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Progressive Supranuclear Palsy

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An enigmatic case of cortical anopsia: Antemortem diagnosis of a 14-3-3 negative Heidenhain-variant MM1-sCJD.

Julius Obergassel, Lisa Lohmann, Sven G Meuth, Heinz Wiendl, Oliver Grauer, Christopher Nelke,

Sporadic Creutzfeldt-Jakob disease is the predominant type of human prion disease. While routine diagnostic in phenotypic cases has advanced considerably, the clinical heterogeneity and rarity of subtypes continue to constitute a major clinical and diagnostic challenge. Here, we report a peculiar case of the Heidenhain-variant of MM1 sporadic Creutzfeldt-Jakob disease ... Read more >>

Prion (Prion)
[2020, 14(1):24-28]

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The Non-Motor Symptom Profile of Progressive Supranuclear Palsy.

Sudhakar Pushpa Chaithra, Shweta Prasad, Vikram Venkappayya Holla, Albert Stezin, Nitish Kamble, Ravi Yadav, Pramod Kumar Pal,

Objective:Non-motor symptoms (NMSs) significantly contribute to increased morbidity and poor quality of life in patients with parkinsonian disorders. This study aims to explore the profile of NMSs in patients with progressive supranuclear palsy (PSP) using the validated Non-Motor Symptom Scale (NMSS). Methods:Seventy-six patients with PSP were evaluated in this study. ... Read more >>

J Mov Disord (Journal of movement disorders)
[2020, :]

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Gradient subthalamic neurodegeneration and tau pathology in the hypoglossal nucleus as essential pathological markers of progressive supranuclear palsy - Richardson syndrome.

T Homma, Y Mochizuki, M Hara, S Kamei, T Mizutani, H Takubo, E Isozaki, M Takahashi, T Komori, H Hao,

Progressive supranuclear palsy - Richardson syndrome (PSP-RS) was first described in 1964 by Steele et al. Tau pathology has not been reported in the hypoglossal nuclei of PSP-RS patients, whereas Steele et al. described gliosis with no remarkable neuronal losses in the hypoglossal nucleus. This study aimed to investigate the distribution and ... Read more >>

Rev. Neurol. (Paris) (Revue neurologique)
[2020, :]

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Progressive Supranuclear Palsy and Statin Use.

Ece Bayram, Connie Marras, David G Standaert, Benzi M Kluger, Yvette M Bordelon, David R Shprecher, Irene Litvan, ,

INTRODUCTION:Statins were proposed to be neuroprotective; however, the effects are unknown in progressive supranuclear palsy (PSP), a pure tauopathy. METHODS:Data of 284 PSP cases and 284 age-matched, sex-matched, and race-matched controls were obtained from the environmental and genetic PSP (ENGENE-PSP) study. Cases were evaluated with the PSP Rating Scale, Unified ... Read more >>

Mov. Disord. (Movement disorders : official journal of the Movement Disorder Society)
[2020, :]

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Tau Imaging in the 4-Repeat-Tauopathies Progressive Supranuclear Palsy and Corticobasal Syndrome: A 11C-Pyridinyl-Butadienyl-Benzothiazole 3 PET Pilot Study.

Nils Schröter, Ganna Blazhenets, Lars Frings, Christoph Barkhausen, Wolfgang H Jost, Cornelius Weiller, Michel Rijntjes, Philipp T Meyer,

BACKGROUND AND OBJECTIVES:To evaluate tau PET using C-pyridinyl-butadienyl-benzothiazole 3 (C-PBB3) in the 4-repeat (4R)-tauopathies progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). METHODS:Retrospective analysis of C-PBB3 PET in 2, 7, and 2 patients with CBS, PSP, and Alzheimer dementia (AD), respectively. Normalized C-PBB3 uptake in clusters with significant hypometabolism on ... Read more >>

Clin Nucl Med (Clinical nuclear medicine)
[2020, 45(4):283-287]

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Midbrain/pons area ratio and clinical features predict the prognosis of progressive Supranuclear palsy.

Shi-Shuang Cui, Hua-Wei Ling, Juan-Juan Du, Yi-Qi Lin, Jing Pan, Hai-Yan Zhou, Gang Wang, Ying Wang, Qin Xiao, Jun Liu, Yu-Yan Tan, Sheng-Di Chen,

BACKGROUND:Progressive supranuclear palsy (PSP) is a rare movement disorder with poor prognosis. This retrospective study aimed to characterize the natural history of PSP and to find predictors of shorter survival and faster decline of activity of daily living. METHOD:All patients recruited fulfilled the movement disorder society (MDS) clinical diagnostic criteria ... Read more >>

BMC Neurol (BMC neurology)
[2020, 20(1):114]

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Generation and Characterization of Novel Monoclonal Antibodies Targeting p62/sequestosome-1 Across Human Neurodegenerative Diseases.

Jorge A Trejo-Lopez, Zachary A Sorrentino, Cara J Riffe, Stefan Prokop, Dennis W Dickson, Anthony T Yachnis, Benoit I Giasson,

Human neurodegenerative diseases can be characterized as disorders of protein aggregation. As a key player in cellular autophagy and the ubiquitin proteasome system, p62 may represent an effective immunohistochemical target, as well as mechanistic operator, across neurodegenerative proteinopathies. In this study, 2 novel mouse-derived monoclonal antibodies 5G3 and 2A5 raised ... Read more >>

J. Neuropathol. Exp. Neurol. (Journal of neuropathology and experimental neurology)
[2020, 79(4):407-418]

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Midbrain/pons area ratio and clinical features predict the prognosis of Progressive Supranuclear Palsy

shengdi chen, shishuang cui, huawei ling, juanjuan du, yiqi lin, jing pan, haiyan zhou, gang wang, ying wang, qin xiao, jun liu, yuyan tan,

<title>Abstract</title> <p> Backgrounds: Progressive Supranuclear Palsy (PSP) is a rare movement disorder with poor prognosis. This retrospective study aims to characterize the natural history of PSP and to find predictors of shorter survival and faster decline of activity of daily living. Method: All patients recruited fulfilled the movement ... Read more >>

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Dysarthria enhancement mechanism under external clear speech instruction in Parkinson's disease, progressive supranuclear palsy and multiple system atrophy.

Dominik Skrabal, Tereza Tykalova, Jiri Klempir, Evzen Ruzicka, Jan Rusz,

Clear speech refers to intentionally modifying conversational speech to maximise intelligibility. This study aimed to compare the speech behaviour of patients with progressive supranuclear palsy (PSP), multiple system atrophy (MSA) and Parkinson's disease (PD) under conversational and clear speech conditions to gain greater pathophysiological insight. A total of 68 participants ... Read more >>

J Neural Transm (Vienna) (Journal of neural transmission (Vienna, Austria : 1996))
[2020, :]

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Automated classification of neurodegenerative parkinsonian syndromes using multimodal magnetic resonance imaging in a clinical setting

Lydia Chougar, Johann Faouzi, Nadya Pyatigorskaya, Rahul Gaurav, Emma Biondetti, Marie Villotte, Romain Valabregue, Jean-Christophe Corvol, Alexis Brice, Louise-Laure Mariani, Florence Cormier, Marie Vidailhet, Gwendoline Dupont, Ines Piot, David Grabli, Christine Payan, Olivier Colliot, Bertrand Degos, Stephane Lehericy,

Background: Several studies have shown that machine learning algorithms using MRI data can accurately discriminate parkinsonian syndromes. Validation under clinical conditions is missing. Objectives: To evaluate the accuracy for the categorization of parkinsonian syndromes of a machine learning algorithm trained with a research cohort and tested on an independent clinical ... Read more >>

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Mitochondrial tRNA methylation in Alzheimer’s disease and progressive supranuclear palsy

Nicole Phillips, Talisa Silzer, Gita Pathak,

<title>Abstract</title> <p> Background Methylation of mitochondrial tRNAs (mt-tRNA) at the 9 th position (“p9 site”) is known to impact translational efficiency and downstream mitochondrial function; however, direct assessment of mt-RNA methylation is challenging. Recent RNA sequence-based methods have been developed to reliably identify post-transcriptional methylation. Though p9 ... Read more >>

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Association of Tripartite Motif Containing 11 rs564309 with Tau Pathology in Progressive Supranuclear Palsy.

Rebecca R Valentino, Shunsuke Koga, Michael G Heckman, Danielle E Brushaber, Nancy N Diehl, Ronald L Walton, Dennis W Dickson, Owen A Ross,

BACKGROUND:Intronic variant rs564309 in tripartite motif containing 11 (TRIM11) is associated with clinical phenotypic differences in progressive supranuclear palsy (PSP), whereby the minor allele (A) is more common in atypical PSP than typical PSP (PSP-Richardson's syndrome). However, rs564309 has not been investigated relative to neuropathological outcomes. OBJECTIVE:Evaluate the association of ... Read more >>

Mov. Disord. (Movement disorders : official journal of the Movement Disorder Society)
[2020, :]

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The Added Value of Tau-PET in the Assessment of Progressive Supranuclear Palsy.

Diego Alfonso López-Mora, Alejandro Fernández León, Alberto Lleó, Rafael Blesa, Valle Camacho,

Progressive supranuclear palsy (PSP) is rare neurodegenerative disorder characterized by deposition of fibrillar aggregates of 4R tau-protein in neurons and glial cells. This disorder is underdiagnosed due to the overlap of the clinical syndrome with other related conditions. The clinical manifestations include cognitive impairment associated with behavioral changes, akinetic rigid ... Read more >>

Clin Nucl Med (Clinical nuclear medicine)
[2020, 45(5):e239-e240]

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Progressive supranuclear palsy presenting with hyperkinetic movement disorder and hemiplegic dystonia: a case report.

Wei Zhang, Wei Mao, Erhe Xu, Jagadish K Chhetri, Piu Chan,

Background: Progressive supranuclear palsy (PSP) is a progressive neurodegenerative brain disease which has been rarely described in association with hyperkinetic symptoms. Here, we report a case of PSP that was presented with hyperkinetic movement disorder, hemiplegic dystonia, and other clinical features that overlap with behavioral variant frontotemporal dementia (bvFTD) and ... Read more >>

Int. J. Neurosci. (The International journal of neuroscience)
[2020, :1-4]

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Progressive Supranuclear Palsy-Parkinsonism Predominant (PSP-P)-A Clinical Challenge at the Boundaries of PSP and Parkinson's Disease (PD).

Piotr Alster, Natalia Madetko, Dariusz Koziorowski, Andrzej Friedman,

Progressive Supranuclear Palsy (PSP) and Parkinson's Disease (PD), especially in their early stages, show overlapping clinical manifestations. The criteria for the diagnosis of PSP, released in 2017, indicate four basic features of the disease-postural instability (P), akinesia (A), oculomotor dysfunction (O) and cognitive and lingual disorders (C), which clarify the ... Read more >>

Front Neurol (Frontiers in Neurology)
[2020, 11:180]

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Insight in frontotemporal dementia and progressive supranuclear palsy.

Andrea Plutino, Emanuele Camerucci, Valentina Ranaldi, Sara Baldinelli, Chiara Fiori, Mauro Silvestrini, Simona Luzzi,

INTRODUCTION:Progressive supranuclear palsy (PSP) and behavioural variant frontotemporal dementia - (bv-FTD) share common neuropsychological features except for online monitoring awareness. Therefore, the aim of our study is to explore if this assessment could be used in standard clinical practice. MATERIALS AND METHODS:We retrospectively analyse 93 subjects (27 FTD, 25 PSP, ... Read more >>

Neurol. Sci. (Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology)
[2020, :]

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Progressive supranuclear palsy and primary lateral sclerosis secondary to globular glial tauopathy: a case report and a practical theoretical framework for the clinical prediction of this rare pathological entity.

Andy J Liu, Jessica E Chang, Georges Naasan, Adam L Boxer, Bruce L Miller, Salvatore Spina,

Globular glial tauopathy (GGT) is a rare 4-repeat tauopathy characterized by the accumulation of tau globular inclusions in astrocytes and oligodendrocytes. Several clinical phenotypes have been associated with GGT, making the prediction of this rare pathological entity difficult. We report the case of a patient with eye-movement abnormalities and gait ... Read more >>

Neurocase (Neurocase)
[2020, :1-7]

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Automated MRI Classification in Progressive Supranuclear Palsy: a Large International Cohort Study.

Salvatore Nigro, Angelo Antonini, David E Vaillancourt, Klaus Seppi, Roberto Ceravolo, Antonio P Strafella, Antonio Augimeri, Andrea Quattrone, Maurizio Morelli, Luca Weis, Eleonora Fiorenzato, Roberta Biundo, Roxana G Burciu, Florian Krismer, Nikolaus R McFarland, Christoph Mueller, Elke R Gizewski, Mirco Cosottini, Eleonora Del Prete, Sonia Mazzucchi, Aldo Quattrone,

BACKGROUND:The Magnetic Resonance Parkinsonism Index is listed as one of the most reliable imaging morphometric markers for diagnosis of progressive supranuclear palsy (PSP). However, the use of this index in diagnostic workup has been limited until now by the low generalizability of published results because of small monocentric patient cohorts, ... Read more >>

Mov. Disord. (Movement disorders : official journal of the Movement Disorder Society)
[2020, :]

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Copathology in Progressive Supranuclear Palsy: Does It Matter?

Milica Jecmenica Lukic, Carolin Kurz, Gesine Respondek, Oriol Grau-Rivera, Yaroslau Compta, Ellen Gelpi, Claire Troakes, , John C van Swieten, Armin Giese, Sigrun Roeber, Thomas Arzberger, Günter Höglinger,

BACKGROUND:The influence of concomitant brain pathologies on the progression rate in PSP is unclear. OBJECTIVES:To analyze the frequency and severity of copathologies and their impact on the progression in PSP. METHODS:We analyzed clinic-pathological features of 101 PSP patients. Diagnoses and stages of copathologies were established according to standardized criteria, including ... Read more >>

Mov. Disord. (Movement disorders : official journal of the Movement Disorder Society)
[2020, :]

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Cerebrospinal fluid neurogranin as a new player in prion disease diagnosis and prognosis.

Anna Villar-Pique, Inga Zerr, Franc Llorens,

Neural Regen Res (Neural regeneration research)
[2020, 15(5):861-862]

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Diagnostic potential of automated tractography in progressive supranuclear palsy variants.

Thomas Potrusil, Florian Krismer, Vincent Beliveau, Klaus Seppi, Christoph Müller, Felix Troger, Georg Göbel, Ruth Steiger, Elke R Gizewski, Werner Poewe, Christoph Scherfler,

BACKGROUND:Microstructural white matter integrity captured by diffusion-tensor imaging (DTI) is significantly more affected in progressive supranuclear palsy-Richardson's syndrome (PSP-RS) compared to PSP-parkinsonism (PSP-P). OBJECTIVES:To characterize the microstructural integrity of large fascicular bundles using standardized probabilistic tractography and combine it with previously established DTI- and volumetric measures of subcortical brain structures ... Read more >>

Parkinsonism Relat. Disord. (Parkinsonism & related disorders)
[2020, 72:65-71]

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Vitamin B12 measurements across neurodegenerative disorders.

Nijee S. Luthra, Ariane H. Marcus, Nancy K. Hills, Chadwick W. Christine,

BackgroundVitamin B12 deficiency causes a number of neurological features including cognitive and psychiatric disturbances, gait instability, neuropathy, and autonomic dysfunction. Clinical recognition of B12 deficiency in neurodegenerative disorders is more challenging because it causes defects that overlap with expected disease progression. We sought to determine whether B12 levels at the ... Read more >>

J Clin Mov Disord (Journal of Clinical Movement Disorders)
[2020, 7:]

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Primary Tau Pathology, Not Copathology, Correlates With Clinical Symptoms in PSP and CBD.

John L Robinson, Ning Yan, Carrie Caswell, Sharon X Xie, EunRan Suh, Vivianna M Van Deerlin, Garrett Gibbons, David J Irwin, Murray Grossman, Edward B Lee, Virginia M-Y Lee, Bruce Miller, John Q Trojanowski,

Distinct neuronal and glial tau pathologies define corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP). Additional Alzheimer disease, TDP-43, and Lewy body copathologies are also common. The interplay of these pathologies with clinical symptoms remains unclear as individuals can present with corticobasal syndrome, frontotemporal dementia, PSP, or atypical Parkinsonism and ... Read more >>

J. Neuropathol. Exp. Neurol. (Journal of neuropathology and experimental neurology)
[2020, 79(3):296-304]

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Central auditory processing in parkinsonian disorders: A systematic review.

Evelien De Groote, Kim De Keyser, Annelies Bockstael, Dick Botteldooren, Patrick Santens, Miet De Letter,

Altered auditory processing has been increasingly recognized as a non-motor feature in parkinsonian disorders. This systematic review provides an overview of behavioral and electrophysiological literature on central auditory processing in patients with Parkinson's disease (PD), multiple system atrophy (MSA) and progressive supranuclear palsy (PSP). A systematic database search was conducted ... Read more >>

Neurosci Biobehav Rev (Neuroscience and biobehavioral reviews)
[2020, 113:111-132]

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High-contrast in-vivo imaging of tau pathologies in Alzheimer's and non-Alzheimer's disease tauopathies

Kenji Tagai, Maiko Ono, Manabu Kubota, Soichiro Kitamura, Keisuke Takahata, Chie Seki, Yuhei Takado, Hitoshi Shinotoh, Yasunori Sano, Kiwamu Matsuoka, Hiroyuki Takuwa, Masafumi Shimojo, Manami Takahashi, Kazunori Kawamura, Tatsuya Kikuchi, Maki Okada, Haruhiko Akiyama, Hisaomi Suzuki, Mitsumoto Onaya, Takahiro Takeda, Kimihito Arai, Nobutaka Arai, Nobuyuki Araki, Yuko Saito, Yasuyuki Kimura, Masanori Ichise, Yutaka Tomita, Ming-Rong Zhang, Tetsuya Suhara, Masahiro Shigeta, Naruhiko Sahara, Makoto Higuchi, Hitoshi Shimada,

Depositions of fibrillary tau protein aggregates are implicated in diverse tauopathies, including Alzheimer's disease (AD) and frontotemporal lobar degeneration (FTLD). A panel of radiochemicals has enabled in-vivo positron emission tomography (PET) of AD tau pathologies composed of all six tau isoforms, while sensitive detection of FTLD tau inclusions, mostly consisting ... Read more >>

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