MRI abnormalities in Creutzfeldt-Jakob disease and other rapidly progressive dementia

Objective: To investigate brain MRI abnormalities in a cohort of patients with rapidly progressive dementia (RPD) with and without a diagnosis of Creutzfeldt-Jakob disease (CJD). Methods: One hundred and seven patients with diagnosis of prion disease (60 with definite sCJD, 33 with probable sCJD and 14 with genetic prion disease) and 40 non-prion related RPD patients (npRPD) underwent brain MRI including DWI and FLAIR. MRIs were evaluated with a semiquantitative rating score, which separately considered abnormal signal extent and intensity in 22 brain regions. Clinical findings at onset, disease duration, cerebrospinal-fluid 14-3-3 and t-tau protein levels, and EEG data were recorded. Results: Among patients with definite/probable diagnosis of CJD or genetic prion disease, 2/107 had normal DWI-MRI: in one patient a 2-months follow-up DWI-MRI showed CJD-related changes while the other had autopsy-proven CJD despite no DWI abnormalities 282 days after clinical onset. CJD-related cortical changes were detected in all lobes and involvement of thalamus was common. In the npRPD groups, 6/40 patients showed DWI alterations that clustered in three different patterns: (1) minimal/doubtful signal alterations (limbic encephalitis, dementia with Lewy bodies); (2) clearly suggestive of alternative diagnoses (status epilepticus, Wernicke or metabolic encephalopathy); (3) highly suggestive of CJD (mitochondrial disease), though cortical swelling let exclude CJD. Conclusions: In the diagnostic work-up of RPD, negative/doubtful DWI makes CJD diagnosis rather unlikely, while specific DWI patterns help differentiating CJD from alternative diagnoses. The pulvinar sign is not exclusive of the variant form

Quality assessment of information about medications in primary care electronic patient record (EPR) systems

Background Many different brands of primary care electronic patient record (EPR) software are available to general practitioners (GPs). Their ability to support GPs in improving prescribing varies greatly. Objective To assess, using a ten-item tool, the quality of drug information provided by EPR software to support the appropriateness of prescriptions and to propose a list of quality standards for this type of application. Methods The eight EPR programmes most used in general practice in Italy were assessed by a multidisciplinary team using the ten-item tool. The tool evaluated information on single drugs and drug safety and information on prescription rules in force. Results Out of eight EPR programmes assessed, none scored more than 55% of the maximum possible score. Two achieved scores higher than 50%, one scored 48%, four ranged from 32% to 39% and one obtained 22%. Information on drug safety, such as the ability to detect interactions, to monitor laboratory parameters or to get updated information on drug safety was particularly limited. None of the eight EPR programmes contained drug information for patients, but two of them contained drug advertising. Conclusions This project highlighted the poor quality of drug information provided by these EPR programmes. The ten-item tool seems suitable for assessing their quality. Based on this analysis, we have proposed a set of ten quality standards for prescribing software

Quality assessment of information about medications in primary care electronic patient record (EPR) systems

Background Many different brands of primary care electronic patient record (EPR) software are available to general practitioners (GPs). Their ability to support GPs in improving prescribing varies greatly. Objective To assess, using a ten-item tool, the quality of drug information provided by EPR software to support the appropriateness of prescriptions and to propose a list of quality standards for this type of application. Methods The eight EPR programmes most used in general practice in Italy were assessed by a multidisciplinary team using the ten-item tool. The tool evaluated information on single drugs and drug safety and information on prescription rules in force. Results Out of eight EPR programmes assessed, none scored more than 55% of the maximum possible score. Two achieved scores higher than 50%, one scored 48%, four ranged from 32% to 39% and one obtained 22%. Information on drug safety, such as the ability to detect interactions, to monitor laboratory parameters or to get updated information on drug safety was particularly limited. None of the eight EPR programmes contained drug information for patients, but two of them contained drug advertising. Conclusions This project highlighted the poor quality of drug information provided by these EPR programmes. The ten-item tool seems suitable for assessing their quality. Based on this analysis, we have proposed a set of ten quality standards for prescribing software

Assessing Physical Processes of Permeable Pavements with a Large-Scale Laboratory Model

In recent decades, due to on-going urbanization and changes in rainfall patterns, urban drainage systems are facing increasing challenges. The expansion of impermeable surfaces and the increase of both frequency and intensity of rainfall events, are responsible for the augmented peak-flows and heavily polluted stormwater volumes conveyed by combined sewer overflows to water bodies. The need of assessing these challenges to mitigate the impact on water bodies’ quality has prompted International Authorities to develop standards and scientific communities to find solutions for an effective stormwater management. Sustainable Drainage Systems are effective at-source stormwater management solutions designed for collecting, retaining, and infiltrating direct rainfall and runoff from impervious surfaces. When properly applied in the urban drainage system, they mitigate pollution coming from wash-off of impervious surfaces and reduce both volumes and flood peaks conveyed to the drainage system. Among others, Permeable pavements (PPs) and infiltration trenches (ITs) are two solutions that can be easily retrofitted into the urban environment. PPs reduce surface runoff allowing direct infiltration of rainfall, whereas ITs collect runoff from nearby impervious surfaces. Both can temporally store relevant amount of water which is then slowly released to deeper native soil layers. Moreover, these systems act as filters trapping solids and pollutants onto or into the filter layers. However, physical clogging related to particle accumulation on the surface or inside the porous media reduce permeability of the system decreasing infiltration rates along time. This is a crucial aspect affecting both PPs and ITs effectiveness that must be accounted in the urban environment maintenance plans. A large-scale laboratory model is currently under development to analyze the main physical processes and to assess the efficiency starting first from the PPs. To this aim, a laboratory facility (Lora et al., 2016), built in the Laboratory of Hydraulics and Hydraulic Works of the Department of Civil, Environmental and Architectural Engineering (University of Padova), is being rearranged. The facility consists of a reinforced concrete box 6 m long x 2 m wide, and the height varies from 3.5 to 0.5 m. It is equipped with 50 openings on each lateral side for the insertion of probes (e.g. water content reflectometers - WCR) to continuously collect long term monitoring data in different positions. The end side of the facility is made of porous bricks allowing subsurface runoff to drain into a V-notch stream gauge. Another stream gauge is installed to measure exceeding surface runoff. During experiments, steady rainfall intensities ranging from 50 to 150 mm/h will be produced with a specifically designed rainfall simulator. Suitable materials for the filter layers package will be laid for 1 m total depth assessing filtration processes through the probes in three positions along the vertical. The rainfall simulator will be rearranged to guarantee uniform rainfall distribution on the PP surface characterized by a mild slope (about 2-3%). In the first set of experiments, the characteristics of the investigated PP will be tested in clear water condition, thus without adding suspended solids, to define the maximum infiltration capacity

Monitoring And Modeling the Impact of Urban Areas on Rivers’ Water Quality: Insights from the Sile River’s (Treviso, Italy) Case Study

Diffuse urban pollution, together with plant protection products and fertilizers used in agriculture activities, is a significant factor in altering and compromising receiving water bodies quality status. Changes in the built environment (e.g. imperviousness) and climatic forces (e.g. intense rainfall events after long dry periods) contribute to the increase of combined sewer system (CSS) overflows and of stormwater directly conveyed to water bodies. These discharges are responsible for water quality deterioration, as a result of high concentrations of pathogens, BOD, suspended solids, heavy metals, hydrocarbons and nutrients. Here we present insights from an ongoing project focusing on monitoring and modeling the Sile River, one of the longest European wellspring rivers, and some of its main tributaries crossing the city of Treviso (Figure). To assess the impact of rural/urban pollution discharges on the quality of the previously mentioned water bodies, a monitoring system consisting of 6 discharge measurement stations, 5 sampling systems, together with 3 multiparametric probes, has been realized. Monitoring buoys containing water probes placed at the centre of the Sile River, upstream and downstream the city of Treviso, are used to continuously monitor and quantify rural and urban area’s drainage system outflows, and pollutants trend, during both dry conditions and rainfall events. Water samples collected from automatic samplers at the same point where the water probes are placed, are being analyzed in the lab to check water probes’ data and monitor other pollutants. A rainfall gauge is used to trigger the automatic samplers during rainfall events. Information regarding the urban drainage and sewerage system, together with topographic surveys of the river network crossing the city of Treviso, has been used to develop a 1D hydrodynamic model. This will be calibrated using the data acquired over time from monitoring activities as input to gain an overview of the current status of the Sile River conditions, identify and quantify the main sources of pollution and suggest mitigation strategies. The results from the monitoring activities and the hydrodynamic model will eventually enable to assess the effects of an ongoing project aimed at separating the CSS in the city of Treviso

Ovarian teratoma or uterine malformation? PET/MRI as a novel useful tool in NMDAR encephalitis

This is a case report of a 17-year-old girl affected by N-methyl-D-aspartate-receptor (NMDAR) encephalitis suspected for a paraneoplastic syndrome. Ultrasound (US) and computed tomography (CT) imaging identified an ovarian lesion compatible with teratoma. 18Ffluorodeoxyglucose (FDG) positron emission tomography/ magnetic resonance imaging (PET/MRI), performed to evaluate metabolic activity of the brain and of the ovarian mass, correctly changed the diagnosis to uterine malformation that was later histologically proven

Sleep-wake profile in dementia with Lewy bodies, Alzheimer's disease, and normal aging.

none7noBACKGROUND: Alterations of the sleep-wake cycle are common features of neurodegenerative dementia. OBJECTIVES: To study differences in sleep-wake profiles in dementia with Lewy bodies (DLB), Alzheimer's disease (AD), and healthy controls. METHODS: 30 DLB and 32 AD patients, and 33 healthy elderly participants were studied. Patients were evaluated for global cognitive impairment, extrapyramidal signs, fluctuations of attention, and behavioral disorders. A comprehensive sleep-wake profile was obtained including a set of questionnaires [Pittsburgh Sleep Quality Index (PSQI), REM Sleep Behavior Disorder Single-Question screen (RBD1Q), Epworth Sleepiness Scale (ESS)] and 12-day sleep diaries. RESULTS: Patients were matched for age, gender, and disease severity. DLB patients showed more severe daytime somnolence/dysfunction due to somnolence, and a higher proportion of RBD-like symptoms (70%) compared to AD and controls (p < 0.001), regardless of the presence of psychoactive drug treatment. As for sleep timing, DLB patients had a greater number of daytime naps and longer night sleep, with the latter being associated with use of clonazepam. The severity of fluctuations was associated with the presence of RBD (Clinician Assessment of Fluctuation score = RBD+: 5.2±3.7; RBD-: 2.1±3.2, p = 0.04). AD patients reported the best sleep-wake profile, while healthy controls declared the poorest sleep quality, although sleep timing and the quality of wakefulness were comparable between AD and controls. DISCUSSION: RBD and daytime fluctuations of attention may coexist in DLB and even reciprocally potentiate each other. Self-reports of sleep quality may lead to an underestimation of sleep disturbances in AD, possibly influenced by anosognosia, compared to normal elderly individuals who complain mainly of insomnia.mixedCagnin, A; Fragiacomo, F; Camporese, G; Turco, M; Bussè, C; Ermani, M; Montagnese, SCagnin, Annachiara; Fragiacomo, F; Camporese, Giulia; Turco, Matteo; Busse', Cinzia; Ermani, Mario; Montagnese, Sar

Long-term preclinical magnetic resonance imaging alterations in sporadic Creutzfeldt-Jakob disease

An asymptomatic 74-year-old woman, on follow-up for a carotid body tumor, showed magnetic resonance imaging (MRI) focal restricted diffusion confined to the left temporal and occipital cortices. Thirteen months later, diffusion-weighted images revealed a bilateral cortical ribbon sign involving all lobes. After 1 month, the patient developed gait instability and cognitive decline rapidly evolving to severe dementia and death within 3 months. Prion protein gene sequence, molecular, and neuropathological studies confirmed the diagnosis of sporadic Creutzfeldt\u2013Jakob disease (sCJD) MM1 subtype. Here we show the kinetics of MRI changes and prion spreading in preclinical sCJD MM1