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Biomedical subjects

O Udwin

Publications and source records attributed to O Udwin.

At least 19 recordsLinked to original sources
PubMed2006

Outcome in adult life for people with Williams syndrome-- results from a survey of 239 families.

BACKGROUND: Although there has been considerable research into the genotype and phenotype of Williams syndrome, there have been relatively few studies of long-term prognosis. As a preliminary to a more detailed investigation of adults with Williams syndrome, a parental questionnaire was distributed to members of the UK Williams Syndrome Foundation, focusing on areas of skill and difficulties in adult life. METHOD: A postal questionnaire was distributed to all members of the UK Williams Syndrome Foundation who had a son or daughter aged 18 years or over. The questionnaire covered issues related to the physical and mental health of the adults with Williams syndrome, together with questions about educational and employment status, self-help and independence skills. RESULTS: Out of a total of 290 questionnaires distributed, 239 were returned (82% response rate). The data highlighted the continuing high rates of physical problems in this group and apparent increases in rates of mental health problems with age. Parents also expressed their concerns about the lack of adequate support and care. Educational and employment attainments were generally low and self-help skills were relatively poor. DISCUSSION: The findings highlight the need for far better understanding of conditions such as Williams syndrome among professionals working in adult services and the need for more adequate support from educational, health and social services.

Activities of Daily Living↗
PubMed2001

Abilities and attainment in Smith-Magenis syndrome.

This study reports on cognitive abilities and attainment in 29 children and 21 adults with Smith-Magenis syndrome. There were 13 boys and 16 girls aged 6 to 16 years, and nine men and 12 women aged 16 to 52 years. All had mild to severe learning disabilities* with no differences overall between verbal and performance skills, but with a particular profile of cognitive strengths and weaknesses. Levels of attainment and of adaptive behaviour were strikingly low, and the group of adults emerged as much more dependent on carers than might have been expected from their general level of intellectual functioning. Reasons for this discrepancy are explored in terms of the severe behavioural difficulties characteristic of the syndrome.

Abnormalities, Multiple↗
PubMed2000

The long-term psychological effects of a disaster experienced in adolescence: I: The incidence and course of PTSD.

Previous studies have shown that children and adolescents exposed to traumatic experience in a disaster can suffer from high levels of post-traumatic stress. The present paper is the first a series reporting on the long-term follow-up of a group of young adults who as teenagers had survived a shipping disaster-the sinking of the "Jupiter" in Greek waters-between 5 and 8 years previously. The general methodology of the follow-up study as a whole is described, and the incidence and long-term course of Post-Traumatic Stress Disorder (PTSD). It is the first study of its kind on a relatively large, representative sample of survivors, using a standardised diagnostic interview, and comparing survivors with a community control group. Survivors of the Jupiter disaster (N = 217), and 87 young people as controls, were interviewed using the Clinician Administered PTSD Scale (CAPS). Of the 217 survivors, 111 (51.7%) had developed PTSD at some time during the follow-up period, compared with an incidence in the control group of 3.4 % (N = 87). In the large majority of cases of PTSD in the survivors for whom time of onset was recorded, 90 % (N = 110), onset was not delayed, being within 6 months of the disaster. About a third of those survivors who developed PTSD (30%, N = 111) recovered within a year of onset, through another third (34 %, N = 111) were still suffering from the disorder at the time of follow-up, between 5 and 8 years after the disaster. Issues relating to the generalisability of these findings are discussed.

Adolescent↗
PubMed2000

The long-term psychological effects of a disaster experienced in adolescence: II: General psychopathology.

Children and adolescents exposed to trauma can suffer major adverse psychological effects including not only post-traumatic stress but also other psychological disorders. This study investigates the long-term course of general psychopathology following trauma in adolescence using a standardised diagnostic interview and comparisons with a matched control group. Young people (N= 216) who as teenagers had survived a shipping disaster-the sinking of the "Jupiter" in Greek waters-between 5 and 8 years previously and 87 young people as matched controls were interviewed. The survivors showed raised rates of diagnosis in a range of anxiety and affective disorders during the follow-up period. The highest rates were among the survivors who had developed Post-Traumatic Stress Disorder (PTSD), and those survivors who had not were generally similar to the controls. Onset of anxiety and affective disorders varied between being indefinitely close to the disaster to years later. Differences in rates of disorder between the survivor and control groups had lessened by the time of follow-up but were still apparent, due to continuing distress among the survivors still suffering from PTSD, and to a lesser extent among those who had recovered from PTSD. Generalisability of the findings are discussed.

Adolescent↗
PubMed2000

Risk factors for long-term psychological effects of a disaster experienced in adolescence: predictors of post traumatic stress disorder.

This paper examines risk factors for the development of Post Traumatic Stress Disorder (PTSD), and its severity and chronicity, in a group of 217 young adults who survived a shipping disaster in adolescence. The survivors were followed up 5 to 8 years after the disaster. Risk factors examined fell into three main categories: pre-disaster child and family vulnerability factors, including childhood psychopathology; objective and subjective disaster-related experiences; and post-disaster factors, including results from screening questionnaires administered 5 months post-disaster, coping mechanisms adopted subsequently, life events, and availability of social supports. Developing PTSD following the disaster was significantly associated with being female, with pre-disaster factors of learning and psychological difficulties in the child and violence in the home, with severity of exposure to the disaster, survivors' subjective appraisal of the experience, adjustment in the early post-disaster period, and life events and social supports subsequently. When all these factors were considered together, measures of the degree of exposure to the disaster and of subjective appraisal of life threat, and ratings of anxiety obtained 5 months post-disaster, best predicted whether survivors developed PTSD. For those survivors who developed PTSD, its duration and severity were best predicted not by objective and subjective disaster-related factors, but by pre-disaster vulnerability factors of social, physical, and psychological difficulties in childhood together with ratings of depression obtained 5 months post-disaster, and whether survivors received post-disaster support at school. The implications of these findings are considered for targeting assessment and intervention efforts at survivors most at risk of developing difficulties in adjustment following similar traumatic experiences.

Adolescent↗
PubMed1998

Linguistic dissociations in Williams syndrome: evaluating receptive syntax in on-line and off-line tasks.

Williams syndrome (WS) is a neurodevelopmental disorder of genetic origin which results in relatively spared language in the face of serious non-verbal deficits. There is controversy, however, about how intact WS language abilities are. The discussion has focused on impairments of lexico-semantics and of morphological feature analysis, with the presumption that WS syntax is intact. We challenged this view and assessed WS receptive syntax by using two tasks testing various syntactic structures: an on-line word monitoring task and an off-line picture-pointing task. WS performance on the off-line task was generally poor. By contrast, their performance on the on-line task was far better and allowed us to ascertain precisely which aspects of WS receptive syntax are preserved and which are impaired. WS participants were sensitive to the violation of auxiliary markers and phrase structure rules but, unlike both the normal young and elderly controls, they did not show sensitivity to violations of subcategory constraints. The present study suggests that there exist dissociations within WS language which are not restricted to lexico-semantics or to morphological feature analysis, but which also invade their processing of certain syntactic structures. We conclude by arguing that WS syntax is not intact and that their language might turn out to be more like second language learning than normal acquisition.

Adolescent↗
PubMed1998

Community care for adults with Williams syndrome: how families cope and the availability of support networks.

A study of the adjustment difficulties and needs of 70 adults with Williams syndrome found that the majority continued to live at home and remained heavily dependent on their families for their self-care. Twenty-nine families (41.4%) had had no contact with a social worker in the preceding 2 years, and 34 out of the 48 families whose children still lived at home (70.8%) had no access to respite care. Advice regarding benefits, and appropriate living and occupational arrangements for the adults was also patchy. Despite progressive medical problems, and high rates of behavioural and emotional difficulties, only 20 adults (29%) were receiving regular health checks, while 21 (30%) had had some contact with a mental health service in the preceding 2 years. In the majority of cases, families continued to shoulder the main burden of care for their sons and daughters with Williams syndrome well into adulthood, with little support from statutory and voluntary agencies. The implications of these findings are considered with regard to the principles of community care.

Adaptation, Psychological↗
PubMed1998

Adults with Williams syndrome. Preliminary study of social, emotional and behavioural difficulties.

BACKGROUND: In recent years there has been an upsurge of interest in the study of distinctive patterns of behavioural and psychological characteristics associated with specific, biologically determined, intellectually disabling conditions. This study investigates whether such a profile can be identified in adults with Williams syndrome. METHOD: Parents and other care-givers were interviewed about the social, emotional and behavioural characteristics of 70 adults with Williams syndrome, aged 19 years to 39 years 9 months. RESULTS: The adults were reported to have high rates of behavioural and emotional difficulties, particularly in terms of poor social relationships, over-friendliness and social disinhibition, preoccupations and obsessions, and high levels of anxiety and distractibility. CONCLUSIONS: The findings provide preliminary support for the existence of a specific pattern of behavioural and personality characteristics and associated difficulties in adults with Williams syndrome, which persist from childhood and often require intervention from mental health professionals. Implications for clinical practice are considered.

Adolescent↗
PubMed1998

Cognitive functioning in adults with Williams syndrome.

This paper reports the results of cognitive, linguistic, and academic assessments in a representative sample of 62 adults with Williams syndrome. The average age of the group was 26 years and their mean full scale IQ was 61. Differences between Verbal and Performance IQ, and between receptive and expressive language skills, were smaller than generally found in studies of children with this condition. However, an examination of subtest scores revealed an almost identical cognitive profile to that found in children. Skills in other areas, such as reading, spelling, arithmetic, and social adaptation remained at a low level, with functioning generally being around a 6-8-year age equivalent. The consistency of reports on intellectual abilities in both child and adult studies of individuals with Williams syndrome lends increased support to the notion of a syndrome specific pattern of cognitive, linguistic, and adaptive functioning.

Adolescent↗
PubMed1997

Independence and adaptive behavior in adults with Williams syndrome.

This study describes the adjustment of 70 adults with Williams syndrome, in terms of self-help skills, independence, and occupational levels. Although the overall mean IQ of the group (62.00) was within the mild mental handicap range, relatively few individuals were able to attain a high level of independence or cope with the demands of employment. Adaptive behavior scores were significantly below chronological age. Outcome measures were compared with available data on other groups of adults of similar age and level of intellectual impairment. Implications for the community care of adults with Williams syndrome are discussed.

Activities of Daily Living↗
PubMed1997

Do individuals with Williams syndrome have bizarre semantics? Evidence for lexical organization using an on-line task.

Williams syndrome, a neurodevelopmental disorder, has attracted a great deal of debate concerning the purported intactness of language in the face of other serious cognitive deficits. As more in-depth studies of specific aspects of WS language have emerged, the notion of a preserved language module has been seriously challenged. Although WS vocabulary scores are often impressive, several investigators have claimed the WS semantics are aberrant. All studies hitherto have been based on off-line experiments which necessarily involve metalinguistic processes. This clearly affects the performance of individuals with cognitive deficits. We report here an on-line study probing the semantic structure of the WS lexicon, using a task-semantic priming-which minimises metalinguistic demands. We show that WS subjects display the same taxonomic/category and thematic/functional priming effects as normal controls. The results are discussed in terms of the differences between receptive and expressive language, as well as the fact that although semantic memory and the automatic access to semantic information for individual words is normal in WS, the integration of semantic information into sentence comprehension may be abnormal. The importance of online tasks to highlight such differences is stressed.

Adolescent↗
PubMed1997

Language and Williams syndrome: how intact is "intact"?

It has been claimed that Williams syndrome (WS), a rare neurodevelopmental disorder, is characterized by serious cognitive deficits alongside intact language. The syndrome is often used as a prime example of the modularity of an innate faculty for morphosyntactic rules. We challenge this claim and hypothesize that morphosyntax, although surprisingly good given WS level of mental retardation, is by no means intact. We make an initial test of this hypothesis through an analysis of the receptive language of a group of English-speaking WS individuals on a standardized morphosyntactic test. We then present an experimental study of expressive language that examines grammatical gender assignment in French-speaking WS patients. Despite a Verbal Mental Age selected to be higher than the chronological age of the young control group, these people with WS continue even in adulthood to show clear-cut deficits in their production of an aspect of morphosyntax that normal children acquire effortlessly very early. The results of the 2 studies, one focusing on receptive language and the other on expressive language, challenge the notion that comprehension and use of morphosyntactic rules in WS individuals are intact. The Within-domain dissociations regarding the use of grammatical gender assignment across several sentence clements and their difficulties in understanding embedded sentences-two quintessentially linguistic skills-suggest that we must rethink the notion of spared, modular, language capacities in Williams syndrome. We conclude that WS language follows a different path to normal acquisition and may turn out to be more like second language learning.

Adolescent↗
PubMed1996

A longitudinal study of cognitive abilities and educational attainment in Williams syndrome.

The paper reports on changes in IQ scores and in reading, spelling and arithmetic skills over time in 23 young adults with Williams syndrome. They were first assessed in their early to mid-teens and followed up 8 to 9 years later, at an average age of 21 years 9 months. Cognitive assessments indicated increases in Full Scale, Verbal and Performance IQ scores. These increases allow us to conclude that in the case of Williams syndrome (unlike some other conditions) there does not appear to be a decline in the rate of cognitive development over time. Comparisons of Reading, Spelling and Arithmetic scores attained at first and second testing periods revealed only modest increases in reading accuracy and spelling scores, a slight decline in reading comprehension scores, and little change in arithmetic test scores. Differences in the tests used at the two assessment periods do not allow for definitive conclusions to be drawn, but the findings suggest that individuals with Williams syndrome make little progress in their educational skills beyond their early teenage years.

Adolescent↗
PubMed1991

A cognitive and behavioural phenotype in Williams syndrome.

Comparisons between children with Williams syndrome and control group children matched for age, sex, social class, and verbal intelligence provide support for a cognitive and behavioural phenotype in Williams syndrome. Children with the syndrome showed higher rates of behavioural and emotional difficulties when compared with the control group children, particularly in terms of concentration difficulties, excessive anxiety, and poor relationships with peers; and they also had significantly poorer visuo-spatial and motor skills. However, the Williams syndrome children were not uniformly poor in all areas of nonverbal abilities. Their visual recall skills were as good as those of the control group children, and their performance was superior to that of the control group children on a task of face recognition and on tasks requiring recall of verbal material.

Abnormalities, Multiple↗
PubMed1991

Screening child survivors for post-traumatic stress disorders: experiences from the 'Jupiter' sinking.

Twenty-four girls who survived the sinking of the 'Jupiter' in October 1988 were screened 10 days after the accident on the Impact of Events Scale, the Birleson Depression Scale and the Revised Children's Manifest Anxiety Scale. Scores at 10 days were associated strongly with help seeking over the following few months and were highly predictive of scores five months after the accident. Survivors were clearly differentiated from controls on all three measures. The battery seems useful in screening post-traumatic stress disorders in teenage children.

Adolescent↗
PubMed1991

Augmentative communication systems taught to cerebral-palsied children--a longitudinal study. II. Pragmatic features of sign and symbol use.

Pragmatic analysis of the sign and symbol utterances produced by 40 language-impaired, cerebral-palsied children in semi-structured conversational settings revealed severe restrictions in the range of communicative functions that were used. The children were able to communicate certain conversational acts effectively, but over 80% of all utterances expressed just four communicative functions. There were also some significant gaps, with certain functions not being used at all. The children's progress was followed up at 6-month intervals over a period of 1 1/2 years. Few changes were found in the range and relative frequencies of communicative functions expressed over time. There were few significant differences between the symbol and sign users on these measures, which suggests that overall neither augmentative mode facilitated greater communicative use than the other.

Cerebral Palsy↗
PubMed1991

Augmentative communication systems taught to cerebral-palsied children--a longitudinal study. III. Teaching practices and exposure to sign and symbol use in schools and homes.

A longitudinal study of augmentative communication training with 40 language-impaired, cerebral-palsied children examined the teaching practices adopted in schools, and the extent to which teachers and parents used the augmentative systems with the children. The study found limited exposure to sign and symbol training in formal teaching sessions and in other school settings, and teachers made relatively few attempts to foster spontaneous use of the systems and generalisation outside of formal training settings. Exposure to augmentative communication in the children's homes was equally limited, and a significant number of parents expressed reservations about the value of the systems for their children. These findings may at least partly account for the children's limited progress in sign and symbol acquisition and use over an 18-month period, which was documented in two earlier reports.

Attitude↗