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Progressive supranuclear palsy

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https://www.readbyqxmd.com/read/28436538/longitudinal-magnetic-resonance-imaging-in-progressive-supranuclear-palsy-a-new-combined-score-for-clinical-trials
#1
Günter U Höglinger, Jakob Schöpe, Maria Stamelou, Jan Kassubek, Teodoro Del Ser, Adam L Boxer, Stefan Wagenpfeil, Hans-Jürgen Huppertz
BACKGROUND: Two recent, randomized, placebo-controlled phase II/III trials (clinicaltrials.gov: NCT01110720, NCT01049399) of davunetide and tideglusib in progressive supranuclear palsy (PSP) generated prospective, 1-year longitudinal datasets of high-resolution T1-weighted three-dimensional MRI. OBJECTIVE: The objective of this study was to develop a quantitative MRI disease progression measurement for clinical trials. METHODS: The authors performed a fully automated quantitative MRI analysis employing atlas-based volumetry and provide sample size calculations based on data collected in 99 PSP patients assigned to placebo in these trials...
April 24, 2017: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/28432758/specificity-and-sensitivity-of-magnetic-resonance-imaging-findings-in-the-diagnosis-of-progressive-supranuclear-palsy
#2
REVIEW
Stephen Bacchi, Ivana Chim, Sandy Patel
Progressive supranuclear palsy (PSP) is a neurodegenerative condition that can only be diagnosed conclusively on pathological examination. Currently, the diagnosis is based upon the National Institute of Neurological Disorders and Stroke and the Society for PSP criteria. These criteria consist of purely clinical findings. Elements of brain MRI that are being investigated for this role include identifying structural features on conventional MRI, volume changes, signal abnormalities and diffusion changes. The aim of this study is to conduct a systematic search to identify which MRI findings have evidence to support their sensitivity/specificity/accuracy in the diagnosis of PSP...
April 22, 2017: Journal of Medical Imaging and Radiation Oncology
https://www.readbyqxmd.com/read/28421028/adult-onset-niemann-pick-disease-type-c-rapid-treatment-initiation-advised-but-early-diagnosis-remains-difficult
#3
Tobias Piroth, Kai Boelmans, Florian Amtage, Michel Rijntjes, Anna Wierciochin, Thomas Musacchio, Cornelius Weiller, Jens Volkmann, Stephan Klebe
Niemann-Pick type C disease (NP-C) presents with heterogeneous neurological and psychiatric symptoms. Adult onset is rare and possibly underdiagnosed due to frequent lack of specific and obvious key symptoms. For both early and adolescent/adult onset, the available data from studies and case reports describe a positive effect of Miglustat (symptom relief or stabilization). However, due to the low frequency of NP-C, experience with this therapy is still limited. We describe two adult-onset cases of NP-C. In both cases, vertical supranuclear gaze palsy was not recognized at symptom onset...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28420838/factors-associated-with-pneumonia-caused-death-in-older-adults-with-autopsy-confirmed-dementia
#4
Toshie Manabe, Katsuyoshi Mizukami, Hiroyasu Akatsu, Yoshio Hashizume, Takayoshi Ohkubo, Koichiro Kudo, Nobuyuki Hizawa
Objective A better understanding of risk factors for pneumonia-caused death may help to improve the clinical management of dementia. Methods A retrospective observational study was conducted by reviewing the medical charts and autopsy reports of 204 patients who were admitted to hospital, underwent a post-mortem examination, and who were neuropathologically diagnosed with dementia. The risk factors for pneumonia-caused death were examined both as underlying and immediate causes of death using logistic regression models...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28415165/progressive-supranuclear-gaze-palsy-with-predominant-cerebellar-ataxia-a-case-series-with-videos
#5
Zheyu Xu, Tchoyoson C C Lim, Wing Lok Au, Louis C S Tan
Progressive supranuclear palsy (PSP) with predominant cerebellar ataxia (PSP-C) is a rare phenotype of PSP. The clinical and radiological features of this disorder remain poorly characterized. Through a retrospective case series, we aim to characterize the clinical and radiological features of PSP-C. Four patients with PSP-C were identified: patients who presented with prominent cerebellar dysfunction that disappeared with the progression of the disease. Supranuclear gaze palsy occurred at a mean of 2.0 ± 2...
April 18, 2017: Journal of Movement Disorders
https://www.readbyqxmd.com/read/28413156/exposure-of-the-amino-terminus-of-tau-is-a-pathological-event-in-multiple-tauopathies
#6
Benjamin Combs, Nicholas M Kanaan
Pathological changes to the tau protein, including conformational changes and aggregation, are major hallmarks of a group of neurodegenerative disorders known as tauopathies. Among the conformational changes are alterations involving the extreme amino terminus of the protein, known as the phosphatase-activating domain (PAD). Aberrant PAD exposure induces a signaling cascade that leads to disruption of axonal transport, a critical function for neuronal survival. Conformational display of PAD is an early marker of pathological tau in Alzheimer disease (AD), but its role in other tauopathies has yet to be firmly established...
April 13, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28412965/multireceptor-fingerprints-in-progressive-supranuclear-palsy
#7
Wang Zheng Chiu, Laura Donker Kaat, Agnita J W Boon, Wouter Kamphorst, Axel Schleicher, Karl Zilles, John C van Swieten, Nicola Palomero-Gallagher
BACKGROUND: Progressive supranuclear palsy (PSP) with a frontal presentation, characterized by cognitive deficits and behavioral changes, has been recognized as an early clinical picture, distinct from the classical so-called Richardson and parkinsonism presentations. The midcingulate cortex is associated with executive and attention tasks and has consistently been found to be impaired in imaging studies of patients with PSP. The aim of the present study was to determine alterations in neurotransmission underlying the pathophysiology of PSP, as well as their significance for clinically identifiable PSP subgroups...
April 17, 2017: Alzheimer's Research & Therapy
https://www.readbyqxmd.com/read/28410663/frontotemporal-dementia
#8
REVIEW
Nicholas T Olney, Salvatore Spina, Bruce L Miller
Frontotemporal dementia (FTD) is a heterogeneous disorder with distinct clinical phenotypes associated with multiple neuropathologic entities. Presently, the term FTD encompasses clinical disorders that include changes in behavior, language, executive control, and often motor symptoms. The core FTD spectrum disorders include behavioral variant FTD, nonfluent/agrammatic variant primary progressive aphasia, and semantic variant PPA. Related FTD disorders include frontotemporal dementia with motor neuron disease, progressive supranuclear palsy syndrome, and corticobasal syndrome...
May 2017: Neurologic Clinics
https://www.readbyqxmd.com/read/28409749/the-role-of-stress-as-a-risk-factor-for-progressive-supranuclear-palsy
#9
Kristen D Kelley, Guerry Peavy, Steven Edland, Whitney Rogers, David E Riley, Yvette Bordelon, David Standaert, Stephen G Reich, Irene Litvan
BACKGROUND: PSP, like Alzheimer's disease (AD), is a tauopathy. The etiopathogenesis of PSP is not well known and the role of stress has not yet been examined. Recent studies have shown that stress increases the risk for developing AD. This study investigates the role of stress as a risk factor for PSP. OBJECTIVE: B To examine the association between the development of progressive supranuclear palsy (PSP) and self-reported life stressors. METHODS: 76 patients diagnosed with PSP according to the NINDS-SPSP criteria and 68 age-matched unrelated controls were administered a life stressor questionnaire...
April 13, 2017: Journal of Parkinson's Disease
https://www.readbyqxmd.com/read/28402959/meta-analysis-of-the-association-between-variants-in-mapt-and-neurodegenerative-diseases
#10
REVIEW
Cheng-Cheng Zhang, Jun-Xia Zhu, Yu Wan, Lin Tan, Hui-Fu Wang, Jin-Tai Yu, Lan Tan
Microtubule-associated protein tau (MAPT) gene is compelling among the susceptibility genes of neurodegenerative diseases which include Alzheimer's disease (AD), Parkinson's disease (PD), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). Our meta-analysis aimed to find the association between MAPT and the risk of these diseases. Published literatures were retrieved from MEDLINE and other databases, and 82 case-control studies were recruited...
March 29, 2017: Oncotarget
https://www.readbyqxmd.com/read/28389938/combined-visual-and-semi-quantitative-assessment-of-123-i-fp-cit-spect-for-the-diagnosis-of-dopaminergic-neurodegenerative-diseases
#11
Jun Ueda, Hajime Yoshimura, Keiji Shimizu, Megumu Hino, Nobuo Kohara
Visual and semi-quantitative assessments of (123)I-FP-CIT single-photon emission computed tomography (SPECT) are useful for the diagnosis of dopaminergic neurodegenerative diseases (dNDD), including Parkinson's disease, dementia with Lewy bodies, progressive supranuclear palsy, multiple system atrophy, and corticobasal degeneration. However, the diagnostic value of combined visual and semi-quantitative assessment in dNDD remains unclear. Among 239 consecutive patients with a newly diagnosed possible parkinsonian syndrome who underwent (123)I-FP-CIT SPECT in our medical center, 114 patients with a disease duration less than 7 years were diagnosed as dNDD with the established criteria or as non-dNDD according to clinical judgment...
April 7, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28386688/beyond-the-midbrain-atrophy-wide-spectrum-of-structural-mri-finding-in-cases-of-pathologically-proven-progressive-supranuclear-palsy
#12
REVIEW
Keita Sakurai, Aya M Tokumaru, Keigo Shimoji, Shigeo Murayama, Kazutomi Kanemaru, Satoru Morimoto, Ikuko Aiba, Motoo Nakagawa, Yoshiyuki Ozawa, Masashi Shimohira, Noriyuki Matsukawa, Yoshio Hashizume, Yuta Shibamoto
PURPOSE: Recently, it has been recognized that pathologically proven progressive supranuclear palsy (PSP) cases are classified into various clinical subtypes with non-uniform symptoms and imaging findings. This article reviews essential imaging findings, general information, and advanced magnetic resonance imaging (MRI) techniques for PSP and presents these MRI findings of pathologically proven typical and atypical PSP cases for educational purposes. METHODS: With the review of literatures, notably including atypical pathologically proven PSP cases, MRI and clinical information of 15 pathologically proven typical and atypical PSP cases were retrospectively evaluated...
April 6, 2017: Neuroradiology
https://www.readbyqxmd.com/read/28385472/gaba-spectra-and-remote-distractor-effect-in-progressive-supranuclear-palsy-a-pilot-study
#13
C Bonnet, J Rusz, J Hanuška, M Dezortová, F Jírů, T Sieger, R Jech, J Klempíř, J Roth, O Bezdíček, T Serranová, P Dušek, T Uher, C Flammand-Roze, M Hájek, E Růžička
Disturbances of the gamma-aminobutyric-acid (GABA) system have been suspected of contributing to the pathophysiology of progressive supranuclear palsy (PSP). The ability to rapidly resolve competitive action decisions, such as shifting the gaze to one particular stimulus rather than another, can be predicted by the concentration of GABA in the region of the frontal cortex relevant to eye movements. For this reason, our study measured GABA levels in seven PSP patients and eight healthy controls, using proton magnetic resonance spectroscopy, and assessed the relationship of these measurements to the remote distractor effect (RDE), an eye-movement paradigm investigating competitive action decisions...
April 3, 2017: Revue Neurologique
https://www.readbyqxmd.com/read/28381508/clinical-manifestations-of-the-anti-iglon5-disease
#14
Carles Gaig, Francesc Graus, Yarko Compta, Birgit Högl, Luis Bataller, Norbert Brüggemann, Caroline Giordana, Anna Heidbreder, Katya Kotschet, Jan Lewerenz, Stefan Macher, Maria J Martí, Teresa Montojo, Jesus Pérez-Pérez, Inmaculada Puertas, Caspar Seitz, Mateus Simabukuro, Nieves Téllez, Klaus-Peter Wandinger, Alex Iranzo, Guadalupe Ercilla, Lidia Sabater, Joan Santamaría, Josep Dalmau
OBJECTIVE: To report the presentation, main syndromes, human leukocyte antigen (HLA) association, and immunoglobulin G (IgG) subclass in the anti-IgLON5 disease: a disorder with parasomnias, sleep apnea, and IgLON5 antibodies. METHODS: This was a retrospective clinical analysis of 22 patients. The IgG subclass was determined using reported techniques. RESULTS: Patients' median age was 64 years (range 46-83). Symptoms that led to initial consultation included sleep problems (8 patients; 36%), gait abnormalities (8; 36%), bulbar dysfunction (3; 14%), chorea (2; 9%), and cognitive decline (1; 5%)...
April 5, 2017: Neurology
https://www.readbyqxmd.com/read/28378615/the-utility-of-fdg-pet-in-the-differential-diagnosis-of-parkinsonism
#15
Leposava Brajkovic, Vladimir Kostic, Dragana Sobic-Saranovic, Elka Stefanova, Milica Jecmenica-Lukic, Ana Jesic, Milica Stojiljkovic, Strahinja Odalovic, Francesca Gallivanone, Isabella Castiglioni, Branislava Radovic, Goran Trajkovic, Vera Artiko
INTRODUCTION: Differential diagnosis of parkinsonian disorders can be difficult on clinical grounds, especially in the early stage. Recent advancements in 18-F-fluorodeoxyglucose positron emission tomography (FDG-PET) imaging reveals different patterns of regional glucose metabolism in idiopathic Parkinson's disease (IPD) and atypical parkinsonian syndromes, such as multiple system atrophy (MSA), progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS), which may help differentiating between these conditions...
April 5, 2017: Neurological Research
https://www.readbyqxmd.com/read/28373956/a-neural-network-underlying-intentional-emotional-facial-expression-in-neurodegenerative-disease
#16
Kelly A Gola, Tal Shany-Ur, Peter Pressman, Isa Sulman, Eduardo Galeana, Hillary Paulsen, Lauren Nguyen, Teresa Wu, Babu Adhimoolam, Pardis Poorzand, Bruce L Miller, Katherine P Rankin
Intentional facial expression of emotion is critical to healthy social interactions. Patients with neurodegenerative disease, particularly those with right temporal or prefrontal atrophy, show dramatic socioemotional impairment. This was an exploratory study examining the neural and behavioral correlates of intentional facial expression of emotion in neurodegenerative disease patients and healthy controls. One hundred and thirty three participants (45 Alzheimer's disease, 16 behavioral variant frontotemporal dementia, 8 non-fluent primary progressive aphasia, 10 progressive supranuclear palsy, 11 right-temporal frontotemporal dementia, 9 semantic variant primary progressive aphasia patients and 34 healthy controls) were video recorded while imitating static images of emotional faces and producing emotional expressions based on verbal command; the accuracy of their expression was rated by blinded raters...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28373925/high-voltage-vim-region-deep-brain-stimulation-mimicking-progressive-supranuclear-palsy
#17
Addie Patterson, Michael S Okun, Christopher Hess
BACKGROUND: Deep brain stimulation (DBS) for essential tremor (ET) can cause unwanted side effects. CASE REPORT: A patient with ET underwent unilateral dual-lead thalamic DBS. He later developed parkinsonism with atypical features and was diagnosed with progressive supranuclear palsy. During presentation for a second opinion, stimulation-induced side effects were suspected. Inactivation of DBS resolved atypical features and superimposed idiopathic Parkinson disease (PD) was diagnosed...
2017: Tremor and Other Hyperkinetic Movements
https://www.readbyqxmd.com/read/28370398/tau-aggregates-where-when-why-and-what-consequences
#18
EDITORIAL
Matthew P Frosch
Neuropathologists have been aware of neurofibrillary tangles for more than a century following their description as part of Alois Alzheimer's initial report. Since those early days, our understanding of the relationship of this particular type of cellular inclusion associated with neurodegeneration has continually broadened. Textbooks, now a partially antiquated concept, commonly list a range of disorders as being associated with tangles -NDASH- including typical neurodegenerative diseases (Alzheimer disease [AD], forms of frontotemporal lobar degenerations [FTLD-tau], progressive supranuclear palsy [PSP], corticobasal degeneration [CBD], primary aged related tauopathy [PART]), secondary degenerative diseases such as chronic traumatic encephalopathy, metabolic disease (Niemann-Pick disease type C) and infections (SSPE)...
April 2, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28370167/tau-aggregates-where-when-why-and-what-consequences
#19
EDITORIAL
Matthew P Frosch
Neuropathologists have been aware of neurofibrillary tangles for more than a century following their description as part of Alois Alzheimer's initial report. Since those early days, our understanding of the relationship of this particular type of cellular inclusion associated with neurodegeneration has continually broadened. Textbooks, now a partially antiquated concept, commonly list a range of disorders as being associated with tangles -NDASH- including typical neurodegenerative diseases (Alzheimer disease [AD], forms of frontotemporal lobar degenerations [FTLD-tau], progressive supranuclear palsy [PSP], corticobasal degeneration [CBD], primary aged related tauopathy [PART]), secondary degenerative diseases such as chronic traumatic encephalopathy, metabolic disease (Niemann-Pick disease type C) and infections (SSPE)...
March 30, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28367478/central-hypoventilation-in-progressive-supranuclear-palsy
#20
Kory S Herrick, Randy Woltjer, Thao Pham, Megan Chalupsky, Amie L Hiller
Central hypoventilation, also known as Ondine's curse, results from an impairment of the autonomic respiratory drive. It is characterized by an attenuated or absent respiratory response to hypoxemia and hypercapnia with preservation of volitional respiratory function. RJ was a 75-year-old woman with a diagnosis of probably PSP who developed central hypoventilation approximately four years after her initial onset of symptoms. Brain MRI showed no evidence of medullary lesions, one of the more common causes of adult onset central hypoventilation...
January 2017: Movement Disorders Clinical Practice
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