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Progressive supranuclear palsy

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https://www.readbyqxmd.com/read/29903629/magnetic-resonance-imaging-in-dementia
#1
L Raposo Rodríguez, D J Tovar Salazar, N Fernández García, L Pastor Hernández, Ó Fernández Guinea
OBJECTIVE: To describe and illustrate the key findings on structural magnetic resonance imaging (MRI) in the most common dementias of neurodegenerative origin: Alzheimer's disease, vascular dementia, dementia with Lewy bodies, variants of frontotemporal dementia, progressive supranuclear palsy, variants of multiple system atrophy, Parkinson dementia, and corticobasal degeneration. CONCLUSION: Today the role of MRI is no longer limited to ruling out underlying causes of cognitive deterioration...
June 11, 2018: Radiología
https://www.readbyqxmd.com/read/29902389/the-diagnosis-of-progressive-supranuclear-palsy-current-opinions-and-challenges
#2
Farwa Ali, Keith Josephs
Progressive supranuclear palsy (PSP) is associated with microtubule associated protein tau dysfunction. Originally thought to result in a syndrome of atypical parkinsonism, vertical supranuclear gaze palsy and cognitive impairment, several additional phenotypic manifestations of PSP pathology have been described over the last 20 years. Furthermore, prototypical PSP features may develop late, making early diagnosis challenging. Areas covered: An in-depth view of emerging knowledge in the field of PSP. Advances in clinicopathologic correlation, blood, cerebrospinal, and more importantly neuroimaging biomarkers are discussed in light of the 2017 PSP diagnostic criteria by the Movement Disorders Society Study Group...
June 14, 2018: Expert Review of Neurotherapeutics
https://www.readbyqxmd.com/read/29898972/diffuse-lewy-body-disease-manifesting-as-corticobasal-syndrome-a-rare-form-of-lewy-body-disease
#3
Koji Kasanuki, Keith A Josephs, Tanis J Ferman, Melissa E Murray, Shunsuke Koga, Takuya Konno, Nobutaka Sakae, Adam Parks, Ryan J Uitti, Jay A Van Gerpen, Neill R Graff-Radford, Zbigniew K Wszolek, Dennis W Dickson
OBJECTIVE: To describe clinical and pathologic characteristics of diffuse Lewy body disease (DLBD) manifesting as corticobasal syndrome (CBS). METHODS: In 523 autopsy-confirmed cases of DLBD, we identified 11 patients diagnosed with CBS. For comparison, we studied 22 DLBD brains with antemortem presentation of dementia with Lewy bodies (DLB). Given previous studies suggesting the importance of pathology in peri-Rolandic cortices in CBS, we used digital pathology to count Lewy bodies and to quantify intracytoplasmic and neuritic α-synuclein and phospho-tau burden in the motor cortex...
June 13, 2018: Neurology
https://www.readbyqxmd.com/read/29896099/evaluation-of-rna-blood-biomarkers-in-the-parkinson-s-disease-biomarkers-program
#4
Jose A Santiago, Virginie Bottero, Judith A Potashkin
There is a high misdiagnosis rate between Parkinson's disease (PD) and atypical parkinsonian disorders (APD), such as progressive supranuclear palsy (PSP), the second most common parkinsonian syndrome. In our earlier studies, we identified and replicated RNA blood biomarkers in several independent cohorts, however, replication in a cohort that includes PSP patients has not yet been performed. To this end, we evaluated the diagnostic potential of nine previously identified RNA biomarkers using quantitative PCR assays in 138 blood samples at baseline from PD, PSP and healthy controls (HCs) nested in the PD Biomarkers Program...
2018: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/29891013/sequential-stages-and-distribution-patterns-of-aging-related-tau-astrogliopathy-artag-in-the-human-brain
#5
Gabor G Kovacs, Sharon X Xie, John L Robinson, Edward B Lee, Douglas H Smith, Theresa Schuck, Virginia M-Y Lee, John Q Trojanowski
Aging-related tau astrogliopathy (ARTAG) describes tau pathology in astrocytes in different locations and anatomical regions. In the present study we addressed the question of whether sequential distribution patterns can be recognized for ARTAG or astroglial tau pathologies in both primary FTLD-tauopathies and non-FTLD-tauopathy cases. By evaluating 687 postmortem brains with diverse disorders we identified ARTAG in 455. We evaluated frequencies and hierarchical clustering of anatomical involvement and used conditional probability and logistic regression to model the sequential distribution of ARTAG and astroglial tau pathologies across different brain regions...
June 11, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29886532/purification-and-fibrillation-of-recombinant-human-amyloid-%C3%AE-prion-protein-and-tau-under-native-conditions
#6
Alexander Sandberg, Sofie Nyström
Protein misfolding, aggregation, and amyloid formation is involved in a large number of diseases. Recombinantly expressed proteins to study the amyloid fibril formation process are important for mechanistic studies. We here report protocols for production, purification, and fibrillation of three different proteins commonly found in cerebral amyloid; Aβ and Tau found in Alzheimer's disease, Chronic traumatic brain injury, Corticobasal degeneration, and Progressive Supranuclear Palsy and human prion protein found in Creutzfeldt-Jakob's disease...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/29878075/neurodegenerative-disease-concomitant-proteinopathies-are-prevalent-age-related-and-apoe4-associated
#7
John L Robinson, Edward B Lee, Sharon X Xie, Lior Rennert, EunRan Suh, Colin Bredenberg, Carrie Caswell, Vivianna M Van Deerlin, Ning Yan, Ahmed Yousef, Howard I Hurtig, Andrew Siderowf, Murray Grossman, Corey T McMillan, Bruce Miller, John E Duda, David J Irwin, David Wolk, Lauren Elman, Leo McCluskey, Alice Chen-Plotkin, Daniel Weintraub, Steven E Arnold, Johannes Brettschneider, Virginia M-Y Lee, John Q Trojanowski
Lewy bodies commonly occur in Alzheimer's disease, and Alzheimer's disease pathology is frequent in Lewy body diseases, but the burden of co-pathologies across neurodegenerative diseases is unknown. We assessed the extent of tau, amyloid-β, α-synuclein and TDP-43 proteinopathies in 766 autopsied individuals representing a broad spectrum of clinical neurodegenerative disease. We interrogated pathological Alzheimer's disease (n = 247); other tauopathies (n = 95) including Pick's disease, corticobasal disease and progressive supranuclear palsy; the synucleinopathies (n = 164) including multiple system atrophy and Lewy body disease; the TDP-43 proteinopathies (n = 188) including frontotemporal lobar degeneration with TDP-43 inclusions and amyotrophic lateral sclerosis; and a minimal pathology group (n = 72)...
June 5, 2018: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29867738/iglon5-associated-encephalitis-with-atypical-brain-magnetic-resonance-imaging-and-cerebrospinal-fluid-changes
#8
Massimiliano Montagna, Rizvana Amir, Ilse De Volder, Martin Lammens, Jef Huyskens, Barbara Willekens
IgLON5-associated encephalitis is a syndrome with different clinical presentations consisting of sleep dysfunction, bulbar dysfunction, chorea, and progressive supranuclear palsy-like symptoms whereas dysautonomy and cognitive decline usually appear in later stages of the disease. We report a case of a patient with IgLON5-associated encephalitis presenting with rapidly progressive cognitive decline and atypical inflammatory lesions on brain magnetic resonance imaging, oligoclonal bands on cerebrospinal fluid, anti-IgLON5 antibodies exclusively of the IgG1 class, and a fierce inflammatory reaction on brain biopsy, who responded favorably to immunotherapy...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29867729/structural-and-functional-brain-mapping-correlates-of-impaired-eye-movement-control-in-parkinsonian-syndromes-a-systems-based-concept
#9
REVIEW
Martin Gorges, Hans-Peter Müller, Jan Kassubek
The investigation of the human oculomotor system by eye movement recordings provides an approach to behavior and its alterations in disease. The neurodegenerative process underlying parkinsonian syndromes, including Parkinson's disease (PD), progressive supranuclear palsy (PSP), and multisystem atrophy (MSA) changes structural and functional brain organization, and thus affects eye movement control in a characteristic manner. Video-oculography has been established as a non-invasive recording device for eye movements, and systematic investigations of eye movement control in a clinical framework have emerged as a functional diagnostic tool in neurodegenerative parkinsonism...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29862010/sensitivity-and-specificity-of-the-ecas-in-parkinson-s-disease-and-progressive-supranuclear-palsy
#10
Jennifer A Foley, Elaine H Niven, Andrew Paget, Kailash P Bhatia, Simon F Farmer, Paul R Jarman, Patricia Limousin, Thomas T Warner, Huw R Morris, Thomas H Bak, Sharon Abrahams, Lisa Cipolotti
Disentangling Parkinson's disease (PD) and progressive supranuclear palsy (PSP) may be a diagnostic challenge. Cognitive signs may be useful, but existing screens are often insufficiently sensitive or unsuitable for assessing people with motor disorders. We investigated whether the newly developed ECAS, designed to be used with people with even severe motor disability, was sensitive to the cognitive impairment seen in PD and PSP and able to distinguish between these two disorders. Thirty patients with PD, 11 patients with PSP, and 40 healthy controls were assessed using the ECAS, as well as an extensive neuropsychological assessment...
2018: Parkinson's Disease
https://www.readbyqxmd.com/read/29860787/purposeless-groaning-in-parkinson-s-disease
#11
Shen-Yang Lim, Ai Huey Tan, Jia Lun Lim, Azlina Ahmad-Annuar
Purposeless groaning has been reported in advanced progressive supranuclear palsy. We present a case of purposeless groaning occurring as a primary complaint in a patient with advanced Parkinson's disease. Purposeless groaning is thought to be a manifestation of disinhibition and perseveration due to frontal-subcortical dysfunction. Proper recognition of this phenomenon will help clinicians to avoid unnecessary investigations and treatment (e.g., prescription of opioid medications).
May 2018: Journal of Movement Disorders
https://www.readbyqxmd.com/read/29860785/prospective-characterization-of-cognitive-function-in-typical-and-brainstem-predominant-progressive-supranuclear-palsy-phenotypes
#12
Young-Eun C Lee, David R Williams, Jacqueline F I Anderson
OBJECTIVE: Clinicopathological studies over the last decade have broadened the clinical spectrum of progressive supranuclear palsy (PSP) to include several distinct clinical syndromes. We examined the cognitive profiles of patients with PSP-Richardson's syndrome (PSP-RS) and two atypical 'brainstem predominant' PSP phenotypes (PSP-parkinsonism, PSP-P; and PSP-pure akinesia with gait freezing, PSP-PAGF) using a comprehensive neuropsychological battery. METHODS: Fourteen patients diagnosed as PSP-RS, three patients with PSP-P and four patients with PSP-PAGF were assessed using a comprehensive battery of neuropsychological tests...
May 2018: Journal of Movement Disorders
https://www.readbyqxmd.com/read/29860433/benfotiamine-treatment-activates-the-nrf2-are-pathway-and-is-neuroprotective-in-a-transgenic-mouse-model-of-tauopathy
#13
Victor Tapias, Shari Jainuddin, Manuj Ahuja, Cliona Stack, Ceyhan Elipenahli, Julie Vignisse, Meri Gerges, Natalia Starkova, Hui Xu, Anatoly A Starkov, Lucien Bettendorff, Dmitry M Hushpulian, Natalya A Smirnova, Irina G Gazaryan, Navneet A Kaidery, Sushama Wakade, Noel Y Calingasan, Bobby Thomas, Gary E Gibson, Magali Dumont, M Flint Beal
Impaired glucose metabolism, decreased levels of thiamine and its phosphate esters, and reduced activity of thiamine-dependent enzymes, such as pyruvate dehydrogenase, alpha-ketoglutarate dehydrogenase, and transketolase occur in Alzheimer's disease (AD). Thiamine deficiency exacerbates amyloid beta (Aβ) deposition, tau hyperphosphorylation, and oxidative stress. Benfotiamine (BFT) rescued cognitive deficits and reduced Aβ burden in APP/PS1 mice. In this study, we examined whether BFT confers neuroprotection against tau phosphorylation and the generation of neurofibrillary tangles (NFTs) in the P301S mouse model of tauopathy...
May 30, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29801913/progressive-spasticity-supranuclear-gaze-palsy-and-postural-instability-without-parkinsonism-what-s-in-a-phenotype
#14
L Ricciardi, M J Edwards, A Fasano, K P Bhatia, M Stamelou
We present a series of patients with vertical supranuclear gaze palsy, postural instability with falls, and progressive spasticity, who mimic Progressive Supranuclear Palsy - Richardson's syndrome (PSP-R) but have no parkinsonism, and in whom dopamine transporter imaging is normal. We suggest possible aetiologies for this constellation of symptoms, discuss the possible origin of these signs and highlight this phenotype as it may mimic atypical parkinsonism and in particular PSP.
July 15, 2018: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29801076/an-autopsy-case-of-progressive-supranuclear-palsy-with-incidental-atxn2-expansion
#15
Ahro Kim, Sung-Hye Park, Beomseok Jeon
No abstract text is available yet for this article.
May 7, 2018: JAMA Neurology
https://www.readbyqxmd.com/read/29779682/predicting-alpha-synuclein-pathology-by-rem-sleep-behavior-disorder-diagnosis
#16
David R Shprecher, Charles H Adler, Nan Zhang, Joseph G Hentz, Geidy E Serrano, Brittany N Dugger, Holly A Shill, Rodolfo Savica, John N Caviness, Marwan N Sabbagh, Christine M Belden, Erika Driver-Dunckley, Shyamal H Mehta, Lucia I Sue, Kathryn J Davis, Edward Zamrini, Thomas G Beach
Inability to accurately diagnose Lewy type alpha-synucleinopathy (LTS) pre-mortem has been a major obstacle to clinical care and research. Probable REM sleep behavior disorder (PRBD) diagnosed with support of instruments such as the Mayo Sleep Questionnaire (MSQ) may provide a cost effective means of predicting LTS. Since 2007, 602 subjects in the Arizona Study of Aging and Neurodegenerative Disorders had clinician assessment for PRBD (298 with, 304 without support of the MSQ), completed cognitive and movement examinations, and had neuropathological assessment...
May 17, 2018: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/29779045/clinical-utility-of-fdg-pet-in-parkinson-s-disease-and-atypical-parkinsonism-associated-with-dementia
#17
REVIEW
Zuzana Walker, Federica Gandolfo, Stefania Orini, Valentina Garibotto, Federica Agosta, Javier Arbizu, Femke Bouwman, Alexander Drzezga, Peter Nestor, Marina Boccardi, Daniele Altomare, Cristina Festari, Flavio Nobili
PURPOSE: There are no comprehensive guidelines for the use of FDG PET in the following three clinical scenarios: (1) diagnostic work-up of patients with idiopathic Parkinson's disease (PD) at risk of future cognitive decline, (2) discriminating idiopathic PD from progressive supranuclear palsy, and (3) identifying the underlying neuropathology in corticobasal syndrome. METHODS: We therefore performed three literature searches and evaluated the selected studies for quality of design, risk of bias, inconsistency, imprecision, indirectness and effect size...
May 19, 2018: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/29764386/parkinson-s-disease-diagnostic-observations-paddo-study-rationale-and-design-of-a-prospective-cohort-study-for-early-differentiation-of-parkinsonism
#18
Anouke van Rumund, Marjolein B Aerts, Rianne A J Esselink, Frederick J A Meijer, Marcel M Verbeek, Bastiaan R Bloem
BACKGROUND: Differentiation of Parkinson's disease (PD) from the various types of atypical parkinsonism (AP) such as multiple system atrophy (MSA), progressive supranuclear palsy (PSP), dementia with Lewy bodies (DLB), corticobasal syndrome (CBS) and vascular parkinsonism (VP), can be challenging, especially early in the disease course when symptoms overlap. A major unmet need in the diagnostic workup of these disorders is a diagnostic tool that differentiates the various disorders, preferably in the earliest disease stages when the clinical presentation is similar...
May 16, 2018: BMC Neurology
https://www.readbyqxmd.com/read/29760288/-neuropathologic-subtypes-of-frontotemporal-lobar-degeneration
#19
Mari Tada, Akiyoshi Kakita
Frontotemporal lobar degeneration (FTLD) is a heterogeneous disease entity encompassing a wide variety of histopathological features and genetic backgrounds. The last two decades have seen the discovery of causative genes and the identification of relevant proteins. The current histopathological classification is based on the major types of protein deposition in the brain, and most FTLD cases can be placed into one of three pathological subgroups: FTLD-tau, FTLD-TDP, and FTLD-FUS. Further sub-classification within each subgroup is based on the morphology of neuronal and glial inclusions and lesion distribution...
May 2018: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/29758948/argyrophilic-grain-pathology-in-frontotemporal-lobar-degeneration-demographic-clinical-neuropathological-and-genetic-features
#20
María José Gil, María Sagrario Manzano, María Luz Cuadrado, Cristina Fernández, Elena Góméz, Carmen Matesanz, Miguel Calero, Alberto Rábano
Frontotemporal lobar degeneration (FTLD) is a clinically, pathologically, and genetically heterogeneous group of disorders that affect the frontal and temporal lobes of the brain. FTLD classification distinguishes three main neuropathological groups: FTLD-tau, FTLD-TDP, and FTLD-FUS. As a four-repeat tauopathy, argyrophilic grain disease (AGD) is included in the FTLD-tau group. AGD may also appear in association with other neuropathological disorders. We describe the demographic, clinical, neuropathological, and genetic characteristics of a series of FTLD cases presenting with AGD...
2018: Journal of Alzheimer's Disease: JAD
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