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Background: The clinical and neuropathological features associated with dementia in Down's syndrome (DS) are not well established. Aims: To examine clinico-pathological correlations and the incidence of cognitive decline in a cohort of adults with DS. Method: A total of 92 hospitalized persons with DS were followed up from 1985 to December 2000. At outset, 87 participants were dementia-free, with a median age of 38 years. Assessments included the Prudhoe Cognitive Function Test (PCFT) and the Adaptive Behavior Scale (ABS), to measure cognitive and behavioural deterioration. Dementia was diagnosed from case records and caregivers' reports. Results: Eighteen (21%) patients developed dementia during follow-up, with a median age of onset 55.5 years (range 45-74). The PCFT demonstrated cognitive decline among those with a less severe intellectual disability (mild and moderate) but not among the profoundly disabled people (severe and profound). Clinical dementia was associated with neuropathological features of Alzheimer's disease, and correlated with neocortical neurofibrillary tangle densities. At the age of 60 years and above, a little more than 50% of patients still alive had clinical evidence of dementia. Conclusions: Clinical dementia associated with measurable cognitive and functional decline is frequent in people with DS after middle age, and can be readily diagnosed among less severely intellectually disabled persons using measures of cognitive function such as the PCFT and behavioural scales such as the ABS. In the more profoundly disabled people, the diagnosis of dementia is facilitated by the use of behavioural and neurological criteria. In this study, the largest prospective DS series including neuropathology on deceased patients, the density of neurofibrillary tangles related more closely to the dementia of DS than senile plaques. In people with DS surviving to middle and old age, the development of dementia of Alzheimer type is frequent but not inevitable, and some people with DS reach old age without clinical features of dementia.

Background: Inpatient beds for the psychiatric assessment and treatment of young people (under the age of 18 years) with intellectual disability have become scarce although there is pressure to redevelop them. In the UK, 63% of the NHS beds are at Prudhoe Hospital. This study examines their role and utility in relation to community services, both local and national. Method: A case note study of the 96 young people admitted over three years was supplemented by a standardised interview canvassing the opinion of the staff involved, both within the units and in the community. Results: Two groups emerged: those with more severe disability who were admitted for neuropsychiatric management (63%) and those, predominantly adolescent, whose problems were closer to mainstream psychiatry but whose intellectual disability prevented their needs being met adequately by mainstream services (37%). Admissions were equally divided between those from the immediate vicinity and nationally. For 43% the primary aim was to disentangle the extent of the environmental effect on the individual: the remainder were admitted for various reasons including, for example, the need to protect the individual from harming themselves or others, unusually complex or hazardous treatment or the carers inability to cope with the treatment plan. All admissions were thought appropriate but, had the community resources been better, 18% were avoidable. Conclusions: Admission is necessary for a small number of young people who are not readily managed within mainstream units. While the number may be reduced by better community provision, this is likely to partially offset by better case finding. Inpatient facilities should be closely linked with community services; a point reinforced by the prolongation of admissions because a shortfall in community resources.

Although earlier claims to identify specific compounds in the urine of people with autism had been discredited, it was subsequently suggested that there might be biochemical characteristics that were specific to early childhood, particularly in those who also did not have a severe degree of intellectual disability This study was to establish whether autism might have a distinctive chromatographic profile on urinary analysis. Thirty-four prepubertal boys with autism were matched with two groups of boys without autism one on ability and chronological age and the other on chronological age alone, being within the normal range of ability. Laboratory analysis of their urine samples was carried out blind as to the clinical diagnosis. The analysis correctly identified 53% of the autism group as against misidentifying 33% and 18% of the other two groups. When children with a severe learning disability (both with and without autism) were excluded from the comparisons, the laboratory then identified 77% of the 13 boys left in the autism group and misidentified 8% and 18% of the other two groups. The results would support the idea of a biological marker in prepubertal children and that it may be absent in, or obscured by the presence of severe LD.

Although earlier claims to identify specific compounds in the urine of people with autism had been discredited, it was subsequently suggested that there might be biochemical characteristics that were specific to early childhood, particularly in those who also did not have a severe degree of intellectual disability This study was to establish whether autism might have a distinctive chromatographic profile on urinary analysis. Thirty-four prepubertal boys with autism were matched with two groups of boys without autism – one on ability and chronological age and the other on chronological age alone, being within the normal range of ability. Laboratory analysis of their urine samples was carried out blind as to the clinical diagnosis. The analysis correctly identified 53% of the autism group as against misidentifying 33% and 18% of the other two groups. When children with a severe learning disability (both with and without autism) were excluded from the comparisons, the laboratory then identified 77% of the 13 boys left in the autism group and misidentified 8% and 18% of the other two groups. The results would support the idea of a biological marker in prepubertal children and that it may be absent in, or obscured by the presence of severe LD. [ABSTRACT FROM AUTHOR]

Abstract The present authors conducted a study of the occurrence of victimization and the perpetration of sexual abuse among 43 in-patients with intellectual disability aged between 9 and 21 years who were admitted to a child and adolescent psychiatric in-patient department over a period of 5 years. A retrospective case-note review was employed that explored the nature and severity of abuse in relation to the age, gender and level of disability. The prevalence of abuse or abusive behaviour, i.e. 14% of 300 admissions, did not change over time. In 13 out of the 43 cases, the issue of sexual abuse was identified after admission. Victimization alone occurred in 21 cases, perpetration alone in six cases, and both victimization and perpetration in 16 cases. Fifty per cent of the victims had been abused by a member of their close or extended family. Most cases (62%) were adolescents. There was only one instance of a victim being abused by a female. However, there were five girls who were perpetrators, all of whom had previously been victims. By contrast, 11 out of the 17 male perpetrators had been victims. Despite difficulties of disclosure, it was possible to establish that severely disabled patients had suffered sexual abuse. The present data support theories which (1) recognize gender differences in sexual abuse patterns and (2) have a developmental perspective, incorporating the influence of adolescence. [ABSTRACT FROM AUTHOR]