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Progressive supranuclear palsy

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https://www.readbyqxmd.com/read/28087578/distinct-binding-of-pet-ligands-pbb3-and-av-1451-to-tau-fibril-strains-in-neurodegenerative-tauopathies
#1
Maiko Ono, Naruhiko Sahara, Katsushi Kumata, Bin Ji, Ruiqing Ni, Shunsuke Koga, Dennis W Dickson, John Q Trojanowski, Virginia M-Y Lee, Mari Yoshida, Isao Hozumi, Yasumasa Yoshiyama, John C van Swieten, Agneta Nordberg, Tetsuya Suhara, Ming-Rong Zhang, Makoto Higuchi
Diverse neurodegenerative disorders are characterized by deposition of tau fibrils composed of conformers (i.e. strains) unique to each illness. The development of tau imaging agents has enabled visualization of tau lesions in tauopathy patients, but the modes of their binding to different tau strains remain elusive. Here we compared binding of tau positron emission tomography ligands, PBB3 and AV-1451, by fluorescence, autoradiography and homogenate binding assays with homologous and heterologous blockades using tauopathy brain samples...
January 12, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28078564/mri-measurements-of-brainstem-structures-in-patients-with-vascular-parkinsonism-progressive-supranuclear-palsy-and-parkinson-s-disease
#2
Byeong C Kim, Seong-Min Choi, Kang-Ho Choi, Tai-Seung Nam, Joon-Tae Kim, Seung-Han Lee, Man-Seok Park, Woong Yoon
Magnetic resonance (MR) measurements of brainstem structures have been reported to be useful in differentiating patients with progressive supranuclear palsy (PSP) from those with Parkinson's disease (PD). The aim of this study was to determine whether quantitative measurements of brainstem structures on MR images can help differentiate vascular parkinsonism (VaP) from degenerative parkinsonism (PD and PSP). Areas of the midbrain and pons, and widths of the superior cerebellar peduncle (SCP) and middle cerebellar peduncle (MCP) were measured in 62 patients with PD, 25 patients with PSP (11 probable and 14 possible), and 24 patients with VaP on T 1-weighted MR images...
January 11, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28064358/incipient-progressive-supranuclear-palsy-is-more-common-than-expected-and-may-comprise-clinicopathological-subtypes-a-forensic-autopsy-series
#3
Koji Yoshida, Yukiko Hata, Koshi Kinoshita, Shutaro Takashima, Kortaro Tanaka, Naoki Nishida
We investigated 998 serial Japanese forensic autopsy cases (0-101 years old, mean age 61.7 ± 21.9), with no case selection, using immunohistochemistry to detect cases with progressive supranuclear palsy (PSP). Twenty-nine cases (mean age 82.3 ± 7.2 years, 11 males, 18 females) fulfilled the National Institute of Neuronal Disorders and Stroke (NINDS)-PSP pathological criteria (2.9% of all cases, 4.6% of cases over 60). All had neuronal and glial inclusions in the basal ganglia and brainstem. However, 13 cases had low tau pathology and were categorized as atypical PSP...
January 7, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28064175/neuroimaging-of-inflammation-in-memory-and-related-other-disorders-nimrod-study-protocol-a-deep-phenotyping-cohort-study-of-the-role-of-brain-inflammation-in-dementia-depression-and-other-neurological-illnesses
#4
W Richard Bevan-Jones, Ajenthan Surendranathan, Luca Passamonti, Patricia Vázquez Rodríguez, Robert Arnold, Elijah Mak, Li Su, Jonathan P Coles, Tim D Fryer, Young T Hong, Guy Williams, Franklin Aigbirhio, James B Rowe, John T O'Brien
INTRODUCTION: Inflammation of the central nervous system is increasingly regarded as having a role in cognitive disorders such as dementia and depression, but it is not clear how such inflammation relates to other aspects of neuropathology, structural and functional changes in the brain and symptoms (as assessed via clinical and neuropsychological assessment and MRI). This study will explore these pathophysiological mechanisms using positron emission tomography (PET) which allows in vivo imaging of inflammation, amyloid and τ deposition, together with neuropsychological profiling, MRI and peripheral biomarker analysis...
January 7, 2017: BMJ Open
https://www.readbyqxmd.com/read/28057431/advanced-structural-neuroimaging-in-progressive-supranuclear-palsy-where-do-we-stand
#5
REVIEW
Albert Stezin, Abhishek Lenka, Ketan Jhunjhunwala, Jitender Saini, Pramod K Pal
Progressive supranuclear palsy (PSP) is a progressive neurological disorder characterized by presence of supranuclear gaze palsy, early postural instability, parkinsonism and cognitive impairment. Advanced structural neuroimaging studies have demonstrated variable areas of grey and white matter involvement in PSP. Grey matter (GM) involvement is predominantly reported in the midbrain, subcortical structures such as thalamus and basal ganglia, and cerebellum. Most white matter (WM) tracts are also reported to be damaged in PSP...
December 26, 2016: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/28034996/a-fully-automated-atlas-based-approach-for-superior-cerebellar-peduncle-evaluation-in-progressive-supranuclear-palsy-phenotypes
#6
G Nicoletti, M E Caligiuri, A Cherubini, M Morelli, F Novellino, G Arabia, M Salsone, A Quattrone
BACKGROUND AND PURPOSE: The superior cerebellar peduncle is damaged in progressive supranuclear palsy. However, alterations differ between progressive supranuclear palsy with Richardson syndrome and progressive supranuclear palsy-parkinsonism. In this study, we propose an automated tool for superior cerebellar peduncle integrity assessment and test its performance in patients with progressive supranuclear palsy with Richardson syndrome, progressive supranuclear palsy-parkinsonism, Parkinson disease, and healthy controls...
December 29, 2016: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28031995/prevalence-of-progressive-supranuclear-palsy-in-yonago-change-throughout-a-decade
#7
Hiroshi Takigawa, Michio Kitayama, Kenji Wada-Isoe, Hisanori Kowa, Kenji Nakashima
BACKGROUND: Progressive supranuclear palsy (PSP) is a neurodegenerative disorder that is sometimes confused with Parkinson's disease, multiple system atrophy, and other disorders. The typical clinical features are categorized as Richardson's syndrome (RS), but other clinical subtypes include PSP-parkinsonism (PSP-P) and PSP-pure akinesia with gait freezing (PSP-PAGF). In this study, we determined the prevalence of PSP in a Japanese rural area compared to our previous 1999 report. METHODS: We collected data in Yonago City from 2009 to 2014 using a service-based study of PSP...
December 2016: Brain and Behavior
https://www.readbyqxmd.com/read/28025406/teaching-video-neuroimages-purposeless-groaning-in-progressive-supranuclear-palsy
#8
Soon Chai Low, Ai Huey Tan, Shen-Yang Lim
No abstract text is available yet for this article.
January 3, 2017: Neurology
https://www.readbyqxmd.com/read/28019685/tauopathy-with-hippocampal-4-repeat-tau-immunoreactive-spherical-inclusions-a-report-of-three-cases
#9
Gabor G Kovacs, Linda K Kwong, Murray Grossman, David J Irwin, Edward B Lee, John L Robinson, Eunran Suh, Vivianna M Van Deerlin, Virginia M Lee, John Q Trojanowski
Tauopathies are a major group of neurodegenerative proteinopathies characterized by the accumulation of abnormal and hyperphosphorylated tau proteins in the brain. Tau pathology is characterized as 3R (repeat) or 4R predominant or mixed 3R and 4R type. Here we report three cases lacking mutations in the microtubule associated protein tau (MAPT) gene with unusual tau pathology. The age at onset and duration of illness, respectively, were 63 and 20 years (male), 67 and 5 years (female), and 72 and 20 years (female)...
December 26, 2016: Brain Pathology
https://www.readbyqxmd.com/read/28009087/distribution-and-characteristics-of-transactive-response-dna-binding-protein-43-kda-pathology-in-progressive-supranuclear-palsy
#10
Shunsuke Koga, Monica Sanchez-Contreras, Keith A Josephs, Ryan J Uitti, Neill Graff-Radford, Jay A van Gerpen, William P Cheshire, Zbigniew K Wszolek, Rosa Rademakers, Dennis W Dickson
BACKGROUND: This study aimed to determine the frequency of transactive response DNA binding protein 43 kDa pathology in PSP, the clinical features of patients with this pathology, and genetic risk factors for it. METHODS: Hippocampal sections were screened with immunohistochemistry for transactive response DNA binding protein 43 kDa in 945 PSP cases. A subset of 261 cases that were negative in hippocampus was screened in the amygdala. The density and disruption of this pathology, as well as regional tau burden, and clinical and genetic characteristics were analyzed...
December 23, 2016: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/28007518/understanding-falls-in-progressive-supranuclear-palsy
#11
Brent Bluett, Irene Litvan, Shumin Cheng, Jorge Juncos, David E Riley, David G Standaert, Stephen G Reich, Deborah A Hall, Benzi Kluger, David Shprecher, Connie Marras, Joseph Jankovic
INTRODUCTION: Progressive supranuclear palsy (PSP) is characterized by frequent falls which worsen with disease progression, causing substantial morbidity and mortality. Few studies have investigated which factors contribute to falls in PSP, and all have involved few participants, thus lacking necessary statistical power. The aim of this study was to identify clinical parameters most significantly associated with increasing falls in PSP, using the largest sample of patients to date. METHODS: Comprehensive clinical data were collected from 339 not demented PSP patients meeting the NINDS-SPSP criteria, who were divided into two groups - Infrequent Fallers (IF; n = 118) with rare falls, and Frequent Fallers (FF; n = 221) who fell occasionally to multiple times a day...
December 15, 2016: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/27999880/-pathomechanisms-and-clinical-aspects-of-frontotemporal-lobar-degeneration
#12
REVIEW
K Bürger, T Arzberger, J Stephan, J Levin, D Edbauer
BACKGROUND: Frontotemporal lobar degeneration (FTLD) includes a spectrum of heterogeneous clinical and neuropathological diseases. In a strict sense this includes the behavioral variant of frontotemporal dementia (bvFTD) and primary progressive aphasia (PPA) and both variants can be associated with amyotrophic lateral sclerosis (FTD-ALS). In a broader sense FTLD also includes progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). In recent years the strong genetic component of FTLD has become increasingly clear...
December 20, 2016: Der Nervenarzt
https://www.readbyqxmd.com/read/27997036/pathologic-correlations-of-f-18-av-1451-imaging-in-non-alzheimer-tauopathies
#13
Marta Marquié, Marc D Normandin, Avery C Meltzer, Michael Siao Tick Chong, Nicolas V Andrea, Alejandro Antón-Fernández, William E Klunk, Chester A Mathis, Milos D Ikonomovic, Manik Debnath, Elizabeth A Bien, Charles R Vanderburg, Isabel Costantino, Sara Makaretz, Sarah L DeVos, Derek H Oakley, Stephen N Gomperts, John H Growdon, Kimiko Domoto-Reilly, Diane Lucente, Bradford C Dickerson, Matthew P Frosch, Bradley T Hyman, Keith A Johnson, Teresa Gómez-Isla
OBJECTIVE: Recent studies have shown that PET tracer AV-1451 exhibits high binding affinity for paired helical filament (PHF)-tau pathology in Alzheimer's brains. However, the ability of this ligand to bind to tau lesions in other tauopathies remains controversial. Our goal was to examine the correlation of in vivo and postmortem AV-1451 binding patterns in three autopsy-confirmed non-Alzheimer tauopathy cases. METHODS: We quantified in vivo retention of [F-18]-AV-1451 and performed autoradiography, [H-3]-AV-1451 binding assays and quantitative tau measurements in postmortem brain samples from two Progressive Supranuclear Palsy (PSP) cases and a MAPT P301L mutation carrier...
December 20, 2016: Annals of Neurology
https://www.readbyqxmd.com/read/27987553/molecular-imaging-of-extrapyramidal-movement-disorders
#14
REVIEW
Kirk A Frey
Extrapyramidal movement disorders including Parkinson disease, multiple systems atrophy, progressive supranuclear palsy, and corticobasal degeneration are neurodegenerative syndromes with distinct neuropathological changes, indicating differing underlying etiologies. Clinical features that may distinguish among these conditions are often absent, particularly early after the onset of symptoms. Therapy is presently limited, and there are no established disease-modifying or neuroprotective interventions. Advances in therapeutics will depend on the early and accurate diagnostic classification of patients...
January 2017: Seminars in Nuclear Medicine
https://www.readbyqxmd.com/read/27981030/9-4%C3%A2-t-mr-microscopy-of-the-substantia-nigra-with-pathological-validation-in-controls-and-disease
#15
L A Massey, M A Miranda, O Al-Helli, H G Parkes, J S Thornton, P-W So, M J White, L Mancini, C Strand, J Holton, A J Lees, T Revesz, T A Yousry
BACKGROUND: The anatomy of the substantia nigra on conventional MRI is controversial. Even using histological techniques it is difficult to delineate with certainty from surrounding structures. We sought to define the anatomy of the SN using high field spin-echo MRI of pathological material in which we could study the anatomy in detail to corroborate our MRI findings in controls and Parkinson's disease and progressive supranuclear palsy. METHODS: 23 brains were selected from the Queen Square Brain Bank (10 controls, 8 progressive supranuclear palsy, 5 Parkinson's disease) and imaged using high field 9...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/27928380/facial-dystonia-with-facial-grimacing-and-vertical-gaze-palsy-with-round-the-houses-sign-in-a-29-year-old-woman
#16
J Crespi, G Bråthen, P Quist-Paulsen, J Pagonabarraga, C Roig-Arnall
A 29-year-old woman developed progressive dysarthria and coordination problems from the age of 15. Examination showed dysarthria, facial dystonia, bibrachial dystonia, hyperreflexia, ataxia, and emotional incontinence. Downward supranuclear gaze palsy was prominent with a "Round the Houses" sign. Magnetic resonance imaging of the brain and medulla, electroneurography, and cerebrospinal fluid were normal. A computed tomography scan showed hepatosplenomegaly. This combination of progressive neurological symptoms together with hepatosplenomegaly was suggestive of inborn error of metabolism...
February 2016: Neuro-ophthalmology
https://www.readbyqxmd.com/read/27927959/vectored-intracerebral-immunization-with-the-anti-tau-monoclonal-antibody-phf1-markedly-reduces-tau-pathology-in-mutant-tau-transgenic-mice
#17
Wencheng Liu, Lingzhi Zhao, Brittany Blackman, Mayur Parmar, Man Ying Wong, Thomas Woo, Fangmin Yu, Dolan Sondhi, Stephen M Kaminsky, Ronald G Crystal, Steven M Paul
: Passive immunization with anti-tau monoclonal antibodies has been shown by several laboratories to reduce age-dependent tau pathology and neurodegeneration in mutant tau transgenic mice. These studies have used repeated high weekly doses of various tau antibodies administered systemically for several months and have reported reduced tau pathology of ∼40-50% in various brain regions. Here we show that direct intrahippocampal administration of the adeno-associated virus (AAV)-vectored anti-phospho-tau antibody PHF1 to P301S tau transgenic mice results in high and durable antibody expression, primarily in neurons...
December 7, 2016: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/27922865/additional-value-of-early-phase-18f-fp-cit-pet-image-for-differential-diagnosis-of-atypical-parkinsonism
#18
Soyoung Jin, Minyoung Oh, Seung Jun Oh, Jungsu S Oh, Sang Ju Lee, Sun Ju Chung, Jae Seung Kim
PURPOSE: Regional cerebral perfusion is coupled to metabolism in general. Early perfusion dominant imaging using F-FP-CIT PET (pCIT) may provide complementary information to delayed dopamine transporter dominant images. We investigated the ability of pCIT to differentiate atypical Parkinson disorder from Parkinson disease (PD) compared to FDG and the image quality for optimizing the acquisition time. METHODS: Sixty-seven subjects [PD, 23 subjects; multiple system atrophy-cerebellar type (MSA-C), 27 subjects; MSA-Parkinson type (MSA-P), 12 subjects; and progressive supranuclear palsy (PSP), 5 subjects] underwent F-FP-CIT and FDG PET...
December 3, 2016: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/27915581/ftld-tdp-and-progressive-supranuclear-palsy-in-comorbidity-a-report-of-two-cases-with-different-clinical-presentations
#19
Kateřina Storey, Silvie Johanidesová, Radoslav Matěj, Jiří Keller, Zdeněk Rohan, Robert Rusina
Frontotemporal lobar degeneration with transactive response DNA-binding protein 43 (FTLD-TDP) and progressive supranuclear palsy (PSP) are distinct neurodegenerations with different clinical presentations. We report two cases with FTLD-TDP and PSP in comorbidity: a patient with amnestic dementia developing frontal lobe dementia, Parkinsonism and supranuclear gaze palsy and a patient with cerebellar ataxia and nystagmus developing akinesia, rigidity, and subcortical dementia. Neuropathological examination revealed neuronal and glial tau pathology together with ubiquitin, and phospho-TDP-43-immunoreactivities in the hippocampus, striatum, mesencephalon, and frontal and temporal cortices...
December 3, 2016: Neurocase
https://www.readbyqxmd.com/read/27911827/tau-prions-from-alzheimer-s-disease-and-chronic-traumatic-encephalopathy-patients-propagate-in-cultured-cells
#20
Amanda L Woerman, Atsushi Aoyagi, Smita Patel, Sabeen A Kazmi, Iryna Lobach, Lea T Grinberg, Ann C McKee, William W Seeley, Steven H Olson, Stanley B Prusiner
Tau prions are thought to aggregate in the central nervous system, resulting in neurodegeneration. Among the tauopathies, Alzheimer's disease (AD) is the most common, whereas argyrophilic grain disease (AGD), corticobasal degeneration (CBD), chronic traumatic encephalopathy (CTE), Pick's disease (PiD), and progressive supranuclear palsy (PSP) are less prevalent. Brain extracts from deceased individuals with PiD, a neurodegenerative disorder characterized by three-repeat (3R) tau prions, were used to infect HEK293T cells expressing 3R tau fused to yellow fluorescent protein (YFP)...
December 13, 2016: Proceedings of the National Academy of Sciences of the United States of America
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