration of analgesia produced by a tramadol and lidocaine–tramadol combination with that produced by lidocaine administration in the epidural space of Cattle. Five healthy adult Holstein dairy cows were selected to this study. Epidural anesthesia was produced in all cows by lidocaine, with 2 weeks intervals repeated by a combination of lidocaine–tramadol and tramadol. Time to onset and duration of analgesia were recorded. Heart rate, respiratory rate and body temperature were recorded at 0 min and at 5, 10, 15, 30, 60, and 75 min after the epidural administrations of each treatments. The tramadol produced a significant (P < 0.05) longer duration of analgesia (306.8 ± 8.58 min) than lidocaine (69.40 ± 8.96 min) alone and lidocaine–tramadol combination (174 ± 4.84 min). Also, lidocaine–tramadol combination produced a significant (P < 0.05) longer duration of analgesia than lidocaine alone. Complete analgesia began at 14.10 ± 1.57 min in the tramadol treatment, being more delayed than in the treatments with lidocaine–tramadol (4.84 ± 0.68 min) and lidocaine (3.90 ± 0.89 min). Body temperatures, heart rates, and respiratory rates were not significantly different in comparison with baseline values throughout the study in the all treatments. The combination of lidocaine–tramadol produced anesthesia of longer duration than lidocaine and the onset time was approximately same as for the lidocaine group. Utilizing this combination, long duration of anesthesia could commence relatively soon after epidural injection and might be used without re‐administration of anesthetic agent in long‐duration obstetrical and surgical procedures.
Strain‐specific immunity induced by immunization with pre‐erythrocytic stages of Plasmodium chabaudi
Authors: CULLETON R. L. , INOUE M. , REECE S. E. , CHEESMAN S. , CARTER R.
Published: July 2010

One of the most promising approaches in the efforts to produce a malaria vaccine involves the use of attenuated whole sporozoite immunizations. Attenuation may be achieved by the use of genetic modification, irradiation, chemical attenuation, or by the contemporaneous administration of antimalarial drugs that target only the erythrocytic stages of the parasite. Most research to date has focused on the efficacy of these approaches upon challenge with parasites homologous to those used for the initial immunizations. We, as have others, have previously shown that a component of the immunity achieved against the erythrocytic stages of the rodent malaria parasite Plasmodium chabaudi chabaudi is strain‐specific, with a stronger immune response targeting the immunizing strain than genetically distinct strains. Here, we show that the immunity induced by infection with the pre‐erythrocytic stages of these parasites, achieved via inoculation of sporozoites contemporaneously with mefloquine, also has a strain‐specific component.
Variables in an Assay of Soluble Human Transplantation Antigens
Authors: ETHEREDGE EDWARD E. , FRANECKI BARBARA H. , NAJARIAN JOHN S.
Published: July 2010

The data demonstrate that an assay for soluble human transplantation antigen, based on the inhibition of a two‐stage lymphocytotoxicity reaction, is (A) affected by platelet contamination of the target lymphocyte suspension; (B) more sensitive to low antigen concentration if antisera are diluted to titer using AB serum; (C) responsive to the particular antiserum‐target cell combination used; (D) relatively insensitive to the solvent of the protein solution; (E) markedly affected by the concentration of the protein solution being tested; (F) maximally sensitive to low antigen concentration with the following conditions: (1) a one‐hour incubation of antigen and antiserum at room temperature, (2) a 40‐minute incubation at room temperature for the interaction of the antigen‐antiserum and cells, and (3) incubation with complement for 20 minutes at 37° C; (G) more sensitive to rabbit complement than an equal mixture of rabbit and human complement, for the sera studied.
HL‐A Polymorphism of Norwegian Lapps
Authors: THORSBY E. , BRATLIE ANNE , TEISBERG P.
Published: July 2010

HL—A typing of 176 unrelated Norwegian Lapps revealed several differences in antigen and gene frequencies and in gametic associations as compared to non‐Lappish populations. The frequencies of HL—A1, HL—A8, and HL—A12 were very much lower and those of HL—A3, HL—A9, AA (W22), and FJH—AJ (>W27) significantly higher than in Caucasoid populations.