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Down syndrome regression disorder (DSRD) is a rare condition involving subacute cognitive decline, loss of previously acquired developmental skills, and prominent neuropsychiatric symptoms, particularly catatonia, in people with Down syndrome. It is thought to involve both autoimmune and neuropsychiatric mechanisms. Research, however, is largely restricted to case studies and retrospective case series and is particularly limited in terms of prospective longitudinal follow-up. We report a case study of a person with DSRD who received both immunomodulatory (intravenous immunoglobulin; IVIG) and psychiatric interventions (electroconvulsive therapy, ECT) over two years with regular assessments using caregiver and clinician ratings. This revealed a small, unsustained response to IVIG and a rapid, sustained response once ECT was introduced. The case highlights the importance of multimodal assessment involving multiple medical specialties, the need to trial different therapies due to the condition's complexity, and the significant barriers that patients and their families face in accessing care. [ABSTRACT FROM AUTHOR]

Objectives: Differentiating schizophrenia from mania in acutely psychotic patients can be difficult, but is important in determining immediate and subsequent management. Such differentiation is generally addressed by clinical interviews, but an observational approach may assist. This paper therefore describes the development of a relevant observational measure. Methods: We developed a provisional list of 49 items (weighting features with suggested specificity to schizophrenia and mania) for independent completion by two nurses and judged its ability to predict diagnosis provided by consultant psychiatrists. Results: Eighty-seven psychotic patients were recruited, and 173 completed data sets were analysed. We refined the item set to two sets of 10 items that best-differentiated schizophrenia from mania and vice versa. Optimal differentiation was achieved with a score of at least 7 on both the schizophrenia and mania item sets. Difference scores (i.e. schizophrenia items affirmed minus mania items affirmed) were also generated, with a difference score of +1 (i.e. one or more schizophrenia items being affirmed than mania items) showing optimal differentiation (sensitivity 0.67, specificity 0.82) between the two conditions. Evaluating all potential difference scores, we demonstrated that, as difference scores increased, diagnostic accuracy in identifying each condition was very high. Conclusion: Analyses allow the properties of an observational measure (the 20-item Sydney Psychosis Observation Tool) to be described. While a single cut-off difference score was derived with acceptable discriminatory ability, we also established the capacity of varying difference scores to assign both schizophrenia and mania diagnoses with high accuracy. [ABSTRACT FROM AUTHOR]

Objectives: Apathy is a common symptom in mild cognitive impairment (MCI) and may predict progression to dementia. Little research, however, has investigated the longitudinal trajectory of apathy in patients with MCI or controlled for depression, which can mimic apathy, when examining its clinical correlates. The current study sought to address these issues. Design: A prospective longitudinal study was conducted over 3 years. Setting: Nine memory clinics around Australia Participants: One hundred and eighty-five patients with MCI at baseline. Measurements: Measures of cognition, function, neuropsychiatric symptoms, caregiver burden, and medication use were completed annually with additional assessments at 3 and 6 months. Patients were also assessed for dementia by expert clinicians at these time points. Results: Of 164 patients who completed measures of neuropsychiatric symptoms, 59 (36.0%) had apathy and 61 (37.2%) had depression. The proportion affected by apathy and overall apathy scores increased over time, in contrast to measures of depression, which remained relatively stable. Apathy was associated with incident dementia and worse cognition, function, neuropsychiatric symptoms, and caregiver burden independent of both depression and incident dementia. Depression was associated with worse function, albeit to lesser degree than apathy, and neuropsychiatric symptoms. Conclusions: Apathy increases in MCI and is associated with worse clinical outcomes. These findings provide further evidence for apathy as a marker of clinical decline in older people and poorer outcomes across neurocognitive disorders. [ABSTRACT FROM AUTHOR]

Objectives: Apathy is a common symptom in dementia, though can be difficult to distinguish from depression due to shared features and frequent co-occurrence. As such, a significant limitation of much previous research on apathy is the failure to control for depression. The current study sought to address this by examining the trajectory and clinical correlates of apathy after controlling for depression. Methods: Seven hundred and seventy-nine patients with dementia were recruited from nine memory clinics around Australia. Measures of dementia severity, cognition, functional ability, neuropsychiatric symptoms, caregiver burden and medication use were completed at baseline and at regular intervals over a 3-year period. Driving and institutionalisation data were obtained throughout the study. Mortality data were obtained from state registries 8 years after baseline. Results: Of the 662 patients with completed measures of neuropsychiatric symptoms, 342 (51.7%) had apathy and 332 (50.2%) had depression at baseline, while 212 (32.0%) had both. Whereas apathy increased over time, depression remained relatively stable. Apathy, but not depression, was associated with greater dementia severity, poorer cognition and function, driving cessation and mortality. Both apathy and depression were associated with greater neuropsychiatric symptoms, psychosis, caregiver burden and institutionalisation. Conclusions: Apathy increases over the course of dementia and is associated with worse clinical outcomes independent of depression. Distinguishing apathy and depression appears important given their different implications for prognosis and management. [ABSTRACT FROM AUTHOR]

Objectives: Paediatric bipolar disorder – bipolar disorder occurring in prepubertal children – is a diagnosis subject to considerable controversy. Whilst historically considered to be very rare, proponents since the 1990s have argued that mania can present differently in children and, as such, is much more common than previously thought. Such proposals raise questions about the validity of proposed phenotypes and potential risks of iatrogenic harm. Methods: I critically examine the construct of paediatric bipolar disorder using Robins and Guze's (1970, American Journal of Psychiatry 126 , 983–987) influential criteria for the validity of a psychiatric diagnosis. I review, in turn, evidence relating to its clinical description, delimitation from other conditions, follow-up studies, family studies, laboratory studies, and treatment response. Results: Across domains, existing research highlights significant challenges establishing the diagnosis. This includes significant heterogeneity in operationalising criteria for children; variable or poor inter-rater reliability; difficulty distinguishing paediatric bipolar disorder from other conditions; large differences in rates of diagnosis between the United States of America and other countries; limited evidence of continuity with adult forms; and a lack of evidence for proposed paediatric phenotypes in children at genetic high-risk of the condition. Laboratory and treatment studies are limited, but also do not provide support for the construct. Conclusions: Evidence for the more widespread existence of paediatric bipolar disorder and its various proposed phenotypes remains weak. The ongoing popularity of the diagnosis, most evident in America, may reflect social pressures and broader limitations in psychiatric nosology. The uncertainty around the diagnosis highlights the need for careful longitudinal assessment of children potentially affected. [ABSTRACT FROM AUTHOR]

Objectives: Dementia, with its progressive cognitive and functional decline and associated neuropsychiatric symptoms, places a large burden on caregivers. While frequently studied, longitudinal findings about the overall trajectory of burden are mixed. The study sought to characterize caregiver burden over a 3-year period and identify predictors of this burden.Methods: Seven-hundred-and-eighty-one patients with dementia were recruited from nine memory clinics around Australia. Measures of caregiver burden, cognition, function, and neuropsychiatric symptoms were completed with patients and their caregivers at regular intervals over a 3-year period. Patients' level of services and medication use were also recorded.Results: Of the 720 patients with measures of caregiver burden at baseline, 47.4% of caregivers had clinically significant levels of burden. This proportion increased over time, with 56.8% affected at 3 years. Overall levels of burden increased for caregivers of patients without services, though did not change for caregivers of patients receiving services or residential care after controlling for other variables. Patient characteristics-including greater neuropsychiatric symptoms, lower functional ability, fewer medications, lack of driving ability-and female sex of caregivers were associated with greater burden.Conclusions: High levels of caregiver burden are present in a large proportion of caregivers of people with dementia and this increases over time for those without services. Clinical characteristics of patients (including neuropsychiatric symptoms, function, overall health, driving status), level of services, and caregiver sex appear to be the best predictors of this burden. These characteristics may help identify caregivers at greater risk of burden to target for intervention. [ABSTRACT FROM AUTHOR]

Objective: Huntington disease (HD) is an autosomal dominant neurodegenerative disease involving motor disturbances, cognitive decline and psychiatric symptoms. Psychotic symptoms occur in a significant proportion of patients. We sought to characterise the clinical outcomes of this group of patients.Methods: Data were drawn from the Cooperative Huntington Observational Research Trial, a prospective, multi-centre observational study. 1082 patients with HD were recruited. Measures of cognition, function, behavioural disturbance and motor function were completed annually over 5 years.Results: Overall, 190 patients (17.6%) displayed psychotic symptoms. These patients demonstrated worse cognition, function and behavioural disturbances than patients without psychosis over time. Patients with psychosis also demonstrated lower levels of chorea than patients without psychosis, despite adjusting for concurrent antipsychotic and tetrabenazine use.Conclusions: Psychosis in HD is associated with poorer outcomes in cognition, function and behavioural symptoms. Patients with psychotic symptoms may also have less chorea. Altogether, the findings suggest patients with psychosis have a distinct clinical course. [ABSTRACT FROM AUTHOR]

Depression and a number of other psychiatric conditions can impair cognition and give the appearance of neurodegenerative disease. Collectively, this group of disorders is known as 'pseudodementia' and are important to identify given their potential reversibility with treatment. Despite considerable interest historically, the longitudinal outcomes of patients with pseudodementia remain unclear. We conducted a systematic review of longitudinal studies of pseudodementia. Bibliographic databases were searched using a wide range of search terms. Two reviewers independently assessed papers for inclusion, rated study quality, and extracted data. The search identified 18 studies with follow-up varying from several weeks to 18 years. Overall, 284 patients were studied, including 238 patients with depression, 18 with conversion disorder, 14 with psychosis, and 11 with bipolar disorder. Irrespective of diagnosis, 33% developed irreversible dementia at follow-up, 53% no longer met criteria for dementia, and 15% were lost to follow-up. Considerable variability was identified, with younger age at baseline, but not follow-up duration, associated with better outcomes. ECT and pharmacological interventions were also reported to be beneficial, though findings were limited by the poor quality of the studies. Overall, the findings suggest that pseudodementia may confer an increased risk of irreversible dementia in older patients. The findings also indicate, however, that a significant proportion improve, while many remain burdened with their psychiatric condition, independent of organic dementia. The findings support the clinical value of the construct and the need for its re-examination in light of developments in neuroimaging, genomics, other investigative tools, and trial methodology. [ABSTRACT FROM AUTHOR]