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Progressive supranuclear palsy

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https://www.readbyqxmd.com/read/29232559/cilostazol-a-phosphodiesterase-3-inhibitor-activates-proteasome-proteolysis-and-attenuates-tauopathy-and-cognitive-decline
#1
Ari W Schaler, Natura Myeku
Alzheimer's disease and several variants of frontotemporal degeneration including progressive supranuclear palsy and corticobasal degeneration are characterized by the accumulation of abnormal tau protein into aggregates. Most proteins, including tau, are degraded via the ubiquitin proteasome system, but when abnormal tau accumulates, the function of 26S proteasomes is downregulated. The negative effect of tau aggregates on the function of the proteasome can have deleterious consequences on protein homeostasis and disease progression...
November 23, 2017: Translational Research: the Journal of Laboratory and Clinical Medicine
https://www.readbyqxmd.com/read/29206491/new-advances-in-tau-imaging-in-parkinsonism
#2
Mikaeel Valli, Antonio P Strafella
Currently, the differential diagnosis between atypical parkinsonisms and classical idiopathic Parkinson's disease can be quite difficult because of the significant overlap of clinical presentation and symptoms. Neurodegenerative conditions, including progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), and frontotemporal dementia (FTD), are primarily characterized by accumulation of tau protein in the brain. Recent imaging developments for tau pathology may provide a promising tool for the assessment of diagnosis, prognosis, and progression of these neurodegenerative disorders...
December 5, 2017: International Review of Psychiatry
https://www.readbyqxmd.com/read/29204015/clinical-profile-of-cognitive-decline-in-patients-with-parkinson-s-disease-progressive-supranuclear-palsy-and-multiple-system-atrophy
#3
Sulena, Dipti Gupta, Anjani Kumar Sharma, Naveen Kumar
Background: There are very less data on the comparison between the cognitive profile in Parkinson's disease (PD) and Parkinson's-plus groups, especially in India. Aims: The aim of this study is to compare the cognitive profile across PD, progressive supranuclear palsy (PSP), and multiple system atrophy (MSA) groups and compare them using Mini-Mental State Examination (MMSE), frontal assessment battery (FAB), and verbal fluency tests. Settings and Design: This was a cross-sectional study...
October 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/29203673/evidence-for-sortilin-modulating-regional-accumulation-of-human-tau-prions-in-transgenic-mice
#4
Noah R Johnson, Carlo Condello, Shenheng Guan, Abby Oehler, Julia Becker, Marta Gavidia, George A Carlson, Kurt Giles, Stanley B Prusiner
Misfolding of tau proteins into prions and their propagation along neural circuits are thought to result in neurodegeneration causing Alzheimer's disease, progressive supranuclear palsy, chronic traumatic encephalopathy, and other tauopathies. Little is known about the molecular processes mediating tau prion replication and spreading in different brain regions. Using transgenic (Tg) mice with a neuronal promoter driving expression of human mutant (P301S) tau, we found that tau prion formation and histopathologic deposition is largely restricted to the hindbrain...
December 4, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29196955/simple-linear-brainstem-mri-measurements-in-the-differential-diagnosis-of-progressive-supranuclear-palsy-from-the-parkinsonian-variant-of-multiple-system-atrophy
#5
Vasilios C Constantinides, George P Paraskevas, Eleftherios Stamboulis, Elisabeth Kapaki
Differential diagnosis of progressive supranuclear palsy (PSP) and the parkinsonian variant of multiple system atrophy (MSA-P) from Parkinson's disease (PD) can be difficult, particularly in atypical cases or early in the disease course. The Magnetic Resonance Parkinsonism Index (MRPI) utilizes linear and surface (planimetry) measurements and has been proposed as a dual MRI biomarker, with high values indicative of PSP and low values of MSA. The aim of this study was to examine the utility of simple linear MRI brainstem measurements, without the use of MRI planimetry, in the diagnosis of patients with Parkinsonism and compare them to the MRPI...
December 1, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/29196058/comparison-of-dystonia-between-parkinson-s-disease-and-atypical-parkinsonism-the-clinical-usefulness-of-dystonia-distribution-and-characteristics-in-the-differential-diagnosis-of-parkinsonism
#6
Won Tae Yoon
OBJECTIVE: Dystonia is occasionally found in patients with Parkinson's disease (PD) and atypical parkinsonisms. However, systematic comparative analysis of the association between dystonia and parkinsonism have seldom been reported. The goals of this study are to compare the clinical characteristics and distributions of dystonia between PD, multiple system atrophy (MSA), progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD). METHODS: We prospectively enrolled 176 patients who presented with dystonia and parkinsonism out of 1278 patients with parkinsonism...
November 14, 2017: Neurologia i Neurochirurgia Polska
https://www.readbyqxmd.com/read/29195744/discrimination-of-atypical-parkinsonisms-with-transcranial-magnetic-stimulation
#7
Alberto Benussi, Valentina Dell'Era, Valentina Cantoni, Clarissa Ferrari, Salvatore Caratozzolo, Luca Rozzini, Antonella Alberici, Alessandro Padovani, Barbara Borroni
BACKGROUND: Differential diagnosis of atypical parkinsonian disorders, i.e. dementia with Lewy bodies (DLB), progressive supranuclear palsy (PSP) or corticobasal syndrome (CBS) still remains problematic. Furthermore, DLB may overlap with Alzheimer's disease (AD) in the early stages of disease. OBJECTIVE: To determine whether transcranial magnetic stimulation (TMS) can be used to classify atypical parkinsonian disorders and AD. METHODS: A paired-pulse TMS multi-paradigm approach assessing multiple intracortical circuits, as short interval intracortical inhibition-facilitation and short latency afferent inhibition, was used to model a decision tree analysis and determine diagnostic accuracy in classifying different neurodegenerative disorders...
November 22, 2017: Brain Stimulation
https://www.readbyqxmd.com/read/29191965/caenorhabditis-elegans-models-of-tauopathy
#8
REVIEW
Ghulam Jeelani Pir, Bikash Choudhary, Eckhard Mandelkow
One of the hallmarks of the tauopathies, which include the neurodegenerative disorders, such as Alzheimer disease (AD), corticobasal degeneration, frontotemporal dementia, and progressive supranuclear palsy (PSP), is the abnormal accumulation of post-translationally modified, insoluble tau. The result is a loss of neurons, decreased mental function, and complete dependence of patients on others. Aggregation of tau, which under physiologic conditions is a highly soluble protein, is thought to be central to the pathogenesis of these diseases...
December 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/29191439/effects-of-cerebellar-neuromodulation-in-movement-disorders-a%C3%A2-systematic-review
#9
REVIEW
Carina França, Daniel Ciampi de Andrade, Manoel Jacobsen Teixeira, Ricardo Galhardoni, Valquiria Silva, Egberto Reis Barbosa, Rubens Gisbert Cury
BACKGROUND: The cerebellum is involved in the pathophysiology of many movement disorders and its importance in the field of neuromodulation is growing. OBJECTIVES: To review the current evidence for cerebellar modulation in movement disorders and its safety profile. METHODS: Eligible studies were identified after a systematic literature review of the effects of cerebellar modulation in cerebellar ataxia, Parkinson's disease (PD), essential tremor (ET), dystonia and progressive supranuclear palsy (PSP)...
November 23, 2017: Brain Stimulation
https://www.readbyqxmd.com/read/29191430/oculomotor-abnormalities-in-children-with-niemann-pick-type-c
#10
James Blundell, Steven Frisson, Anupam Chakrapani, Paul Gissen, Chris Hendriksz, Suresh Vijay, Andrew Olson
Niemann-Pick type C (NP-C) is a rare recessive disorder associated with progressive supranuclear gaze palsy. Degeneration occurs initially for vertical saccades and later for horizontal saccades. There are studies of oculomotor degeneration in adult NP-C patients [1, 2] but no comparable studies in children. We used high-resolution video-based eye tracking to record monocular vertical and horizontal eye movements in 2 neurological NP-C patients (children with clinically observable oculomotor abnormalities) and 3 pre-neurological NP-C patients (children without clinically observable oculomotor abnormalities)...
November 16, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29187684/-a-case-of-hereditary-sensory-and-autonomic-neuropathy-type-1e-with-frontal-lobe-dysfunction-as-an-initial-symptom
#11
Masashi Watanabe, Yushi Matsumoto, Kensho Okamoto, Bungo Okuda, Ikuko Mizuta, Toshiki Mizuno
A 49-year-old man had developed gradually personality change, gait disturbance, and hearing loss for five years. On admission, he presented with frontal release signs, stuttering, vertical gaze palsy, sensorineural deafness, muscle rigidity, ataxia, and sensory disturbance with areflexia in the lower extremities. Brain MRI demonstrated atrophy in the cerebellum and midbrain tegmentum as well as cerebral atrophy, predominantly in the frontal lobe. He was tentatively diagnosed as progressive supranuclear palsy on the basis of clinical features and imagings...
November 28, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/29184432/role-of-magnetic-resonance-planimetry-and-magnetic-resonance-parkinsonism-index-in-discriminating-parkinson-s-disease-and-progressive-supranuclear-palsy-a-retrospective-study-based-on-1-5-and-3-t-mri
#12
Waseem Mehmood Nizamani, Fatima Mubarak, Muhammad Danish Barakzai, Muhammad Saad Ahmed
Objective: The objective of the study was to assess magnetic resonance (MR) planimetric measurements and MR parkinsonism index (MRPI) in differentiating progressive supranuclear palsy (PSP) from Parkinson's disease (PD) using 1.5 and 3 T MRI scanner. Subjects and methods: After ethical approval was obtained, analysis of 34 consecutive patients with PSP, 34 patients with PD and 34 healthy controls (HCs) was performed. HCs were age-matched adults without any history of neurodegenerative disease or movement disorders...
2017: International Journal of General Medicine
https://www.readbyqxmd.com/read/29181488/preclinical-and-clinical-development-of-abbv-8e12-a-humanized-anti-tau-antibody-for-treatment-of-alzheimer-s-disease-and-other-tauopathies
#13
T West, Y Hu, P B Verghese, R J Bateman, J B Braunstein, I Fogelman, K Budur, H Florian, N Mendonca, D M Holtzman
Tau neurofibrillary tangles are found in the brains of patients suffering from Alzheimer's disease and other tauopathies. The progressive spreading of tau pathology from one brain region to the next is believed to be caused by extracellular transsynaptic transmission of misfolded tau between neurons. Preclinical studies have shown that antibodies against tau can prevent this transfer of misfolded tau between cells. Thus, antibodies against tau have the potential to stop or slow the progression of tau pathology observed in human tauopathies...
2017: Journal of Prevention of Alzheimer's Disease
https://www.readbyqxmd.com/read/29164600/18f-pet-with-florbetaben-for-the-early-diagnosis-of-alzheimer-s-disease-dementia-and-other-dementias-in-people-with-mild-cognitive-impairment-mci
#14
REVIEW
Gabriel Martínez, Robin Wm Vernooij, Paulina Fuentes Padilla, Javier Zamora, Leon Flicker, Xavier Bonfill Cosp
BACKGROUND: 18F-florbetaben uptake by brain tissue, measured by positron emission tomography (PET), is accepted by regulatory agencies like the Food and Drug Administration (FDA) and the European Medicine Agencies (EMA) for assessing amyloid load in people with dementia. Its added value is mainly demonstrated by excluding Alzheimer's pathology in an established dementia diagnosis. However, the National Institute on Aging and Alzheimer's Association (NIA-AA) revised the diagnostic criteria for Alzheimer's disease and confidence in the diagnosis of mild cognitive impairment (MCI) due to Alzheimer's disease may be increased when using some amyloid biomarkers tests like 18F-florbetaben...
November 22, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29157745/differential-diagnosis-of-parkinsonian-syndromes-using-dopamine-transporter-and-perfusion-spect
#15
Shigetoshi Takaya, Nobukatsu Sawamoto, Tomohisa Okada, Gosuke Okubo, Sei Nishida, Kaori Togashi, Hidenao Fukuyama, Ryosuke Takahashi
OBJECTIVE: We aimed to assess whether a combined analysis of dopamine transporter (DAT)- and perfusion-SPECT images (or either) could: (1) distinguish atypical parkinsonian syndromes (APS) from Lewy body diseases (LBD; majority Parkinson disease [PD]), and (2) differentiate among APS subgroups (progressive supranuclear palsy [PSP], corticobasal syndrome [CBS], and multiple system atrophy [MSA]). METHODS: We recruited consecutive patients with neurodegenerative parkinsonian syndromes (LBD, n = 46; APS, n = 33)...
November 14, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/29137817/beyond-als-and-ftd-the-phenotypic-spectrum-of-tbk1-mutations-includes-psp-like-and-cerebellar-phenotypes
#16
Carlo Wilke, Jonathan Baets, Jan L De Bleecker, Tine Deconinck, Saskia Biskup, Stefanie N Hayer, Stephan Züchner, Rebecca Schüle, Peter De Jonghe, Matthis Synofzik
Mutations in the TANK-binding kinase 1 gene (TBK1) are a rare, but recurrent cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). However, the complete phenotypic spectrum of syndromes associated with TBK1 mutations remains to be elucidated. Using next-generation panel-sequencing of neurodegenerative disease genes, we identified a TBK1 index patient presenting with a progressive supranuclear palsy-like syndrome. Consecutively, we screened the whole-exome sequencing data of 439 index subjects presenting with various neurodegenerative syndromes outside the ALS-FTD spectrum for TBK1 mutations...
October 24, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/29126761/mr-planimetry-in-neurodegenerative-parkinsonism-yields-high-diagnostic-accuracy-for-psp
#17
Stephanie Mangesius, Anna Hussl, Florian Krismer, Philipp Mahlknecht, Eva Reiter, Susanne Tagwercher, Atbin Djamshidian, Michael Schocke, Regina Esterhammer, Gregor Wenning, Christoph Müller, Christoph Scherfler, Elke R Gizewski, Werner Poewe, Klaus Seppi
INTRODUCTION: Several previous studies examined different brainstem-derived MR planimetric measures with regards to their diagnostic accuracy in separating patients with neurodegenerative parkinsonian disorders and reported conflicting results. The current study aimed to compare their performance in a well-characterized sample of patients with neurodegenerative parkinsonian disorders. METHODS: MR planimetric measurements were assessed in a large retrospective cohort of 55 progressive supranuclear palsy (PSP), 194 Parkinson's disease (PD) and 63 multiple system atrophy (MSA) patients...
October 31, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/29118735/divergent-thinking-in-parkinsonism-a-case-control-study
#18
Margherita Canesi, Maria Luisa Rusconi, Emanuele Cereda, Alessandra Ranghetti, Viviana Cereda, Federica Moroni, Gianni Pezzoli
Background: Creativity is a multidimensional phenomenon and an important component of human capacities. This ability is characterized by the involvement of several cognitive functions particularly linked to the prefrontal cortex. We compared divergent thinking, a measure of creativity, in patients affected by progressive supranuclear palsy (PSP), other parkinsonian syndromes, and healthy controls (HCs). Methods: Creativity features were evaluated using the Abbreviated Torrance Test for Adults (ATTA)...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/29113357/brain-gray-matter-abnormalities-in-progressive-supranuclear-palsy-revisited
#19
PingLei Pan, Yi Liu, Yang Zhang, Hui Zhao, Xing Ye, Yun Xu
Whole-brain voxel-based morphometry (VBM) studies of progressive supranuclear palsy (PSP) have demonstrated heterogeneous findings regarding gray matter (GM) abnormalities. Here, we used Seed-based d Mapping, a coordinate-based meta-analytic approach to identify consistent regions of GM anomalies across studies of PSP. Totally, 18 original VBM studies, comprising 284 patients with PSP and 367 healthy controls were included. As compared to healthy controls, patients with PSP demonstrated significant GM reductions in both cortical and subcortical regions, including the frontal motor cortices, medial (including anterior cingulate cortex) and lateral frontal cortices, insula, superior temporal gyrus, striatum (putamen and caudate nucleus), thalamus, midbrain, and anterior cerebellum...
October 6, 2017: Oncotarget
https://www.readbyqxmd.com/read/29111000/natural-history-and-predictors-of-survival-in-progressive-supranuclear-palsy
#20
Maura Cosseddu, Alberto Benussi, Stefano Gazzina, Marta A Manes, Valentina Dell'Era, Viviana Cristillo, Rosanna Turrone, Antonella Alberici, Barbara Borroni
BACKGROUND: Progressive supranuclear palsy is a neurodegenerative disorder characterized by high functional disability and rapidly progressive dependency. The predictors of survival are still unclear. METHODS: The predictors of survival were evaluated in a group of clinically diagnosed PSP patients, focusing primarily on extensive cognitive assessment. RESULTS: The mean survival time from symptom onset was 8.25±3.0years. Sex, age at onset, education, occupation and severity of extrapyramidal symptoms did not correlate with survival...
November 15, 2017: Journal of the Neurological Sciences
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