Factors associated with acute care service use after epilepsy hospitalisation in people with intellectual disability.

BACKGROUND: This study aimed to identify factors associated with unplanned acute hospital readmission and emergency department (ED) presentation after hospitalisation for epilepsy in people with intellectual disability (ID). METHODS: This study is a retrospective cohort study using linked administrative datasets. We identified 3293 people with ID aged 5-64 years with a hospitalisation for epilepsy between 2005 and 2014 in New South Wales, Australia. We examined unplanned readmission and ED presentation within 30 or 365 days and associations with demographic, socio-economic and health status variables. Modified Poisson regression with robust estimation was used to model outcomes within 30 days. Negative binomial regression was used to account for the overdispersion of the data and to model 365-day outcome rates. RESULTS: Around half of the cohort had an unplanned readmission and ED presentation within 365 days of the index hospitalisation. In fully adjusted models, being female, being a young adult and having a longer or acute care index admission, mental and physical comorbidities and a history of incarceration were associated with an elevated risk of readmission or ED presentation. The strongest association was observed between history of self-harm and 365-day readmission (incidence rate ratio 2.15, 95% confidence interval 1.41-3.29). CONCLUSIONS: Socio-demographic, justice and health factors are associated with unplanned readmission and ED presentation risk after hospitalisation for epilepsy in people with ID. Interventions targeting improving continuity of care should be tailored for individuals and their support workers. The findings also emphasise the importance of person-centred multidisciplinary care across different health sectors.

Reviewing causes of death of individuals with intellectual disability in New South Wales, Australia: a record-linkage study.

BACKGROUND: People with intellectual disability (ID) have a much higher mortality rate than the general population. To reduce the rate of mortality of people with ID, it is critical that causes of death are properly understood, recorded and reported. Formal reviews of causes of death are used in some countries to ensure that causes of death are accurate. To date, the impact of these formal reviews on understanding causes of death of people with ID has not been quantified. METHODS: The study aimed to quantify the impact of formal reviews of deaths on the understanding of causes of death of people with ID who died while living in residential care. Individuals (851) with ID who died in residential care in New South Wales (NSW), Australia, between 1 December 2002 and 31 December 2013, who had a cause of death recorded in both the NSW Cause of Death Unit Record File (COD-URF; cause of death recorded at time of death) and NSW Ombudsman dataset (cause of death recorded after in-depth review) were included in the study. We assessed agreement in coding for cause of death by comparing the International Classification of Diseases 10th Revision (ICD-10) codings at three levels of diagnostic specificity, for both underlying and additional causes of death. We conducted our analysis through both descriptive comparison and through two boosted regression trees. RESULTS: Approximately half of the underlying causes of death were different after review by the NSW Ombudsman compared with the COD-URF. Certain causes of death (determined by ICD-10 chapter) were less likely to predict matches between the dataset than others, with individuals with mental, behavioural and neurodevelopmental disorders recorded in the COD-URF least likely to have a matching cause of death in NSW Ombudsman dataset. For deaths where there was no agreement at any level between the datasets, a high level of unknown causes of death was recorded. CONCLUSIONS: Formal review of deaths of people with ID in residential care is important to determining true causes of death and therefore developing appropriate health policy for people with ID.

Using linked administrative data to determine the prevalence of intellectual disability in adult prison in New South Wales, Australia.

BACKGROUND: Studies of the representation of people with intellectual disability (ID) in custody report widely inconsistent findings that reflect variation in how ID is defined and the methods employed for identification. Using linked administrative data may be of utility in studies of the representation of people with ID in custody. However, this approach requires an understanding of the purpose of and factors influencing identification in disparate administrative datasets. METHODS: This study uses linked administrative data encompassing disability, health and corrections data for the year 2014 to estimate the prevalence of ID in adult custody and explore how ID representation within administrative data impacts prevalence estimates and what patterns of identification reveal about support service access for this group. RESULTS: This study finds that 4.3% of the New South Wales adult custody population had an identified ID. Prisoners with ID were younger, more likely to have had a previous custodial episode and more likely to be Indigenous than the general prison population. Identification of ID across linked administrative datasets is uneven, which, if used in isolation, would result in variation in prevalence estimates according to source data. CONCLUSIONS: The utilisation of linkage data from a broad range of health and support services including custody offers a comprehensive identification methodology. Inconsistency in the identification of ID across datasets indicates a potential disjuncture between prisoners with ID and support services, which may have relevance for efforts to reduce reincarceration of those in this population.

Survival of children and adolescents with intellectual disability following gastrostomy insertion.

BACKGROUND: Positive health outcomes have been observed following gastrostomy insertion in children with intellectual disability, which is being increasingly used at younger ages to improve nutritional intake. This study investigated the effect of gastrostomy insertion on survival of children with severe intellectual disability. METHODS: We used linked disability and health data of children and adolescents who were born in Western Australia between 1983 and 2009 to compare survival of individuals with severe intellectual disability by exposure to gastrostomy status. For those born in 2000-2009, we employed propensity score matching to adjust for confounding by indication. Effect of gastrostomy insertion on survival was compared by pertinent health and sociodemographic risk factors. RESULTS: Compared with children born in the 1980s-1990s, probability of survival following first gastrostomy insertion for those born in 2000-2009 was higher (2 years: 94% vs. 83%). Mortality risk was higher in cases than that in their matched controls (hazard ratio 2.9, 95% confidence interval 1.1, 7.3). The relative risk of mortality (gastrostomy vs. non-gastrostomy) may have differed by sex, birthweight and time at first gastrostomy insertion. Respiratory conditions were a common immediate or underlying cause of death among all children, particularly among those undergoing gastrostomy insertion. CONCLUSIONS: Whilst gastrostomy insertion was associated with lower survival rates than children without gastrostomy, survival improved with time, and gastrostomy afforded some protection for the more vulnerable groups, and earlier use appears beneficial to survival. Specific clinical data that may be used to prioritise the need for gastrostomy insertion may be responsible for the survival differences observed.

Genetic and Environmental Influences on Language Ability in Older Adults: Findings from the Older Australian Twins Study.

We used a sub-sample from the Older Australian Twins Study to estimate the heritability of performance on three tests of language ability: Boston Naming Test (BNT), Letter/Phonemic Fluency (FAS) and Category/Semantic Fluency (CFT) Tests. After adjusting for age, sex, education, mood, and global cognition (GC), heritability estimates obtained for the three tests were 0.35, 0.59, and 0.20, respectively. Multivariate analyses showed that the genetic correlation were high for BNT and CFT (0.61), but low for BNT and FAS (0.17), and for FAS and CFT (0.28). Genetic modelling with Cholesky decomposition indicated that the covariation between the three measures could be explained by a common genetic factor. Environmental correlations between the language ability measures were low, and there were considerable specific environmental influences for each measure. Future longitudinal studies with language performance and neuroimaging data can further our understanding of genetic and environmental factors involved in the process of cognitive aging.

Primary health care for people with an intellectual disability: an exploration of consultations, problems identified, and their management in Australia.

BACKGROUND: People with an intellectual disability (ID) have more complex and different patterns of health care needs than the general population. They experience a greater burden of multi-morbidity, high levels of undetected and unmanaged health issues, and premature mortality than the general population. Primary care has a key role in the health care of people with an ID. Currently, very little is known about the consultation type and length, problems managed, and how general practitioners (GPs) manage these problems for people with an ID compared with the general population. This information would provide valuable insights into how GPs are achieving the health guidelines and facilitating people with an ID to achieve the highest attainable standard of health. METHODS: A secondary analysis of data was collected from January 2003 to December 2012 from the Bettering the Evaluation and Care of Health (BEACH) programme. Consultation type, consultation length in minutes, problem(s) managed during the consultation, medications, treatments provided, and referrals made, pre and post age-sex standardisation, at all GP encounters with people identified in the encounter record as having an ID ('ID' encounters, n = 690) were compared with those at 'non-ID' encounters (n = 970 641). Statistical significance was tested with 95% confidence intervals. RESULTS: This study identified significant differences in consultation types, consultation length, problem(s) managed during the consultation, medications, treatments provided, and referrals made at 'ID' encounters compared with 'non-ID' encounters. 'ID' encounters had more indirect encounters, longer consultations, more problems managed, but an under management of common health conditions in people with an ID. Administrative rather than medically related actions dominated clinical treatments for people at 'ID' encounters, and they received fewer procedural treatments, referrals to specialists, and medications compared with those at 'non-ID' encounters. CONCLUSION: The significant differences in consultations, problems identified and managed suggest that GPs may require additional support to (1) identify and manage common medical conditions experienced by people with an ID; (2) manage the increased time required for consultations; and (3) directly consult with people with an ID. Further research is required to determine why GPs managed problems in a significantly different way for people with an ID.

Primary health care for people with an intellectual disability: an exploration of demographic characteristics and reasons for encounters from the BEACH programme.

BACKGROUND: People with an intellectual disability (ID) have complex and different patterns of healthcare needs. Poor participation in primary health care contributes to the high levels of undetected and unmanaged health issues and premature deaths of people with an ID. Limited research is available on the characteristics of people with an ID, their reasons for consulting general practitioners (GPs), and if these differ to people without an ID. Gaining such insights may provide an avenue to better understand patterns of primary care use and potential gaps in usage by people with an ID given their complex health profile compared with people without an ID. METHOD: A secondary analysis of data collected January 2003 to December 2012 from The Bettering the Evaluation and Care of Health programme was used. Participant characteristics and their reasons for encounter, pre- and post-age-sex standardisation, at all GP encounters with people identified in the encounter record as having an ID ('ID' encounters, n = 690) were compared with those at 'non-ID' encounters (n = 970 641). Statistical significance was tested with chi-squared statistics or 95% confidence intervals as appropriate. RESULTS: This study identified significant differences in participant characteristics and their reasons for consulting GPs at ID encounters compared with non-ID encounters. Participants at ID encounters had a skewed demography, an over-representation of presentations for psychological, social and 'general and unspecified' reasons, and an under-representation of presentations for core physical health and preventive health measures. Administrative rather than medically related reasons dominated presentations to general practice at ID encounters. CONCLUSION: There are significant differences in the characteristics of participants and their reasons for presentation to general practice in Australia for participants at ID encounters compared with non-ID encounters. This work suggests that there is a difference in service use patterns between these two groups. These findings may suggest that people with an ID experience barriers to participating in essential primary healthcare services.

Preliminary evidence of an effect of cerebellar volume on postural sway in FMR1 premutation males.

Recent evidence suggests that early changes in postural control may be discernible among females with premutation expansions (55-200 CGG repeats) of the fragile X mental retardation 1 (FMR1) gene at risk of developing fragile X-associated tremor ataxia syndrome (FXTAS). Cerebellar dysfunction is well described in males and females with FXTAS, yet the interrelationships between cerebellar volume, CGG repeat length, FMR1 messenger RNA (mRNA) levels and changes in postural control remain unknown. This study examined postural sway during standing in a cohort of 22 males with the FMR1 premutation (ages 26-80) and 24 matched controls (ages 26-77). The influence of cerebellar volume, CGG repeat length and FMR1 mRNA levels on postural sway was explored using multiple linear regression. The results provide preliminary evidence that increasing CGG repeat length and decreasing cerebellar volume were associated with greater postural sway among premutation males. The relationship between CGG repeat length and postural sway was mediated by a negative association between CGG repeat size and cerebellar volume. While FMR1 mRNA levels were significantly elevated in the premutation group and correlated with CGG repeat length, FMR1 mRNA levels were not significantly associated with postural sway scores. These findings show for the first time that greater postural sway among males with the FMR1 premutation may reflect CGG repeat-mediated disruption in vulnerable cerebellar circuits implicated in postural control. However, longitudinal studies in larger samples are required to confirm whether the relationships between cerebellar volume, CGG repeat length and postural sway indicate greater risk for neurological decline.

Understanding the neuropsychiatric phenotype of fragile X-associated tremor ataxia syndrome: a systematic review.

Fragile X-associated tremor ataxia syndrome (FXTAS) is a recently identified X-linked neurodegenerative disorder affecting a proportion of premutation carriers of the Fragile X Mental Retardation 1 (FMR1) gene. Previous research suggests that cognitive and psychiatric features of FXTAS may include primary impairments in executive function and increased vulnerability to mood and anxiety disorders. A number of these reports, however, are based on overlapping cohorts or have produced inconsistent findings. A systematic review was therefore conducted to further elucidate the neuropsychiatric features characteristic of FXTAS. Fourteen papers met inclusion criteria for the review and were considered to represent nine independent FXTAS cohorts. Findings from the review suggest that the neuropsychiatric phenotype of FXTAS is characterised primarily by poorer performance on measures of executive function, working memory, information processing speed, and fine motor control when compared to matched comparison groups. Two studies were identified in which psychiatric symptoms in FXTAS were compared with controls, and these yielded mixed results. Overall the results of this review support previous reports that the neuropsychiatric profile of FXTAS is consistent with a dysexecutive fronto-subcortical syndrome. However, additional controlled studies are required to progress our understanding of FXTAS and how the neuropsychiatric profile relates to underlying pathological mechanisms.

Symbolic sequence learning is associated with cognitive-affective profiles in female FMR1 premutation carriers.

This study examines implicit sequence learning impairments that may indicate at-risk cerebellar profiles proposed to underlie some aspects of subtle cognitive and affective dysfunctions found among female fragile X mental retardation 1 (FMR1) premutation (PM)-carriers. A total of 34 female PM-carriers and 33 age- and intelligence-matched controls completed an implicit symbolically primed serial reaction time task (SRTT) previously shown to be sensitive to cerebellar involvement. Implicit learning scores indicated a preservation of learning in both groups; however, PM-carriers demonstrated poorer learning through significantly elevated response latencies overall and at each specific block within the symbolic SRTT. Group comparisons also revealed a core deficit in response inhibition, alongside elevated inattentive symptoms in female PM-carriers. Finally, strong and significant associations were observed between poor symbolic SRTT performance and executive, visuospatial and affective deficits in the PM-carrier group. These associations remained strong even after controlling motor speed, and were not observed in age- and intelligence quotient-matched participants. The findings implicate cerebellar non-motor networks subserving the implicit sequencing of responses in cognitive-affective phenotypes previously observed in female PM-carriers. We contend that symbolic SRTT performance may offer clinical utility in future pharmaceutical interventions in female PM-carriers.

Impairment in instrumental activities of daily living with high cognitive demand is an early marker of mild cognitive impairment: the Sydney memory and ageing study.

BACKGROUND: Criteria for mild cognitive impairment (MCI) consider impairment in instrumental activities of daily living (IADL) as exclusionary, but cross-sectional studies suggest that some high-level functional deficits are present in MCI. This longitudinal study examines informant-rated IADL in MCI, compared with cognitively normal (CN) older individuals, and explores whether functional abilities, particularly those with high cognitive demand, are predictors of MCI and dementia over a 2-year period in individuals who were CN at baseline. METHOD: A sample of 602 non-demented community dwelling individuals (375 CN and 227 with MCI) aged 70-90 years underwent baseline and 24-month assessments that included cognitive and medical assessments and an interview with a knowledgeable informant on functional abilities with the Bayer Activities of Daily Living Scale. RESULTS: Significantly more deficits in informant-reported IADL with high cognitive demand were present in MCI compared with CN individuals at baseline and 2-year follow-up. Functional ability in CN individuals at baseline, particularly in activities with high cognitive demand, predicted MCI and dementia at follow-up. Difficulties with highly cognitively demanding activities specifically predicted amnestic MCI but not non-amnestic MCI whereas those with low cognitive demand did not predict MCI or dementia. Age, depressive symptoms, cardiovascular risk factors and the sex of the informant did not contribute to the prediction. CONCLUSIONS: IADL are affected in individuals with MCI, and IADL with a high cognitive demand show impairment predating the diagnosis of MCI. Subtle cognitive impairment is therefore likely to be a major hidden burden in society.

Prevalence and characteristics of depression in mild cognitive impairment: the Sydney Memory and Ageing Study.

OBJECTIVE: Depression might be a risk factor for dementia. However, little is known about the prevalence of depressive symptoms in mild cognitive impairment (MCI) and whether mood or motivation-related symptoms are predominant. METHOD: A total of 767 non-demented community-dwelling adults aged 70-90 years completed a comprehensive assessment, including neuropsychological testing, and a past psychiatric/medical history interview. Depressive symptoms were assessed using the Geriatric Depression Scale (GDS) and Kessler Psychological Distress Scale (K10). Exploratory factor analysis was performed on the GDS and K10 to derive 'mood' and 'motivation' subscales. RESULTS: A total of 290 participants were classified as having MCI and 468 as cognitively normal (CN). Participants with MCI reported more depressive symptoms, and more MCI participants met the cut-off for clinically significant symptoms, relative to CN participants. Those with amnestic MCI (aMCI), but not non-amnestic MCI, had more depressive symptoms and were more likely to meet the cut-off for clinically significant depressive symptoms, relative to CN participants. Participants with MCI reported more mood-related symptoms than CN participants, while there were no differences between groups on motivation-related symptoms. CONCLUSION: Individuals with MCI, especially aMCI, endorse more depressive symptoms when compared with cognitively intact individuals. These findings highlight the importance of assessing and treating depressive symptoms in MCI.

Service development for intellectual disability mental health: a human rights approach.

BACKGROUND: People with intellectual disability (ID) experience higher rates of major mental disorders than their non-ID peers, but in many countries have difficulty accessing appropriate mental health services. The aim of this paper is to review the current state of mental health services for people with ID using Australia as a case example, and critically appraise whether such services currently meet the standards set by the Convention on the Rights of Persons with Disabilities. METHODS: The literature regarding the current state of mental health services for people with ID was reviewed, with a particular focus on Australia. RESULTS: The review highlighted a number of issues to be addressed to meet the mental health needs of people with ID to ensure that their human rights are upheld like those of all other citizens. Many of the barriers to service provision encountered in Australia are likely also to be relevant to other nations, including the culture of division between disability and mental health services, the inadequate training of both disability and mental health workers in ID mental health, and the lack of relevant epidemiological data. None of these barriers are insurmountable. CONCLUSIONS: Recommendations are made for adopting a human rights-based approach towards the development and provision of mental health services for people with ID. These include improved policy with measurable outcomes, improved service access via clear referral pathways and the sharing of resources across disability and mental health services, and improved service delivery through training and education initiatives for both the mental health and disability workforce.

Inflammatory biomarkers predict depressive, but not anxiety symptoms during aging: the prospective Sydney Memory and Aging Study.

This study addresses the paucity of research on the prospective relationship between a range of inflammatory markers and symptoms of depression and anxiety during aging. In the Sydney Memory and Aging Study, the relationships between remitted depression, current and first onset of symptoms of depression or anxiety (Geriatric Depression Scale and Goldberg Anxiety Scale (GDS, GAS), and markers of systemic inflammation (C-reactive protein (CRP), interleukins-1ß, -6, -8, -10, -12, plasminogen activator inhibitor-1 (PAI-1), serum amyloid A, tumor necrosis factor-α, and vascular adhesion molecule-1) were investigated. The sample consists of N=1037 non-demented community-dwelling elderly participants aged 70-90 years assessed at baseline and after 2-years. All analyses were adjusted for gender, age, years of education, total number of medical disorders diagnosed by a doctor, cardiovascular disorders, endocrine disorders, smoking, body mass index, currently using anti-depressants, NSAIDS or statins and diabetes mellitus. The results show a significant linear relationship between increasing levels of IL-6 and depressive symptoms at baseline only, whereas IL-8 was associated with depressed symptoms at baseline and at 2 years follow-up. In addition, IL-8 was associated with first onset of mild to moderate depressive symptoms over 2 years. Logistic regression analyses showed that PAI-1 (OR=1.37, 95% CI=1.10-1.71, p=0.005) was associated with remitted depression. Results for anxiety symptoms were negative. The findings are suggestive of IL-6 and IL-8 being associated with current symptoms and IL-8 being associated with first onset of depressive symptoms, whereas PAI-1 could be regarded as a marker of remitted depression.

Relationships between serum vitamin D levels, neuromuscular and neuropsychological function and falls in older men and women.

UNLABELLED: Among 463 community dwellers aged 70-90 years, those with vitamin D insufficiency showed reduced neuromuscular function, balance control and stepping ability and performed worse in tests of cognitive function. In men, vitamin D insufficiency was associated with an increased risk of falling. INTRODUCTION: The purpose of this study was to investigate the relationship between serum 25-hydroxy vitamin D (serum 25OHD) levels, physiological and neuropsychological function in older people, and to examine the relationship between serum 25OHD and prospective falls. METHODS: Four hundred sixty-three community-dwelling people aged 70-90 years underwent an assessment of physiological and neuropsychological performance and structured interviews relating to comorbidity and disability. Fall frequency during the 12 months follow-up was monitored with monthly falls diaries. RESULTS: Twenty-one percent of the men and 44% of the women were vitamin D insufficient (serum 25OHD ≤ 50 nmol/L). Participants with vitamin D insufficiency had weaker upper and lower limb strength, slower simple finger press and choice stepping reaction time, poorer leaning balance and slower gait speed, after controlling for age and body mass index, and, poorer executive function and visuospatial ability, after controlling for age and education. Vitamin D insufficiency significantly increased the rate of falls in men (IRR = 1.94, 95% CI = 1.19-3.15, p = 0.008) but not in women. CONCLUSIONS: These findings highlight the associations between vitamin D insufficiency and impairments in physiological and neuropsychological function that predispose older people to fall. The significant relationship between vitamin D insufficiency and falls found in the men may relate to the stronger association found between serum 25OHD levels and dynamic balance measures evident in this male population.

The relationship of current depressive symptoms and past depression with cognitive impairment and instrumental activities of daily living in an elderly population: the Sydney Memory and Ageing Study.

Depressive symptoms are common in the elderly and they have been associated with cognitive and functional impairment. However, relatively less is known about the relationship of a lifetime history of depression to cognitive impairment and functional status. The aim of this cross-sectional study was to assess whether current depressive symptoms and past depression are associated with cognitive or functional impairment in a community-based sample representative of east Sydney, Australia. We also examined whether there was an interaction between current and past depression in their effects on cognitive performance. Eight hundred non-demented aged participants received a neuropsychological assessment, a past psychiatric history interview and the 15-item Geriatric Depression Scale. The Bayer-Activities of Daily Living scale was completed by an informant to determine functional ability. Clinically relevant depressive symptoms were present in 6.1% of the sample and 16.6% reported a history of depression. Participants with current depression had significantly higher levels of psychological distress and anxiety, and lower life satisfaction and performed worse on memory and executive function compared to participants without current depression. After controlling for anxiety the effect on executive function was no longer significant while the effect on memory remained significant. A history of depression was associated with worse executive function, higher levels of psychological distress and anxiety, and lower life satisfaction. After controlling for psychological distress the effect of past depression on executive function was no longer significant. There were no significant interactions between current and past depression in their effects on cognitive performance. There were no differences between participants with or without current depression and with or without past depression on functional abilities. These results support the view that current and past depressive episodes are associated with poorer cognitive performance but not with functional abilities.