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While early diagnosis of younger-onset dementia (YOD) is crucial in terms of accessing appropriate services and future planning, diagnostic delays are common. This study aims to identify predictors of delay to diagnosis in a large sample of people with YOD and to investigate the impact of a specialist YOD service on this time to diagnosis.
A retrospective cross-sectional study.
The inpatient unit of a tertiary neuropsychiatry service in metropolitan Victoria, Australia.
People diagnosed with a YOD.
Measurements and methods:
We investigated the following predictors using general linear modeling: demographics including sex and location, age at onset, dementia type, cognition, psychiatric diagnosis, and number of services consulted with prior to diagnosis.
A total of 242 inpatients were included. The mean time to diagnosis was 3.4 years. Significant predictors of delay included younger age at onset, dementia type other than Alzheimer’s disease (AD) and behavioral-variant frontotemporal dementia (bvFTD), and increased number of services consulted. These predictors individually led to an increased diagnostic delay of approximately 19 days, 5 months, and 6 months, respectively. A specialized YOD service reduced time to diagnosis by 12 months.
We found that younger age at onset, having a dementia which was not the most commonly occurring AD or bvFTD, and increasing number of services were significant predictors of diagnostic delay. A novel result was that a specialist YOD service may decrease diagnostic delay, highlighting the importance of such as service in reducing time to diagnosis as well as providing post-diagnostic support.
A major barrier to the adoption of an approach that integrates spirituality into palliative care is the lack of preparation/education of healthcare professionals on the topic. This study aimed to evaluate the effectiveness of a continuing education activity for healthcare professionals addressing spirituality and spiritual care provision to patients and families within palliative care.
We conducted an intervention study using a quantitative pre- and posttest design in a convenience sample of 52 healthcare professionals. Participants completed the Brazilian version of the Spiritual Care Competence Scale before and after attending a four-hour continuing education activity.
Significant differences were observed between pre- and postintervention scores in the following dimensions: assessment and implementation of spiritual care, professionalization and improving the quality of spiritual care, personal support, and patient counseling (p < 0.001), and referral (p = 0.003).
Significance of results
The results of this study provide preliminary evidence of a positive effect of this educational intervention on the development of the competences needed by healthcare professionals to deliver a comprehensive approach centered on the patient/family, which includes attention to spirituality and spiritual care in the decision-making process.
This study describes the cross-cultural validation and psychometric evaluation of the Spiritual Care Competence Scale – Brazilian Portuguese version. This reliable and valid instrument is recommended in the literature to measure the outcomes of the education process in the development of spiritual care competences.
This is a cross-sectional validation study following the stages proposed by Beaton et al.: translation into Portuguese, back translation into English, expert committee review for semantic equivalence, assessment of the clarity of the pre-final version, and evaluation of the psychometric properties of the final version in Portuguese. Health professionals working at a public hospital in South Brazil participated in the different stages of this study.
Regarding internal consistency, total Cronbach's alpha was 0.92 and the mean inter-item correlation was 0.29. The test-retest procedure showed no statistically significant differences in the six subscales. The intraclass correlation coefficient ranged from 0.67 to 0.84, demonstrating the stability of the scale.
Significance of results
The results support the psychometric quality of the scale and indicate that the adapted instrument is a valid and reliable scale with good internal consistency for measuring spiritual care competencies of health professionals in Brazilian healthcare settings.
Clinical manifestations of acute bronchiolitis (AB) vary from minimal disease to severe respiratory failure. The response to respiratory viral infections is possibly influenced by genetic polymorphisms linked to the regulation of the inflammatory response. In the present study, we investigated whether interleukin-8 (IL-8) and interleukin-17 (IL-17) genetic variants are associated with the severity of AB. A group of Brazilian infants hospitalized with AB and a control group (infants with no or mild AB, without hospitalization) were genotyped for four IL-8/IL-17 variations. For replication, we studied an Argentinean population sample of infants with mild and severe AB. IL-8 polymorphism (rs 2227543) and IL-17 (rs2275913) variants showed significant associations with the severity of AB. The effect of the IL-8 variation could be replicated in the Argentinean sample. This finding suggests that IL-8 variations may influence the severity of AB in young infants. Further genetic association studies in low- or middle-income populations are necessary with the aim of expanding knowledge in this area.
In this article we survey descriptions of Christmas celebrations contained in the diaries and narratives of polar explorers (mostly British) from 1818 to 1912. We find that Christmas was a time almost universally associated with the display of positive emotions, although this was in the context of increased amounts of stress associated with the challenges of over-wintering at high latitudes. Firstly, we argue that Christmas was crucial to the well-being of expedition participants because it opened emotional channels that enabled them to cope with stress. Secondly, we argue that Christmas revealed a play space in which certain types of normally deviant behaviour were welcomed. Thirdly, we argue that Christmas was a major nutritional event for over-wintering crew members, satisfying a need for calories that was rarely met in the everyday rations.
Synchrotron radiation-based spectroscopic techniques are discussed. Their relevance to obtain information regarding the prebiotic evolution problem is pointed out. We present photoelectron–photoion coincidence (PEPICO) spectra of adenine and glycine obtained using 12 and 21 eV photons. The fragmentation pattern belonging to these molecules was found to present striking differences, which are discussed. Adenine partial ion yield in the energy region 12–21 eV is also presented. The neutral fragments were found to have very simple assignment. The importance of hydrogen cyanide (HCN) as a building block of these molecules is confirmed. A special instrumentation allowing precise comparisons between photon-induced desorption and energetic ion bombardment desorption is described. As an example, we show, for the first time, the frozen CO2 ice mass spectra bombarded by photons and energetic ions, under the same experimental conditions. The comparison shows that prebiotic evolution may only be properly understood if more than one particle, as energy source, is considered.
Few studies have investigated the relationship between schizophrenia and frontotemporal dementia.
To investigate this relationship through a clinicopathological investigation of young-onset frontotemporal dementia and a review of the case literature.
Cases of young-onset frontotemporal dementia were identified within the local brain bank. The clinical course and pathological findings were collated. For the literature review, cases of frontotemporal dementia identified through Medline were selected according to defined criteria. The demographic, clinical, pathological and genetic characteristics of cases presenting with a psychotic illness were identified.
In the case series, 5 of 17 patients with frontotemporal dementia had presented with a psychotic illness (schizophrenia/schizoaffective disorder n=4, bipolar disorder n=1) an average of 5 years prior to the dementia diagnosis. Patients with schizophrenia exhibited changes consistent with TDP-43 and ubiquitin-positive frontotemporal dementia. In the cases review, a third of patients aged 30 years or under and a quarter of those aged 40 years or under had been diagnosed with psychosis at presentation.
Patients with young-onset frontotemporal dementia may be diagnosed with a psychotic illness years before the dementia diagnosis is made. These findings have implications for clinicians and for our further understanding of the neurobiology of psychotic illness.
The perception of music without a stimulus, or musical hallucination, is reported in both organic and psychiatric disorders. It is most frequently described in the elderly with associated hearing loss and accompanied by some degree of insight. In this setting it is often referred to as ‘musical hallucinosis’. The aim of the authors was to present examples of this syndrome and review the current understanding of its neurobiological basis.
We describe three cases of persons experiencing musical hallucinosis in the context of hearing deficits with varying degrees of associated central nervous system abnormalities.
Putative neurobiological mechanisms, in particular those involving de-afferentation of a complex auditory recognition system by complete or partial deafness, are discussed in the light of current information from the literature.
Musical hallucinosis can be experienced in those patients with hearing impairment and is phenomenologically distinct for hallucinations described in psychiatric disorders.
In consultation-liaison settings, neuropsychiatrists are commonly asked to assess patients with hallucinatory syndromes and to differentiate ‘functional’ from ‘organic’ psychotic presentations.
The occurrence and management of visual hallucinations (VH) in healthy individuals, lesion states, neurodegenerative disorders, intoxication/withdrawal states and delirium are reviewed.
The presence of VH has been shown to predict a secondary rather than primary psychotic illness and an understanding of the neurobiology of the visual system – including how and where underlying neurotransmitter systems interact in visual processing and how perturbations can result in VH – allows for appropriate clinical assessment and management.
We describe the presentation of a young woman with long-standing complex partial seizures with occasional secondary generalization, who presented with complex visual hallucinations (CVHs) and delusions.
Routine biological workup including magnetic resonance imaging revealed an area of significant left-sided occipital gliosis. Video telemetry monitoring revealed a left occipital focus for the origin of the electrographic seizure discharge.
CVHs occur in a range of organic states, including epilepsy, and can be understood in terms of the underpinning neuroanatomy and neurotransmitter systems of the visual system.
In this study, we present an analysis of the internal stresses generated
during cooling of alumina-chromium composites using a micromechanical approach
by a finite element method. Residual stress fields are calculated for microstructural
models derived from a scanning electron microscope image. Results show in particular
that particles with concave shapes can generate relatively high local plastic
deformation and residual stress distributions in the adjacent matrix that are very
different from those of particles with a spherical shape. We present also the experimental
determination of residual stresses using synchrotron radiation from LURE. We show that
measurements are possible and the results concerning the mean stresses in the alumina
and chromium phases are analyzed.
Apart from its original use for closing an open duct, the Rashkind ductal occluder has been used to close a variety of other defects. We report successful use of this device to close a residual aortopulmonary window persisting after corrective surgery had been carried out at the age of six months.
A right-sided modified Blalock-Taussig anastomosis was created using a 5 mm Gore-Tex tube in a 4½ month old infant with unilateral “absence” of the right pulmonary artery. Wide patency of the shunt was confirmed shortly after by angiography, but rarefaction of the peripheral right pulmonary arteries was seen at the age of 2½ years, when corrective surgery was performed. Postoperative hypoperfusion of the right lung, along with severe pulmonary vascular abnormalities seen in a lung biopsy, indicated failure of the two-stage correction in spite of appropriate surgery.
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