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Progressive supranuclear palsy

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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
#1
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
#2
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
#3
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
#4
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
#5
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)...
November 28, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27894006/distinct-patterns-of-imprecise-consonant-articulation-among-parkinson-s-disease-progressive-supranuclear-palsy-and-multiple-system-atrophy
#6
Tereza Tykalova, Jan Rusz, Jiri Klempir, Roman Cmejla, Evzen Ruzicka
Distinct speech characteristics that may aid in differentiation between Parkinson's disease (PD), progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) remain tremendously under-explored. Here, the patterns and degree of consonant articulation deficits across voiced and voiceless stop plosives in 16 PD, 16 PSP, 16 MSA and 16 healthy control speakers were evaluated using acoustic and perceptual methods. Imprecise consonant articulation was observed across all Parkinsonian groups. Voice onset time of voiceless plosives was more prolonged in both PSP and MSA compared to PD, presumably due to greater severity of dysarthria and slower articulation rate...
November 25, 2016: Brain and Language
https://www.readbyqxmd.com/read/27890451/sniffing-the-diagnosis-olfactory-testing-in-neurodegenerative-parkinsonism
#7
F Krismer, B Pinter, C Mueller, P Mahlknecht, M Nocker, E Reiter, A Djamshidian-Tehrani, S M Boesch, G K Wenning, C Scherfler, W Poewe, K Seppi
OBJECTIVE: To determine the diagnostic utility of olfactory testing in patients with neurodegenerative parkinsonism. METHODS: The Sniffin' Sticks test battery for assessment of odor identification, discrimination, and threshold was applied to patients with Parkinson's disease (PD), multiple system atrophy (MSA) and progressive supranuclear palsy (PSP) as well as healthy controls (HC). Two different cohorts were analyzed: A PD/healthy control that included PD patients and HC as well as a PD/diseased control cohort for which patients PD, MSA and PSP were recruited...
November 21, 2016: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/27879155/managing-cognition-in-progressive-supranuclear-palsy
#8
Timothy Rittman, Ian Ts Coyle-Gilchrist, James B Rowe
Cognitive impairment is integral to the syndrome of progressive supranuclear palsy. It is most commonly described as a frontal dysexecutive syndrome but other impairments include apathy, impulsivity, visuospatial and memory functions. Cognitive dysfunction may be exacerbated by mood disturbance, medication and communication problems. In this review we advocate an individualized approach to managing cognitive impairment in progressive supranuclear palsy with the education of caregivers as a central component...
December 2016: Neurodegenerative Disease Management
https://www.readbyqxmd.com/read/27862260/tau-imaging-in-progressive-supranuclear-palsy
#9
EDITORIAL
Alexandra Perez-Soriano, A Jon Stoessl
No abstract text is available yet for this article.
November 8, 2016: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/27861377/familial-atypical-parkinsonism-with-rare-variant-in-vps35-and-fbxo7-genes-a-case-report
#10
Tereza Bartonikova, Katerina Mensikova, Lenka Mikulicova, Radek Vodicka, Radek Vrtel, Marek Godava, Miroslav Vastik, Michaela Kaiserova, Pavel Otruba, Iva Dolinova, Martin Nevrly, Petr Kanovsky
BACKGROUND: A higher prevalence of parkinsonism was recently identified in southeastern Moravia (Czech Republic). Further research confirmed 3 large pedigrees with familial autosomal-dominant parkinsonism spanning 5 generations. METHODS: This case report concerns a patient belonging to one of these 3 pedigrees, in whom motor and oculomotor symptoms were accompanied by frontal-type dementia, who finally developed a clinical phenotype of progressive supranuclear palsy...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27861346/unusual-phenotype-of-pathologically-confirmed-progressive-supranuclear-palsy-with-autonomic-dysfunction-and-cerebellar-ataxia-case-report
#11
Katerina Mensikova, Lucie Tuckova, Jiri Ehrmann, Petr Kanovsky
BACKGROUND: Based on the results of recent multicenter clinical-pathological studies, it seems that the clinical heterogeneity of progressive supranuclear palsy (PSP) is much broader than previously thought. We will report 2 cases of patients with unusual manifestation of pathologically confirmed PSP. METHODS: Two female patients were diagnosed with the parkinsonian phenotype of multiple system atrophy (MSAP) according to current clinical diagnostic criteria at the ages of 55 and 60 years, respectively...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27856628/analysis-of-extrastriatal-123i-fp-cit-binding-contributes-to-the-differential-diagnosis-of-parkinsonian-diseases
#12
Merijn Joling, Chris Vriend, Odile A Van den Heuvel, Pieter G H M Raijmakers, Paul A Jones, Henk W Berendse, Jan Booij
: (123)I-FP-CIT SPECT can visualise and quantify striatal dopamine transporter (DAT) binding in vivo. In addition, (123)I-FP-CIT has modest affinity for the serotonin transporter (SERT), predominantly represented in extrastriatal binding. Based on previous imaging studies that have suggested more pronounced degeneration of other monoaminergic systems in multiple system atrophy (MSA) and progressive supranuclear palsy (PSP) than in Parkinson's disease (PD), we hypothesised that, in addition to striatal DAT binding, there would be differences in extrastriatal (123)I-FP-CIT SPECT binding to SERT between MSA, PSP and PD...
November 17, 2016: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
https://www.readbyqxmd.com/read/27853238/assessment-of-brain-reference-genes-for-rt-qpcr-studies-in-neurodegenerative-diseases
#13
Rasmus Rydbirk, Jonas Folke, Kristian Winge, Susana Aznar, Bente Pakkenberg, Tomasz Brudek
Evaluation of gene expression levels by reverse transcription quantitative real-time PCR (RT-qPCR) has for many years been the favourite approach for discovering disease-associated alterations. Normalization of results to stably expressed reference genes (RGs) is pivotal to obtain reliable results. This is especially important in relation to neurodegenerative diseases where disease-related structural changes may affect the most commonly used RGs. We analysed 15 candidate RGs in 98 brain samples from two brain regions from Alzheimer's disease (AD), Parkinson's disease (PD), Multiple System Atrophy, and Progressive Supranuclear Palsy patients...
November 17, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27844039/increased-prevalence-of-autoimmune-disease-within-c9-and-ftd-mnd-cohorts-completing-the-picture
#14
Zachary A Miller, Virginia E Sturm, Gamze Balci Camsari, Anna Karydas, Jennifer S Yokoyama, Lea T Grinberg, Adam L Boxer, Howard J Rosen, Katherine P Rankin, Maria Luisa Gorno-Tempini, Giovanni Coppola, Daniel H Geschwind, Rosa Rademakers, William W Seeley, Neill R Graff-Radford, Bruce L Miller
OBJECTIVE: To determine the prevalence of autoimmune disease in symptomatic C9ORF72 (C9) mutation carriers and frontotemporal dementia with motor neuron disease (FTD/MND) cohorts. METHODS: In this case-control study, we reviewed the clinical histories of 66 patients with FTD/MND and 57 symptomatic C9 carriers (24 overlapping cases), a total of 99 charts, for history of autoimmune disease. The prevalence of autoimmune disease in C9 and FTD/MND cohorts was determined by χ(2) and Fisher exact comparisons between the combined C9 and FTD/MND group with normal control, Alzheimer disease, and progressive supranuclear palsy cohorts, as well as comparisons within C9 and FTD/MND cohorts...
December 2016: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/27842611/tau-pathology-in-aged-cynomolgus-monkeys-is-progressive-supranuclear-palsy-corticobasal-degeneration-but-not-alzheimer-disease-like-ultrastructural-mapping-of-tau-by-edx
#15
Toshiki Uchihara, Kentaro Endo, Hiromi Kondo, Sachi Okabayashi, Nobuhiro Shimozawa, Yasuhiro Yasutomi, Eijiro Adachi, Nobuyuki Kimura
Concomitant deposition of amyloid -beta protein (Aβ) and neuronal tau as neurofibrillary tangles in the human brain is a hallmark of Alzheimer disease (AD). Because these deposits increase during normal aging, it has been proposed that aging brains may also undergo AD-like changes. To investigate the neuropathological changes that occur in the aging primate brain, we examined 21 brains of cynomolgus monkeys (7-36 years old) for Aβ- and tau-positive lesions. We found, 1) extensive deposition of Aβ in brains of cynomolgus monkeys over 25 years of age, 2) selective deposition of 4-repeat tau as pretangles in neurons, and as coiled body-like structures in oligodendroglia-like cells and astrocytes, 3) preferential distribution of tau in the basal ganglia and neocortex rather than the hippocampus, and 4) age-associated increases in 30-34 kDa AT8- and RD4-positive tau fragments in sarkosyl-insoluble fractions...
November 14, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27842578/an-autopsy-confirmed-case-of-progressive-supranuclear-palsy-with-predominant-postural-instability
#16
Carolin Kurz, Georg Ebersbach, Gesine Respondek, Armin Giese, Thomas Arzberger, Günter Ulrich Höglinger
Postural instability and supranuclear gaze palsy represent the key symptoms of Richardson's syndrome, the most frequent clinical manifestation of progressive supranuclear palsy (PSP). However, a proportion of PSP patients never develops ocular motor symptoms, which prevents clinicians from establishing the diagnosis during lifetime according to current diagnostic criteria. We present one instructive autopsy-confirmed PSP case with prospective video-documented clinical course, showing striking temporal divergence of initially present postural instability and delayed development of ocular motor dysfunction...
November 14, 2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27813160/subcortical-18-f-av-1451-binding-patterns-in-progressive-supranuclear-palsy
#17
Hanna Cho, Jae Yong Choi, Mi Song Hwang, Seung Ha Lee, Young Hoon Ryu, Myung Sik Lee, Chul Hyoung Lyoo
BACKGROUND: Accumulation of cortical and subcortical tau pathology is the primary pathological substrate for progressive supranuclear palsy (PSP). (18) F-AV-1451, a radiotracer that binds to the pathological tau protein, may be helpful for in vivo visualization and quantitation of tau pathology in PSP. OBJECTIVES: The objectives of this study were to investigate cortical and subcortical (18) F-AV-1451 binding patterns in patients with PSP. METHODS: We recruited 14 PSP patients and compared their cortical and subcortical binding patterns in (18) F-AV-1451 positron emission tomography (PET) studies with those of 15 Parkinson's disease (PD) patients and 15 healthy controls...
November 3, 2016: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/27812167/usefulness-of-cardiac-mibg-scintigraphy-olfactory-testing-and-substantia-nigra-hyperechogenicity-as-additional-diagnostic-markers-for-distinguishing-between-parkinson-s-disease-and-atypical-parkinsonian-syndromes
#18
Hiroaki Fujita, Keisuke Suzuki, Ayaka Numao, Yuji Watanabe, Tomoyuki Uchiyama, Tomoyuki Miyamoto, Masayuki Miyamoto, Koichi Hirata
BACKGROUND: We aimed to evaluate the utility of the combined use of cardiac 123I-metaiodobenzylguanidine (MIBG) scintigraphy, olfactory testing, and substantia nigra (SN) hyperechogenicity on transcranial sonography (TCS) in differentiating Parkinson's disease (PD) from atypical parkinsonian syndromes (APSs), such as multiple system atrophy (MSA) and progressive supranuclear palsy (PSP). METHODS: Cardiac MIBG scintigraphy, card-type odor identification testing (Open Essence (OE), Wako, Japan), and TCS were performed with 101 patients with PD and 38 patients with APSs (MSA and PSP)...
2016: PloS One
https://www.readbyqxmd.com/read/27803826/a-corticobasal-syndrome-variant-of-familial-creutzfeldt-jakob-disease-with-stroke-like-onset
#19
Ján Necpál, Martin Stelzer, Silvia Koščová, Michal Patarák
Creutzfeldt-Jakob disease (CJD) is an untreatable rare human prion disease characterized by rapidly progressive dementia along with various neurological features, including myoclonus and sometimes other movement disorders. The clinical course is typically insidious and rapid, leading to an early death. In general, the most common form is sporadic CJD; however, Slovakia is typical for a high percentage of genetic cases. We present an unusual case report of a 65-year-old man with a sudden, stroke-like onset of motor aphasia with right-sided levodopa unresponsive parkinsonism, alien hand, and other characteristic features of corticobasal syndrome (CBS), with rapid deterioration and death on the 32nd day of the disease...
2016: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/27797445/tau-imaging-with-18-f-thk-5351-in-progressive-supranuclear-palsy
#20
A Ishiki, R Harada, N Okamura, N Tomita, C C Rowe, V L Villemagne, K Yanai, Y Kudo, H Arai, S Furumoto, M Tashiro, K Furukawa
BACKGROUND AND PURPOSE: Visualization of pathogenic protein aggregates is crucial to elucidate pathomechanisms and to make an accurate diagnosis in many neurodegenerative conditions. Aggregates of the microtubule-binding protein, tau, are one of the most important pathogenic molecules in neurodegenerative disorders. Progressive supranuclear palsy (PSP) is characterized by the deposition of tau proteins in some specific area such as the basal ganglia and brainstem. We tried to detect tau lesions in the brains of living patients with PSP with a novel positron emission tomography (PET) tracer, [(18) F]THK-5351, which we have recently developed...
October 31, 2016: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
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