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Progressive supranuclear palsy

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https://www.readbyqxmd.com/read/29661694/descriptive-epidemiology-of-parkinsonism-in-the-canton-of-geneva-switzerland
#1
Vanessa Fleury, Pauline Brindel, Nicolas Nicastro, Pierre R Burkhard
OBJECTIVES: A large descriptive cross-sectional population-based prevalence study as well as a retrospective incidence study were undertaken to ascertain the frequency of Parkinson's Disease (PD) and other types of degenerative and non-degenerative parkinsonism in the Canton of Geneva, Switzerland. METHODS: An extensive case-finding approach including records from public hospitals, private neurologists and nursing homes was utilized. All patients with a diagnosis of parkinsonism established between 2003 and 2012 were included...
April 9, 2018: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/29656245/individual-differences-in-socioemotional-sensitivity-are-an-index-of-salience-network-function
#2
Gianina Toller, Jesse Brown, Marc Sollberger, Suzanne M Shdo, Laura Bouvet, Paul Sukhanov, William W Seeley, Bruce L Miller, Katherine P Rankin
Connectivity in intrinsically connected networks (ICNs) may predict individual differences in cognition and behavior. The drastic alterations in socioemotional awareness of patients with behavioral variant frontotemporal dementia (bvFTD) are presumed to arise from changes in one such ICN, the salience network (SN). We examined how individual differences in SN connectivity are reflected in overt social behavior in healthy individuals and patients, both to provide neuroscientific insight into this key brain-behavior relationship, and to provide a practical tool to diagnose patients with early bvFTD...
February 27, 2018: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
https://www.readbyqxmd.com/read/29643007/the-diagnostic-accuracy-of-the-hummingbird-and-morning-glory-sign-in-patients-with-neurodegenerative-parkinsonism
#3
Christoph Mueller, Anna Hussl, Florian Krismer, Beatrice Heim, Philipp Mahlknecht, Michael Nocker, Christoph Scherfler, Katherina Mair, Regina Esterhammer, Michael Schocke, Gregor K Wenning, Werner Poewe, Klaus Seppi
INTRODUCTION: The hummingbird sign and the morning glory flower sign, reflecting midbrain pathology on MRI, have previously been shown to separate patients with progressive supranuclear palsy (PSP) from those with Parkinson's disease (PD) and multiple system atrophy (MSA). The aim of the present study was to determine the diagnostic accuracy and reproducibility of visual assessment of midbrain atrophy patterns in a large cohort of patients with neurodegenerative parkinsonism. METHODS: Retrospective analysis of midbrain atrophy patterns on T1-weighted MRI in a large cohort of patients with neurodegenerative parkinsonism and healthy controls who underwent MR imaging during their diagnostic work-up...
April 3, 2018: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/29622555/mri-planimetry-and-magnetic-resonance-parkinsonism-index-in-the-differential-diagnosis-of-patients-with-parkinsonism
#4
V C Constantinides, G P Paraskevas, G Velonakis, P Toulas, E Stamboulis, E Kapaki
BACKGROUND AND PURPOSE: Differential diagnosis of multiple system atrophy, progressive supranuclear palsy, and corticobasal degeneration from Parkinson disease on clinical grounds is often difficult. MR imaging biomarkers could assist in a more accurate diagnosis. We examined the utility of MR imaging surface measurements (MR imaging planimetry) in the differential diagnosis of patients with parkinsonism. MATERIALS AND METHODS: Fifty-two patients with Parkinson-plus (progressive supranuclear palsy, n = 24; corticobasal degeneration, n = 9; multiple system atrophy, n = 19), 18 patients with Parkinson disease, and 15 healthy controls were included...
April 5, 2018: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/29621183/tau-induced-pathology-in-epilepsy-and-dementia-notions-from-patients-and-animal-models
#5
REVIEW
Marina P Sánchez, Ana M García-Cabrero, Gentzane Sánchez-Elexpuru, Daniel F Burgos, José M Serratosa
Patients with dementia present epilepsy more frequently than the general population. Seizures are more common in patients with Alzheimer's disease (AD), dementia with Lewy bodies (LBD), frontotemporal dementia (FTD) and progressive supranuclear palsy (PSP) than in other dementias. Missense mutations in the microtubule associated protein tau (MAPT) gene have been found to cause familial FTD and PSP, while the P301S mutation in MAPT has been associated with early-onset fast progressive dementia and the presence of seizures...
April 5, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29608591/novel-monoclonal-antibodies-targeting-the-microtubule-binding-domain-of-human-tau
#6
Cara L Croft, Brenda D Moore, Yong Ran, Paramita Chakrabarty, Yona Levites, Todd E Golde, Benoit I Giasson
Tauopathies including Alzheimer's disease and Progressive Supranuclear Palsy are a diverse group of progressive neurodegenerative disorders pathologically defined by inclusions containing aberrantly aggregated, post-translationally modified tau. The tau pathology burden correlates with neurodegeneration and dementia observed in these diseases. The microtubule binding domain of tau is essential for its physiological functions in promoting neuronal cytoskeletal stability, however it is also required for tau to assemble into an amyloid structure that comprises pathological inclusions...
2018: PloS One
https://www.readbyqxmd.com/read/29602288/preclinical-phase-i-and-phase-ii-investigational-clinical-trials-for-treatment-of-progressive-supranuclear-palsy
#7
Ali Shoeibi, Nahid Olfati, Irene Litvan
Our understanding of the pathological basis of progressive supranuclear palsy (PSP), as the most common atypical parkinsonian syndrome, has greatly increased in recent years and a number of disease-modifying therapies are under evaluation as a result of these advances. Areas covered: In this review, we discuss disease-modifying therapeutic options which are currently under evaluation or have been evaluated in preclinical or clinical trials based on their targeted pathophysiologic process. The pathophysiologic mechanisms are broadly divided into three main categories: genetic mechanisms, abnormal post-translational modifications of tau protein, and transcellular tau spread...
April 2018: Expert Opinion on Investigational Drugs
https://www.readbyqxmd.com/read/29574085/combined-measurement-of-plasma-cystatin-c-and-low-density-lipoprotein-cholesterol-a-valuable-tool-for-evaluating-progressive-supranuclear-palsy
#8
Ruihui Weng, Xiaobo Wei, Bin Yu, Shuzhen Zhu, Xiaohua Yang, Fen Xie, Mahui Zhang, Ying Jiang, Zhong-Ping Feng, Hong-Shuo Sun, Ying Xia, Kunlin Jin, Piu Chan, Qing Wang, Xiaoya Gao
INTRODUCTION: Progressive supranuclear palsy (PSP) was previously thought as a cause of atypical Parkinsonism. Although Cystatin C (Cys C) and low-density cholesterol lipoprotein-C (LDL-C) are known to play critical roles in Parkinsonism, it is unknown whether they can be used as markers to distinguish PSP patients from healthy subjects and to determine disease severity. METHODS: We conducted a cross-sectional study to determine plasma Cys C/HDL/LDL-C levels of 40 patients with PSP and 40 healthy age-matched controls...
March 19, 2018: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/29562525/rab10-phosphorylation-is-a-prominent-pathological-feature-in-alzheimer-s-disease
#9
Tingxiang Yan, Luwen Wang, Ju Gao, Sandra L Siedlak, Mikayla L Huntley, Pichet Termsarasab, George Perry, Shu G Chen, Xinglong Wang
Alzheimer's disease (AD) is the leading cause of dementia in the elderly, characterized by neurofibrillary tangles (NFTs), senile plaques (SPs), and a progressive loss of neuronal cells in selective brain regions. Rab10, a small Rab GTPase involved in vesicular trafficking, has recently been identified as a novel protein associated with AD. Interestingly, Rab10 is a key substrate of leucine-rich repeat kinase 2 (LRRK2), a serine/threonine protein kinase genetically associated with the second most common neurodegenerative disease Parkinson's disease...
March 16, 2018: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29554190/a-2-step-cerebrospinal-algorithm-for-the-selection-of-frontotemporal-lobar-degeneration-subtypes
#10
Alberto Lleó, David J Irwin, Ignacio Illán-Gala, Corey T McMillan, David A Wolk, Edward B Lee, Vivianna M Van Deerlin, Leslie M Shaw, John Q Trojanowski, Murray Grossman
Importance: Cerebrospinal fluid (CSF) core Alzheimer disease (AD) biomarkers have shown an excellent capacity for the in vivo detection of AD. Previous studies have shown that CSF levels of phosphorylated tau (p-tau) also correlate with tau pathology in frontotemporal lobar degeneration (FTLD) after accounting for AD copathology. Objective: To develop an algorithm based on core AD CSF measures to exclude cases with AD pathology and then differentiate between FTLD-tau and FTLD transactive response DNA-binding protein of approximately 43kDa (FTLD-TDP)...
March 19, 2018: JAMA Neurology
https://www.readbyqxmd.com/read/29536636/reproducible-network-and-regional-topographies-of-abnormal-glucose-metabolism-associated-with-progressive-supranuclear-palsy-multivariate-and-univariate-analyses-in-american-and-chinese-patient-cohorts
#11
Jingjie Ge, Jianjun Wu, Shichun Peng, Ping Wu, Jian Wang, Huiwei Zhang, Yihui Guan, David Eidelberg, Chuantao Zuo, Yilong Ma
Progressive supranuclear palsy (PSP) is a rare movement disorder and often difficult to distinguish clinically from Parkinson's disease (PD) and multiple system atrophy (MSA) in early phases. In this study, we report reproducible disease-related topographies of brain network and regional glucose metabolism associated with PSP in clinically-confirmed independent cohorts of PSP, MSA, and PD patients and healthy controls in the USA and China. Using18 F-FDG PET images from PSP and healthy subjects, we applied spatial covariance analysis with bootstrapping to identify a PSP-related pattern (PSPRP) and estimate its reliability, and evaluated the ability of network scores for differential diagnosis...
March 13, 2018: Human Brain Mapping
https://www.readbyqxmd.com/read/29525296/further-evidence-for-a-distinctive-atypical-degenerative-parkinsonism-in-the-caribbean-a-new-cluster-in-the-french-west-indian-island-of-martinique
#12
Annie Lannuzel, Régine Edragas, Angéla Lackmy, Benoit Tressières, Véronique Pelonde, Mireille Edimo Nana Kaptué, Sylvie Mécharles, Alexis Demas, Billy François, Eavan McGovern, Marie Vidailhet, Bertrand Gaymard, Emmanuel Roze
BACKGROUND: A high prevalence of an atypical levodopa-resistant parkinsonism has been reported in the Caribbean island of Guadeloupe. These seminal observations have not been replicated or extended to neighbouring populations who share genetic and environmental characteristics. METHODS: To further characterise this atypical parkinsonism we prospectively investigated 305 consecutive patients with neurodegenerative parkinsonism in a community-based population from Guadeloupe and Martinique, a neighbouring French Caribbean island where the population has similar environmental and genetic backgrounds...
February 6, 2018: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29514947/clinical-value-of-neurofilament-and-phospho-tau-tau-ratio-in-the-frontotemporal-dementia-spectrum
#13
Lieke H H Meeter, Everard G Vijverberg, Marta Del Campo, Annemieke J M Rozemuller, Laura Donker Kaat, Frank Jan de Jong, Wiesje M van der Flier, Charlotte E Teunissen, John C van Swieten, Yolande A L Pijnenburg
OBJECTIVE: To examine the clinical value of neurofilament light chain (NfL) and the phospho-tau/total tau ratio (p/t-tau) across the entire frontotemporal dementia (FTD) spectrum in a large, well-defined cohort. METHODS: CSF NfL and p/t-tau levels were studied in 361 patients with FTD: 179 behavioral variant FTD, 17 FTD with motor neuron disease (FTD-MND), 36 semantic variant primary progressive aphasia (PPA), 19 nonfluent variant PPA, 4 logopenic variant PPA (lvPPA), 42 corticobasal syndrome, and 64 progressive supranuclear palsy...
April 3, 2018: Neurology
https://www.readbyqxmd.com/read/29503154/deep-brain-stimulation-of-the-pedunculopontine-nucleus-for-treatment-of-gait-and-balance-disorder-in-progressive-supranuclear-palsy-effects-of-frequency-modulations-and-clinical-outcome
#14
Imke Galazky, Jörn Kaufmann, Stefan Lorenzl, Georg Ebersbach, Florin Gandor, Tino Zaehle, Sylke Specht, Sabine Stallforth, Uwe Sobieray, Edyta Wirkus, Franziska Casjens, Hans-Jochen Heinze, Andreas Kupsch, Jürgen Voges
BACKGROUND: The pedunculopontine nucleus has been suggested as a potential deep brain stimulation target for axial symptoms such as gait and balance impairment in idiopathic Parkinson's disease as well as atypical Parkinsonian disorders. METHODS: Seven consecutive patients with progressive supranuclear palsy received bilateral pedunculopontine nucleus deep brain stimulation. Inclusion criteria comprised of the clinical diagnosis of progressive supranuclear palsy, a levodopa-resistant gait and balance disorder, age <75 years, and absence of dementia or major psychiatric co-morbidities...
February 19, 2018: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/29502334/microtubule-defects-in-mesenchymal-stromal-cells-distinguish-patients-with-progressive-supranuclear-palsy
#15
Alessandra Maria Calogero, Mariele Viganò, Silvia Budelli, Daniela Galimberti, Chiara Fenoglio, Daniele Cartelli, Lorenza Lazzari, Petri Lehenkari, Margherita Canesi, Rosaria Giordano, Graziella Cappelletti, Gianni Pezzoli
Progressive Supranuclear Palsy (PSP) is a rare neurodegenerative disease whose etiopathogenesis remains elusive. The intraneuronal accumulation of hyperphosphorylated Tau, a pivotal protein in regulating microtubules (MT), leads to include PSP into tauopathies. Pathological hallmarks are well known in neural cells but no word yet if PSP-linked dysfunctions occur also in other cell types. We focused on bone marrow mesenchymal stromal cells (MSCs) that have recently gained attention for therapeutic interventions due to their anti-inflammatory, antiapoptotic and trophic properties...
March 4, 2018: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/29499916/dctn1-f52l-mutation-case-of-perry-syndrome-with-progressive-supranuclear-palsy-like-tauopathy
#16
Hiroyuki Honda, Naokazu Sasagasako, Chang Shen, Masahiro Shijo, Hideomi Hamasaki, Satoshi O Suzuki, Yoshio Tsuboi, Naoki Fujii, Toru Iwaki
INTRODUCTION: Perry syndrome is a rapidly progressive, autosomal dominant parkinsonism characterized by central hypoventilation, depression and severe weight loss. To date, eight DCTN1 mutations have been identified associated with Perry syndrome. A novel F52L DCTN1 mutation case of Perry syndrome is characterized by late-onset parkinsonism and frontotemporal atrophy. METHODS: A Japanese woman suffered from slowly progressing parkinsonism since age 48. At age 59, she developed central hypoventilation, and required breathing assistance...
February 23, 2018: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/29496136/minimal-neuropathologic-diagnosis-for-brain-banking-in-the-normal-middle-aged-and-aged-brain-and-in-neurodegenerative-disorders
#17
Irina Alafuzoff
Research on human brain diseases is currently often conducted on cell cultures and animals. Several questions however can only be addressed by studying human postmortem brain tissue. However, brain tissue obtained postmortem almost always displays pathology that is often related to the aging phenomenon. Thus, in order to be certain that the answers obtained are reliable, a systematic and thorough assessment of the brain tissue to be studied should be carried out. We are currently aware of several protein alterations that are found in middle-aged and aged brains that are obtained from neurologically unimpaired subjects...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29480228/urinary-dysfunctions-and-post-void-residual-urine-in-typical-and-atypical-parkinson-diseases
#18
Yang-Hyun Lee, Jee-Eun Lee, Dong-Woo Ryu, Yoon-Sang Oh, Kwang-Soo Lee, Sung-Hoo Hong, Joong-Seok Kim
BACKGROUND: Urinary dysfunction is relatively common, however, is often underestimated and diagnosed by subjective questionnaires in patients with Parkinsonism. OBJECTIVE: To determine the correlation between subjective urinary dysfunction and post-void residual urine volume in patients with Parkinson's disease (PD), multiple system atrophy (MSA) and progressive supranuclear palsy (PSP), and to assess the relationship between clinical characteristics and subjective or objective urinary dysfunction...
2018: Journal of Parkinson's Disease
https://www.readbyqxmd.com/read/29480226/lrrk2-p-ile1371val-mutation-in-a-case-with-neuropathologically-confirmed-multi-system-atrophy
#19
Kelsey Lee, Khanh-Dung Nguyen, Chao Sun, Mei Liu, Faria Zafar, Jimmy Saetern, Adrian Flierl, James W Tetrud, J William Langston, Dennis Dickson, Birgitt Schüle
BACKGROUND: Mutations in the leucine rich repeat kinase 2 (LRRK2) gene are among the most common genetic causes of Lewy body Parkinson's disease (PD). However, LRRK2 mutations can also lead to a variety of pathological phenotypes other than typical PD, including relatively pure nigrostriatal cell loss without alpha-synuclein-positive Lewy bodies or Lewy neurites, progressive supranuclear palsy (PSP), and multiple system atrophy (MSA). The mechanisms behind this remarkable pleomorphic pathology are currently unclear...
2018: Journal of Parkinson's Disease
https://www.readbyqxmd.com/read/29478699/%C3%AE-synuclein-oligomers-induce-a-unique-toxic-tau-strain
#20
Diana L Castillo-Carranza, Marcos J Guerrero-Muñoz, Urmi Sengupta, Julia E Gerson, Rakez Kayed
BACKGROUND: The coexistence of α-synuclein and tau aggregates in several neurodegenerative disorders, including Parkinson's disease and Alzheimer's disease, raises the possibility that a seeding mechanism is involved in disease progression. METHODS: To further investigate the role of α-synuclein in the tau aggregation pathway, we performed a set of experiments using both recombinant and brain-derived tau and α-synuclein oligomers to seed monomeric tau aggregation in vitro and in vivo...
January 17, 2018: Biological Psychiatry
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