| 1. |
- Grimm, Max Joseph, et al.
(författare)
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Clinical Conditions “Suggestive of Progressive Supranuclear Palsy”—Diagnostic Performance
- 2020
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Ingår i: Movement Disorders. - : Wiley. - 0885-3185 .- 1531-8257. ; 35:12, s. 2301-2313
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Tidskriftsartikel (refereegranskat)abstract
- Background: The Movement Disorder Society diagnostic criteria for progressive supranuclear palsy introduced the diagnostic certainty level “suggestive of progressive supranuclear palsy” for clinical conditions with subtle signs, suggestive of the disease. This category aims at the early identification of patients, in whom the diagnosis may be confirmed as the disease evolves. Objective: To assess the diagnostic performance of the defined clinical conditions suggestive of progressive supranuclear palsy in an autopsy-confirmed cohort. Methods: Diagnostic performance of the criteria was analyzed based on retrospective clinical data of 204 autopsy-confirmed patients with progressive supranuclear palsy and 216 patients with other neurological diseases. Results: The conditions suggestive of progressive supranuclear palsy strongly increased the sensitivity compared to the National Institute of Neurological Disorders and Stroke and Society for Progressive Supranuclear Palsy criteria. Within the first year after symptom onset, 40% of patients with definite progressive supranuclear palsy fulfilled criteria for suggestive of progressive supranuclear palsy. Two-thirds of patients suggestive of progressive supranuclear palsy evolved into probable progressive supranuclear palsy after an average of 3.6 years. Application of the criteria for suggestive of progressive supranuclear palsy reduced the average time to diagnosis from 3.8 to 2.2 years. Conclusions: Clinical conditions suggestive of progressive supranuclear palsy allow earlier identification of patients likely to evolve into clinically possible or probable progressive supranuclear and to have underlying progressive supranuclear palsy pathology. Further work needs to establish the specificity and positive predictive value of this category in real-life clinical settings, and to develop specific biomarkers that enhance their diagnostic accuracy in early disease stages.
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| 2. |
- Hecken, Tobias, et al.
(författare)
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Conceptual Design Studies of “Boosted Turbofan” Configuration for short range
- 2020
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Ingår i: AIAA 2020-0506 Session: Hybrid Electric Aircraft Design Under Clean Sky 2 (LPA WP1.6.1.4). - Reston, Virginia : American Institute of Aeronautics and Astronautics.
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Konferensbidrag (refereegranskat)abstract
- This paper describes the current activities at the German Aerospace Center (DLR) and an associated consortium related to conceptual design studies of an aircraft configuration with hybrid electric propulsion for a typical short range commercial transport mission. The work is implemented in the scope of the European Clean Sky 2 program in the project “Advanced Engine and Aircraft Configurations” (ADEC) and “Turbo electric Aircraft Design Environment” (TRADE). The configuration analyzed incorporates parallel hybrid architecture consisting of gas turbines, electric machines, and batteries that adds electric power to the fans of the engines. A conceptual aircraft sizing workflow built in the DLR’s “Remote Component Environment” (RCE) incorporating tools of DLR that are based on semi-empirical and low level physics based methods. The TRADE consortium developed a simulation and optimization design platform with analysis models of higher fidelity for an aircraft with hybrid electric propulsion architecture. An implementation of the TRADE simulation and optimization design platform into the DLR’s RCE workflow by replacing the DLR models was carried out. The focus of this paper is on the quantitative evaluation of the “Boosted Turbofan” configuration utilizing the resulting workflow. In order to understand the cooperation between the DLR and TRADE consortium, a brief overview of the activities is given. Then the multi-disciplinary overall aircraft sizing workflow for hybrid electric aircraft built in RCE is shown. Hereafter, the simulation and optimization models of the TRADE design platform are described. Subsequently, an overview of the aircraft configuration considered in the scope of this work is given. The design space studies of the “Boosted Turbofan” configuration are presented. Finally, the deviations of the results between the workflows with and without the TRADE modules are discussed.
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| 3. |
- Respondek, Gesine, et al.
(författare)
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Validation of the movement disorder society criteria for the diagnosis of 4-repeat tauopathies
- 2020
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Ingår i: Movement Disorders. - : Wiley. - 0885-3185 .- 1531-8257. ; 35:1, s. 171-176
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Tidskriftsartikel (refereegranskat)abstract
- Background: The Movement Disorder Society criteria for progressive supranuclear palsy introduced the category “probable 4-repeat (4R)-tauopathy” for joint clinical diagnosis of progressive supranuclear palsy and corticobasal degeneration. Objectives: To validate the accuracy of these clinical criteria for “probable 4R-tauopathy” to predict underlying 4R-tauopathy pathology. Methods: Diagnostic accuracy for 4R-tauopathies according to the established criteria was estimated retrospectively in autopsy-confirmed patients with progressive supranuclear palsy and corticobasal degeneration (grouped as 4R-tauopathies), and Parkinson's disease, multiple system atrophy, and frontotemporal lobar degeneration (grouped as non-4R-tauopathies). Results: We identified 250 cases with progressive supranuclear palsy (N = 195) and corticobasal degeneration (N = 55) and with and non-4R-tauopathies (N = 161). Sensitivity and specificity of “probable 4R-tauopathy” was 10% and 99% in the first year and 59% and 88% at final record. Conclusions: The new diagnostic category “probable 4R-tauopathy” showed high specificity and may be suitable for the recruitment of patients with progressive supranuclear palsy and corticobasal degeneration into therapeutic trials targeting 4R-tauopathy. The low sensitivity underpins the need for diagnostic biomarkers.
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