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Progressive supranuclear palsy

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https://www.readbyqxmd.com/read/28341224/palliative-care-and-its-emerging-role-in-multiple-system-atrophy-and-progressive-supranuclear-palsy
#1
REVIEW
Louise Wiblin, Mark Lee, David Burn
Multiple System Atrophy (MSA) and Progressive Supranuclear Palsy (PSP) are sporadic, neurodegenerative conditions and belong to a group known as the atypical Parkinsonian disorders. The atypical Parkinsonian (AP) disorders have some features of idiopathic Parkinson's disease (PD) but often with poor or transient levodopa response [1], distinct symptom profiles, more rapid progression and reduced survival. They have significant symptom burden [2]. It is beginning to be appreciated that AP disorders not only carry a heavy symptom burden equivalent to that of patients with advanced cancer but that AP patients experience significant morbidity for an extended period of time...
January 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/28334843/a152t-tau-allele-causes-neurodegeneration-that-can-be-ameliorated-in-a-zebrafish-model-by-autophagy-induction
#2
Ana Lopez, Suzee E Lee, Kevin Wojta, Eliana Marisa Ramos, Eric Klein, Jason Chen, Adam L Boxer, Maria Luisa Gorno-Tempini, Daniel H Geschwind, Lars Schlotawa, Nikolay V Ogryzko, Eileen H Bigio, Emily Rogalski, Sandra Weintraub, Marsel M Mesulam, Angeleen Fleming, Giovanni Coppola, Bruce L Miller, David C Rubinsztein
Mutations in the gene encoding tau (MAPT) cause frontotemporal dementia spectrum disorders. A rare tau variant p.A152T was reported as a risk factor for frontotemporal dementia spectrum and Alzheimer's disease in an initial case-control study. Such findings need replication in an independent cohort. We analysed an independent multinational cohort comprising 3100 patients with neurodegenerative disease and 4351 healthy control subjects and found p.A152T associated with significantly higher risk for clinically defined frontotemporal dementia and progressive supranuclear palsy syndrome...
February 9, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28326008/disease-specific-regions-outperform-whole-brain-approaches-in-identifying-progressive-supranuclear-palsy-a-multicentric-mri-study
#3
Karsten Mueller, Robert Jech, Cecilia Bonnet, Jaroslav Tintěra, Jaromir Hanuška, Harald E Möller, Klaus Fassbender, Albert Ludolph, Jan Kassubek, Markus Otto, Evžen Růžička, Matthias L Schroeter
To identify progressive supranuclear palsy (PSP), we combined voxel-based morphometry (VBM) and support vector machine (SVM) classification using disease-specific features in multicentric magnetic resonance imaging (MRI) data. Structural brain differences were investigated at four centers between 20 patients with PSP and 20 age-matched healthy controls with T1-weighted MRI at 3T. To pave the way for future application in personalized medicine, we applied SVM classification to identify PSP on an individual level besides group analyses based on VBM...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28322905/bilingualism-delays-the-onset-of-behavioral-but-not-aphasic-forms-of-frontotemporal-dementia
#4
Suvarna Alladi, Thomas H Bak, Mekala Shailaja, Divyaraj Gollahalli, Amuya Rajan, Bapiraju Surampudi, Michael Hornberger, Vasanta Duggirala, Jaydip Ray Chaudhuri, Subhash Kaul
Bilingualism has been found to delay onset of dementia and this has been attributed to an advantage in executive control in bilinguals. However, the relationship between bilingualism and cognition is complex, with costs as well as benefits to language functions. To further explore the cognitive consequences of bilingualism, the study used Frontotemporal dementia (FTD) syndromes, to examine whether bilingualism modifies the age at onset of behavioral and language variants of Frontotemporal dementia (FTD) differently...
March 18, 2017: Neuropsychologia
https://www.readbyqxmd.com/read/28318985/functional-disconnection-of-thalamic-and-cerebellar-dentate-nucleus-networks-in-progressive-supranuclear-palsy-and-corticobasal-syndrome
#5
Neeraj Upadhyay, Antonio Suppa, Maria Cristina Piattella, Costanza Giannì, Matteo Bologna, Flavio Di Stasio, Nikolaos Petsas, Francesca Tona, Giovanni Fabbrini, Alfredo Berardelli, Patrizia Pantano
AIM: To assess functional rearrangement following neurodegeneration in the thalamus and dentate nucleus in patients with progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). METHODS: We recruited 19 patients with PSP, 11 with CBS and 14 healthy subjects. All the subjects underwent resting-state (rs) fMRI using a 3T system. Whole brain functional connectivity of the thalamus and dentate nucleus were calculated by means of a seed-based approach with FEAT script in FSL toolbox...
March 15, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/28315409/in-vivo-tau-pet-imaging-in-dementia-pathophysiology-radiotracer-quantification-and-a-systematic-review-of-clinical-findings
#6
REVIEW
Benjamin Hall, Elijah Mak, Simon Cervenka, Franklin I Aigbirhio, James B Rowe, John T O'Brien
In addition to the deposition of β-amyloid plaques, neurofibrillary tangles composed of aggregated hyperphosphorylated tau are one of the pathological hallmarks of Alzheimer's disease and other neurodegenerative disorders. Until now, our understanding about the natural history and topography of tau deposition has only been based on post-mortem and cerebrospinal fluid studies, and evidence continues to implicate tau as a central driver of downstream neurodegenerative processes and cognitive decline. Recently, it has become possible to assess the regional distribution and severity of tau burden in vivo with the development of novel radiotracers for positron emission tomography (PET) imaging...
March 15, 2017: Ageing Research Reviews
https://www.readbyqxmd.com/read/28293793/ultrasensitive-and-selective-detection-of-3-repeat-tau-seeding-activity-in-pick-disease-brain-and-cerebrospinal-fluid
#7
Eri Saijo, Bernardino Ghetti, Gianluigi Zanusso, Adrian Oblak, Jennifer L Furman, Marc I Diamond, Allison Kraus, Byron Caughey
The diagnosis and treatment of diseases involving tau-based pathology such as Alzheimer disease and certain frontotemporal dementias is hampered by the inability to detect pathological forms of tau with sufficient sensitivity, specificity and practicality. In these neurodegenerative diseases, tau accumulates in self-seeding filaments. For example, Pick disease (PiD) is associated with frontotemporal degeneration and accumulation of 3-repeat (3R) tau isoforms in filaments constituting Pick bodies. Exploiting the self-seeding activity of tau deposits, and using a 3R tau fragment as a substrate, we have developed an assay (tau RT-QuIC) that can detect tau seeds in 2 µl aliquots of PiD brain dilutions down to 10(-7)-10(-9)...
March 14, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28291592/white-matter-and-cortical-changes-in-atypical-parkinsonisms-a-multimodal-quantitative-mr-study
#8
Stefano Zanigni, Stefania Evangelisti, Claudia Testa, David N Manners, Giovanna Calandra-Buonaura, Maria Guarino, Anna Gabellini, Laura Ludovica Gramegna, Giulia Giannini, Luisa Sambati, Pietro Cortelli, Raffaele Lodi, Caterina Tonon
OBJECTIVES: To evaluate white matter and cortical changes in patients with parkinsonisms and healthy controls (HC), applying both hypothesis-free and regions of interest (ROI)-based advanced brain MR analyses. METHODS: Twenty-five patients with Progressive Supranuclear Palsy - Richardson's Syndrome (PSP-RS), nine with cerebellar and nine with parkinsonian Multiple System Atrophy variants (MSA-C and MSA-P), forty-seven with Parkinson's Disease (PD) and twenty-seven HC underwent a 1...
March 6, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/28276873/the-hidden-niemann-pick-type-c-patient-clinical-niches-for-a-rare-inherited-metabolic-disease
#9
Christian J Hendriksz, Mathieu Anheim, Peter Bauer, Olivier Bonnot, Anupam Chakrapani, Jean-Christophe Corvol, Tom J de Koning, Anna Degtyareva, Carlo Dionisi-Vici, Sarah Doss, Thomas Duning, Paola Giunti, Rosa Iodice, Tracy Johnston, Dierdre Kelly, Hans-Hermann Klünemann, Stefan Lorenzl, Alessandro Padovani, Miguel Pocovi, Matthis Synofzik, Alta Terblanche, Florian Then Bergh, Meral Topçu, Christine Tranchant, Mark Walterfang, Christian Velten, Stefan A Kolb
BACKGROUND: Niemann-Pick disease type C (NP-C) is a rare, inherited neurodegenerative disease of impaired intracellular lipid trafficking. Clinical symptoms are highly heterogeneous, including neurological, visceral, or psychiatric manifestations. The incidence of NP-C is under-estimated due to under-recognition or misdiagnosis across a wide range of medical fields. New screening and diagnostic methods provide an opportunity to improve detection of unrecognized cases in clinical sub-populations associated with a higher risk of NP-C...
March 2, 2017: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/28271184/shared-genetic-risk-between-corticobasal-degeneration-progressive-supranuclear-palsy-and-frontotemporal-dementia
#10
Jennifer S Yokoyama, Celeste M Karch, Chun C Fan, Luke W Bonham, Naomi Kouri, Owen A Ross, Rosa Rademakers, Jungsu Kim, Yunpeng Wang, Günter U Höglinger, Ulrich Müller, Raffaele Ferrari, John Hardy, Parastoo Momeni, Leo P Sugrue, Christopher P Hess, A James Barkovich, Adam L Boxer, William W Seeley, Gil D Rabinovici, Howard J Rosen, Bruce L Miller, Nicholas J Schmansky, Bruce Fischl, Bradley T Hyman, Dennis W Dickson, Gerard D Schellenberg, Ole A Andreassen, Anders M Dale, Rahul S Desikan
Corticobasal degeneration (CBD), progressive supranuclear palsy (PSP) and a subset of frontotemporal dementia (FTD) are neurodegenerative disorders characterized by tau inclusions in neurons and glia (tauopathies). Although clinical, pathological and genetic evidence suggests overlapping pathobiology between CBD, PSP, and FTD, the relationship between these disorders is still not well understood. Using summary statistics (odds ratios and p values) from large genome-wide association studies (total n = 14,286 cases and controls) and recently established genetic methods, we investigated the genetic overlap between CBD and PSP and CBD and FTD...
March 7, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28268209/patterns-of-eye-movement-impairment-correlate-with-regional-brain-atrophy-in-neurodegenerative-parkinsonism
#11
Olga Vintonyak, Martin Gorges, Hans-Peter Müller, Elmar H Pinkhardt, Albert C Ludolph, Hans-Jürgen Huppertz, Jan Kassubek
BACKGROUND: One common feature of neurodegenerative parkinsonism including Parkinson's disease (PD), multisystem atrophy (MSA), and progressive supranuclear palsy (PSP) is altered eye movement control. Characteristic regional structural atrophy patterns in MRI can be observed in PD, MSA, and PSP. OBJECTIVE: To investigate the association between eye movement disturbances and regional brain atrophy in patients with PD, MSA, and PSP. METHODS: High-resolution 3-dimensional T1-weighted MRI images and video-oculographic recordings (EyeLink®) were obtained from 39 PD, 32 PSP, and 18 MSA patients and 24 matched healthy control subjects...
March 8, 2017: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/28268100/intrafamilial-variable-phenotype-including-corticobasal-syndrome-in-a-family-with-p-p301l-mutation-in-the-mapt-gene-first-report-in-south-america
#12
Emilia M Gatto, Ricardo F Allegri, Gustavo Da Prat, Patricio Chrem Mendez, David S Hanna, Michael O Dorschner, Ezequiel I Surace, Cyrus P Zabetian, Ignacio F Mata
Frontotemporal lobar degeneration is a neuropathological disorder that causes a variety of clinical syndromes including frontotemporal dementia (FTD), progressive supranuclear palsy, and corticobasal syndrome (CBS). FTD associated with parkinsonism occurs frequently as a result of mutations in the C9orf72 gene and also in the genes coding for the protein associated with microtubule tau (MAPT) and progranulin (GRN) on chromosome 17 (FTDP-17). Herein, we report an Argentinean family, of Basque ancestry, with an extensive family history of behavioral variant of FTD...
February 10, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28267762/impaired-perception-of-surface-tilt-in-progressive-supranuclear-palsy
#13
Marian L Dale, Fay B Horak, W Geoffrey Wright, Bernadette M Schoneburg, John G Nutt, Martina Mancini
INTRODUCTION: Progressive supranuclear palsy (PSP) is characterized by early postural instability and backward falls. The mechanisms underlying backward postural instability in PSP are not understood. The aim of this study was to test the hypothesis that postural instability in PSP is a result of dysfunction in the perception of postural verticality. METHODS: We gathered posturography data on 12 subjects with PSP to compare with 12 subjects with idiopathic Parkinson's Disease (PD) and 12 healthy subjects...
2017: PloS One
https://www.readbyqxmd.com/read/28256434/pathologic-correlates-of-supranuclear-gaze-palsy-with-parkinsonism
#14
W R W Martin, J Hartlein, B A Racette, N Cairns, J S Perlmutter
INTRODUCTION: Supranuclear gaze palsy (SGP) is a classic clinical feature of progressive supranuclear palsy (PSP) but is not specific for this diagnosis and has been reported to occur in several other neurodegenerative parkinsonian conditions. Our objective was to evaluate the association between SGP and autopsy-proven diagnoses in a large population of patients with parkinsonism referred to a tertiary movement disorders clinic. METHODS: We reviewed clinical and autopsy data maintained in an electronic medical record from all patients seen in the Movement Disorders Clinic at Washington University, St...
February 24, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/28250027/predictors-of-survival-in-progressive-supranuclear-palsy-and-multiple-system-atrophy-a-systematic-review-and-meta-analysis
#15
Stella Andrea Glasmacher, Peter Nigel Leigh, Romi Anirban Saha
OBJECTIVE: To undertake a systematic review and meta-analysis of studies that investigated prognostic factors and survival in patients with progressive supranuclear palsy (PSP) and multiple system atrophy (MSA). METHODS: Publications of at least 10 patients with a likely or confirmed diagnosis of PSP or MSA were eligible for inclusion. Methodological quality was rated using a modified version of the Quality in Prognostic Studies tool. For frequently examined prognostic factors, HRs derived by univariate and multivariate analysis were pooled in separate subgroups; other results were synthesised narratively and HRs could not be reported here...
March 1, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28246328/synaptic-vesicle-glycoprotein-2c-sv2c-modulates-dopamine-release-and-is-disrupted-in-parkinson-disease
#16
Amy R Dunn, Kristen A Stout, Minagi Ozawa, Kelly M Lohr, Carlie A Hoffman, Alison I Bernstein, Yingjie Li, Minzheng Wang, Carmelo Sgobio, Namratha Sastry, Huaibin Cai, W Michael Caudle, Gary W Miller
Members of the synaptic vesicle glycoprotein 2 (SV2) family of proteins are involved in synaptic function throughout the brain. The ubiquitously expressed SV2A has been widely implicated in epilepsy, although SV2C with its restricted basal ganglia distribution is poorly characterized. SV2C is emerging as a potentially relevant protein in Parkinson disease (PD), because it is a genetic modifier of sensitivity to l-DOPA and of nicotine neuroprotection in PD. Here we identify SV2C as a mediator of dopamine homeostasis and report that disrupted expression of SV2C within the basal ganglia is a pathological feature of PD...
March 14, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28244178/evaluation-of-an-optimized-18-f-fluoro-deoxy-glucose-positron-emission-tomography-voxel-wise-method-to-early-support-differential-diagnosis-in-atypical-parkinsonian-disorders
#17
S P Caminiti, P Alongi, L Majno, M A Volontè, C Cerami, L Gianolli, G Comi, D Perani
BACKGROUND AND PURPOSE: Atypical Parkinsonian disorders (APD) frequently overlap in clinical presentations, making the differential diagnosis challenging in the early stages. The present study aimed to evaluate the accuracy of the [(18) F]fluoro-deoxy-glucose positron emission tomography Statistical Parametric Mapping (SPM) optimized procedure in supporting the early and differential diagnosis of APD. METHODS: Seventy patients with possible APD were retrospectively included from a large clinical cohort...
February 28, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28243754/differentiation-of-atypical-parkinson-syndromes
#18
REVIEW
Günter U Höglinger, Jan Kassubek, Ilona Csoti, Reinhard Ehret, Heinz Herbst, Ingmar Wellach, Jürgen Winkler, Wolfgang H Jost
In distinction to idiopathic Parkinson's disease (PD), the diagnosis of atypical Parkinson syndromes comprises dementia with Lewy bodies (DLB), multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD). We set out to write a state-of-the-art guideline as to which investigations and examinations help to differentiate PD vs. atypical Parkinson syndromes in clinical routine.
February 27, 2017: Journal of Neural Transmission
https://www.readbyqxmd.com/read/28239526/weight-loss-in-the-early-stage-of-progressive-supranuclear-palsy
#19
Ayako Tsuge, Satoshi Kaneko, Reika Wate, Mitsuaki Oki, Masato Nagashima, Shinya Asayama, Masataka Nakamura, Kengo Fujita, Akemi Saito, Norihiro Takenouchi, Hirofumi Kusaka
OBJECTIVES: To clarify whether weight change in patients with Parkinson's disease (PD) or progressive supranuclear palsy (PSP) is caused by the disease itself or secondarily by other factors. MATERIALS AND METHODS: We conducted a retrospective analysis of 51 patients with PD and 14 patients with PSP, especially during the early stage of their diseases. All patients were independent in terms of their activities of daily living and did not have any feeding difficulty...
February 2017: Brain and Behavior
https://www.readbyqxmd.com/read/28236526/-atypical-atypical-parkinsonism-critical-appraisal-of-a-cohort
#20
Stephanie T Hirschbichler, Roberto Erro, Christos Ganos, Maria Stamelou, Amit Batla, Bettina Balint, Kailash P Bhatia
BACKGROUND: Atypical parkinsonian conditions such as multiple system atrophy (MSA), progressive supranuclear palsy (PSP), corticobasal syndrome (CBS) and Dementia with Lewy bodies (DLB) comprise 10-15% of parkinsonian syndromes. Misdiagnosis with Parkinson disease (PD) and within the entities is common, given the absence of reliable biomarkers. However a correct diagnosis is not only important in clinical practice, but also crucial for any trial attempting to identify biomarkers or new treatments...
December 14, 2016: Parkinsonism & related Disorders
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