Neuropathologic basis of frontotemporal dementia in progressive supranuclear palsy.

BackgroundProgressive supranuclear palsy (PSP) is a neurodegenerative disorder characterized by neuronal loss in the extrapyramidal system with pathologic accumulation of tau in neurons and glia. The most common clinical presentation of PSP, referred to as Richardson syndrome, is that of atypical parkinsonism with vertical gaze palsy, axial rigidity, and frequent falls. Although cognitive deficits in PSP are often ascribed to subcortical dysfunction, a subset of patients has dementia with behavioral features similar to the behavioral variant of frontotemporal dementia. In this study we aimed to identify the clinical and pathological characteristics of PSP presenting with frontotemporal dementia.MethodsIn this study, we compared clinical and pathologic characteristics of 31 patients with PSP with Richardson syndrome with 15 patients with PSP with frontotemporal dementia. For pathological analysis, we used semiquantitative methods to assess neuronal and glial lesions with tau immunohistochemistry, as well image analysis of tau burden using digital microscopic methods.ResultsWe found greater frontal and temporal neocortical neuronal tau pathology in PSP with frontotemporal dementia compared with PSP with Richardson syndrome. White matter tau pathology was also greater in PSP with frontotemporal dementia than PSP with Richardson syndrome. Genetic and demographic factors were not associated with atypical distribution of tau pathology in PSP with frontotemporal dementia.ConclusionsThe results confirm the subset of cognitive-predominant PSP mimicking frontotemporal dementia in PSP. PSP with frontotemporal dementia has distinct clinical features that differ from PSP with Richardson syndrome, as well as differences in distribution and density of tau pathology. © 2019 The Authors. Movement Disorders published by Wiley Periodicals, Inc. on behalf of International Parkinson and Movement Disorder Society

PSP Performance Analysis Report

The Personal Software Process (PSP) is a structured software development process that is intended to help software engineers understand and improve their performance, by using a disciplined, data-driven procedure. The PSP was created by Watts Humphrey to apply the underlying principles of the Software Engineering Institute’s (SEI) Capability Maturity Model (CMM) to the software development practices of a single developer. It gives software engineers the process skills necessary to work on a Team Software Process (TSP) team. PSP training includes eight assignments in two courses – PSP Fundamentals and PSP Advanced. The report includes final analysis of all the data that was gathered during the training

Abnormal resting-state functional connectivity in progressive supranuclear palsy and corticobasal syndrome

Background: Pathological and MRI-based evidence suggests that multiple brain structures are likely to be involved in functional disconnection between brain areas. Few studies have investigated resting-state functional connectivity (rsFC) in progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). In this study, we investigated within- and between-network rsFC abnormalities in these two conditions. Methods: Twenty patients with PSP, 11 patients with CBS, and 16 healthy subjects (HS) underwent a resting-state fMRI study. Resting-state networks (RSNs) were extracted to evaluate within- and between-network rsFC using the Melodic and FSLNets software packages. results: Increased within-network rsFC was observed in both PSP and CBS patients, with a larger number of RSNs being involved in CBS. Within-network cerebellar rsFC positively correlated with mini-mental state examination scores in patients with PSP. Compared to healthy volunteers, PSP and CBS patients exhibit reduced functional connectivity between the lateral visual and auditory RSNs, with PSP patients additionally showing lower functional connectivity between the cerebellar and insular RSNs. Moreover, rsFC between the salience and executive-control RSNs was increased in patients with CBS compared to HS. conclusion: This study provides evidence of functional brain reorganization in both PSP and CBS. Increased within-network rsFC could represent a higher degree of synchronization in damaged brain areas, while between-network rsFC abnormalities may mainly reflect degeneration of long-range white matter fibers

Search for double charmonium decays of the P-wave spin-triplet bottomonium states

Using a sample of 158 million $\Upsilon(2S)$ events collected with the Belle detector, we search for the first time for double charmonium decays of the $P$-wave spin-triplet bottomonium states ($\Upsilon(2S) \to \gamma \chi_{bJ}$, \chi_{bJ} \to \jpsi \jpsi, \jpsi \psp, \psp \psp for J=0, 1, and 2). No significant $\chi_{bJ}$ signal is observed in the double charmonium mass spectra, and we obtain the following upper limits, \BR(\chi_{bJ} \to \jpsi \jpsi)<7.1\times 10^{-5}, $2.7\times 10^{-5}$, $4.5\times 10^{-5}$, \BR(\chi_{bJ} \to \jpsi \psp)<1.2\times 10^{-4}, $1.7\times 10^{-5}$, $4.9\times 10^{-5}$, \BR(\chi_{bJ} \to \psp \psp)<3.1\times 10^{-5}, $6.2\times 10^{-5}$, $1.6\times 10^{-5}$ for J=0, 1, and 2, respectively, at the 90% confidence level. These limits are significantly lower than the central values (with uncertainties of 50% to 70%) predicted using the light cone formalism but are consistent with calculations using the NRQCD factorization approach.Comment: 7 pages, 4 figures, 1 tabl

Cortical thickness, surface area and volume measures in Parkinson's disease, multiple system atrophy and progressive supranuclear palsy

OBJECTIVE Parkinson's disease (PD), Multiple System Atrophy (MSA) and Progressive Supranuclear Palsy (PSP) are neurodegenerative diseases that can be difficult to distinguish clinically. The objective of the current study was to use surface-based analysis techniques to assess cortical thickness, surface area and grey matter volume to identify unique morphological patterns of cortical atrophy in PD, MSA and PSP and to relate these patterns of change to disease duration and clinical features. METHODS High resolution 3D T1-weighted MRI volumes were acquired from 14 PD patients, 18 MSA, 14 PSP and 19 healthy control participants. Cortical thickness, surface area and volume analyses were carried out using the automated surface-based analysis package FreeSurfer (version 5.1.0). Measures of disease severity and duration were assessed for correlation with cortical morphometric changes in each clinical group. RESULTS Results show that in PSP, widespread cortical thinning and volume loss occurs within the frontal lobe, particularly the superior frontal gyrus. In addition, PSP patients also displayed increased surface area in the pericalcarine. In comparison, PD and MSA did not display significant changes in cortical morphology. CONCLUSION These results demonstrate that patients with clinically established PSP exhibit distinct patterns of cortical atrophy, particularly affecting the frontal lobe. These results could be used in the future to develop a useful clinical application of MRI to distinguish PSP patients from PD and MSA patients

Application of AA-PSP to hypersonic flows: the double ramp model

Anodized Aluminium Pressure Sensitive Paint (AA-PSP) is known for its rapid response characteristics, making it a highly desirable technique when studying high-speed phenomenon on a global scale. The current study examines the efficacy of the AA-PSP technique, which is prepared with a more practical approach than that reported in literature, in analysing the flow characteristics of a double ramp model placed in hypersonic flow of M = 5. Three different flow angles of 0°, −2°, and −4° are studied. Two-dimensional colour schlieren visualisation, using a colour wheel, is employed alongside high sensitivity Kulite pressure tap data to corroborate the AA-PSP findings. The AA-PSP results show good correlation between the qualitative schlieren and ±8.9% discrepency with the quantitative pressure tap data. The more practical AA-PSP preparation proposed in the current study, which uses aluminium alloy 6-series rather than pure aluminium, is proved to have the response time and the accuracy to be applied to unsteady high-speed flows

The 2 × 2 model of perfectionism and school‐ and community‐based sport participation.

The authors adopted the 2 × 2 model of perfectionism to examine the unique and interactive effects of two dimensions of perfectionism (personal standards perfectionism PSP and evaluative concerns perfectionism ECP) on personal and interpersonal indicators of participant experience in youth sport (enjoyment, physical self‐worth, and friendship quality). Participants (N = 219, M age = 15.12, SD = 2.02) were recruited from various school‐ and community‐based sports and completed a multi‐section questionnaire. Consideration of main and interaction effects indicated that pure PSP (high PSP/low ECP) was associated with the most positive sport experience and pure ECP (low PSP/high ECP) was associated with the least positive sport experience. The findings suggest that subtypes of perfectionism from the 2 × 2 model are predictive of differing experiences in youth sport participation. (PsycINFO Database Record (c) 2015 APA, all rights reserved). (journal abstract

Heavy metals contaminating the environment of a progressive supranuclear palsy cluster induce tau accumulation and cell death in cultured neurons

Progressive supranuclear palsy (PSP) is a neurodegenerative disorder characterized by the presence of intracellular aggregates of tau protein and neuronal loss leading to cognitive and motor impairment. Occurrence is mostly sporadic, but rare family clusters have been described. Although the etiopathology of PSP is unknown, mutations in the MAPT/tau gene and exposure to environmental toxins can increase the risk of PSP. Here, we used cell models to investigate the potential neurotoxic effects of heavy metals enriched in a highly industrialized region in France with a cluster of sporadic PSP cases. We found that iPSC-derived iNeurons from a MAPT mutation carrier tend to be more sensitive to cell death induced by chromium (Cr) and nickel (Ni) exposure than an isogenic control line. We hypothesize that genetic variations may predispose to neurodegeneration induced by those heavy metals. Furthermore, using an SH-SY5Y neuroblastoma cell line, we showed that both heavy metals induce cell death by an apoptotic mechanism. Interestingly, Cr and Ni treatments increased total and phosphorylated tau levels in both cell types, implicating Cr and Ni exposure in tau pathology. Overall, this study suggests that chromium and nickel could contribute to the pathophysiology of tauopathies such as PSP by promoting tau accumulation and neuronal cell death