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multiple system atrophy

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https://www.readbyqxmd.com/read/28531809/multifocal-visual-evoked-potentials-in-optic-neuritis-and-multiple-sclerosis-a-review
#1
REVIEW
Gorm Pihl-Jensen, Mathias Falck Schmidt, Jette Lautrup Frederiksen
Multifocal visual evoked potential (mf-VEP) represents a new approach to the classical full field (ff-)VEP with separate responses from up to 60 sectors of the visual field. A thorough literature survey of the use of mf-VEP in optic neuritis (ON) and multiple sclerosis (MS) is presented (38 published studies were retrieved). Mf-VEP provides direct topographical information of specific lesions and facilitates investigations on structural-functional correlations thus providing new methods for exploring the interplay between demyelination, atrophy and remyelination in MS...
April 11, 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28527404/relevance-of-non-specific-mri-features-in-multiple-system-atrophy
#2
Sunil Pradhan, Ruchika Tandon
OBJECTIVES: Rarity of specific MRI features like 'hot-cross bun' sign and 'hyperintense putamen rim' reduce diagnostic utility of MRI in MSA. We therefore, studied some non-specific MRI features in addition to the specific ones, to find their diagnostic utility. PATIENTS AND METHODS: Clinical and MRI features of 53 indoor and outdoor patients with MSA were analyzed in the context of its Parkinsonian (MSA-P) and cerebellar (MSA-C) variants. RESULTS: Of 53 cases (mean age: 59...
May 10, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28522081/natural-course-and-potential-prognostic-factors-for-sleep-disordered-breathing-in-multiple-system-atrophy
#3
Yasuyoshi Ohshima, Hideaki Nakayama, Naho Matsuyama, Satoshi Hokari, Takuro Sakagami, Tomoe Sato, Toshiyuki Koya, Tetsuya Takahashi, Toshiaki Kikuchi, Masatoyo Nishizawa, Takayoshi Shimohata
OBJECTIVE/BACKGROUND: Multiple system atrophy (MSA) frequently results in the development of sleep-disordered breathing (SDB). Few reports have described the natural course of this phenomenon. The aim of the present study was to determine the natural course of SDB and prognostic factors associated with such conditions in MSA. PATIENTS/METHODS: Twenty-four consecutive patients were recruited with probable MSA, who had not been treated with continuous positive airway pressure (CPAP) and had undergone overnight polysomnography (PSG) more than once following the development of snoring or stridor...
June 2017: Sleep Medicine
https://www.readbyqxmd.com/read/28513079/rem-sleep-behavior-disorder-in-parkinson-s-disease-and-other-synucleinopathies
#4
REVIEW
Erik K St Louis, Angelica R Boeve, Bradley F Boeve
Rapid eye movement sleep behavior disorder is characterized by dream enactment and complex motor behaviors during rapid eye movement sleep and rapid eye movement sleep atonia loss (rapid eye movement sleep without atonia) during polysomnography. Rapid eye movement sleep behavior disorder may be idiopathic or symptomatic and in both settings is highly associated with synucleinopathy neurodegeneration, especially Parkinson's disease, dementia with Lewy bodies, multiple system atrophy, and pure autonomic failure...
May 2017: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/28505261/survival-and-causes-of-death-among-people-with-clinically-diagnosed-synucleinopathies-with-parkinsonism-a-population-based-study
#5
Rodolfo Savica, Brandon R Grossardt, James H Bower, J Eric Ahlskog, Bradley F Boeve, Jonathan Graff-Radford, Walter A Rocca, Michelle M Mielke
Importance: To our knowledge, a comprehensive study of the survival and causes of death of persons with synucleinopathies compared with the general population has not been conducted. Understanding the long-term outcomes of these conditions may inform patients and caregivers of the expected disease duration and may help with care planning. Objective: To compare survival rates and causes of death among patients with incident, clinically diagnosed synucleinopathies and age- and sex-matched referent participants...
May 15, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28501823/cerebellar-atrophy-in-neurodegeneration-a-meta-analysis
#6
REVIEW
Helena M Gellersen, Christine C Guo, Claire O'Callaghan, Rachel H Tan, Saber Sami, Michael Hornberger
INTRODUCTION: The cerebellum has strong cortical and subcortical connectivity, but is rarely taken into account for clinical diagnosis in many neurodegenerative conditions, particularly in the absence of clinical ataxia. The current meta-analysis aims to assess patterns of cerebellar grey matter atrophy in seven neurodegenerative conditions (Alzheimer's disease (AD), Parkinson's disease (PD) and Huntington's disease (HD), frontotemporal dementia (FTD), amyotrophic lateral sclerosis (ALS), multiple system atrophy (MSA), progressive supranuclear palsy (MSP))...
May 13, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28500752/which-ante-mortem-clinical-features-predict-progressive-supranuclear-palsy-pathology
#7
Gesine Respondek, Carolin Kurz, Thomas Arzberger, Yaroslau Compta, Elisabet Englund, Leslie W Ferguson, Ellen Gelpi, Armin Giese, David J Irwin, Wassilios G Meissner, Christer Nilsson, Alexander Pantelyat, Alex Rajput, John C van Swieten, Claire Troakes, Keith A Josephs, Anthony E Lang, Brit Mollenhauer, Ulrich Müller, Jennifer L Whitwell, Angelo Antonini, Kailash P Bhatia, Yvette Bordelon, Jean-Christophe Corvol, Carlo Colosimo, Richard Dodel, Murray Grossman, Jan Kassubek, Florian Krismer, Johannes Levin, Stefan Lorenzl, Huw Morris, Peter Nestor, Wolfgang H Oertel, Gil D Rabinovici, James B Rowe, Thilo van Eimeren, Gregor K Wenning, Adam Boxer, Lawrence I Golbe, Irene Litvan, Maria Stamelou, Günter U Höglinger
BACKGROUND: Progressive supranuclear palsy (PSP) is a neuropathologically defined disease presenting with a broad spectrum of clinical phenotypes. OBJECTIVE: To identify clinical features and investigations that predict or exclude PSP pathology during life, aiming at an optimization of the clinical diagnostic criteria for PSP. METHODS: We performed a systematic review of the literature published since 1996 to identify clinical features and investigations that may predict or exclude PSP pathology...
May 13, 2017: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/28496050/multiple-system-atrophy-clinicopathological-characteristics-in-japanese-patients
#8
Tetsutaro Ozawa, Osamu Onodera
Multiple system atrophy (MSA) is an adult-onset neurodegenerative disorder that has both clinical and pathological variants. Clinical examples include MSA with predominant cerebellar ataxia (MSA-C) and MSA with predominant parkinsonism (MSA-P), whereas olivopontocerebellar atrophy and striatonigral degeneration represent pathological variants. We performed systematic reviews of studies that addressed the relative frequencies of clinical or pathological variants of MSA in various populations to determine the clinicopathological characteristics in Japanese MSA...
2017: Proceedings of the Japan Academy. Series B, Physical and Biological Sciences
https://www.readbyqxmd.com/read/28490649/pathologic-confirmation-of-retinal-ganglion-cell-loss-in-multiple-system-atrophy
#9
Carlos E Mendoza-Santiesteban, Jose-Alberto Palma, Isabel Ortuño-Lizarán, Nicolás Cuenca, Horacio Kaufmann
No abstract text is available yet for this article.
May 10, 2017: Neurology
https://www.readbyqxmd.com/read/28482862/higher-levels-of-myelin-phospholipids-in-brains-of-neuronal-%C3%AE-synuclein-transgenic-mice-precede-myelin-loss
#10
Jessica Grigoletto, Katharina Pukaß, Ayelet Gamliel, Dana Davidi, Rachel Katz-Brull, Christiane Richter-Landsberg, Ronit Sharon
α-Synuclein is a protein involved in the pathogenesis of synucleinopathies, including Parkinson's disease (PD), dementia with Lewy bodies (DLB) and multiple system atrophy (MSA). We investigated the role of neuronal α-Syn in myelin composition and abnormalities. The phospholipid content of purified myelin was determined by (31)P NMR in two mouse lines modeling PD, PrP-A53T α-Syn and Thy-1 wt-α-Syn. Significantly higher levels of phospholipids were detected in myelin purified from brains of these α-Syn transgenic mouse models than in control mice...
May 8, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28469572/retrospective-diagnosis-of-parkinsonian-syndromes-using-whole-brain-atrophy-rates
#11
Carlos Guevara, Kateryna Bulatova, Wendy Soruco, Guido Gonzalez, Gonzalo A Farías
Objective: The absence of markers for ante-mortem diagnosis of idiopathic Parkinson's disease (IPD), multiple system atrophy (MSA), and progressive supranuclear palsy (PSP) results in these disorders being commonly mistaken for each other, particularly in the initial stages. We aimed to investigate annualized whole-brain atrophy rates (a-WBAR) in these disorders to aid in the diagnosis between IPD vs. PSP and MSA. Methods: Ten healthy controls, 20 IPD, 39 PSP, and 41 MSA patients were studied using Structural Imaging Evaluation with Normalization of Atrophy (SIENA)...
2017: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/28464831/principal-component-analysis-for-ataxic-gait-using-a-triaxial-accelerometer
#12
Akira Matsushima, Kunihiro Yoshida, Hirokazu Genno, Shu-Ichi Ikeda
BACKGROUND: It is quite difficult to evaluate ataxic gait quantitatively in clinical practice. The aim of this study was to analyze the characteristics of ataxic gait using a triaxial accelerometer and to develop a novel biomarker of integrated gate parameters for ataxic gait. METHODS: Sixty-one patients with spinocerebellar ataxia (SCA) or multiple system atrophy with predominant cerebellar ataxia (MSA-C) and 57 healthy control subjects were enrolled. The subjects were instructed to walk 10 m for a total of 12 times on a flat floor at their usual walking speed with a triaxial accelerometer attached to their back...
May 2, 2017: Journal of Neuroengineering and Rehabilitation
https://www.readbyqxmd.com/read/28461838/bilateral-chorioretinal-scars-in-a-child-case-report
#13
Tomi Anca, Stefan Irina
The eye manifestations of intrauterine infections are multiple, but chorioretinal scars and/or active chorioretinitis are the most frequently reported. When associated with other systemic manifestations of the infection, the diagnosis becomes more obvious, but when eye involvement is the only abnormality, etiology often remains uncertain. We are presenting the case of an 8-year-old female patient whose fundus lesions revealed an unusual choroidoretinopathy, associated with cataract in one eye. Her general examination and her medical history were unremarkable...
June 2016: Mædica
https://www.readbyqxmd.com/read/28458138/quercetin-promotes-motor-and-sensory-function-recovery-following-sciatic-nerve-crush-injury-in-c57bl-6j-mice
#14
Ming-Ming Chen, Jing Qin, Shu-Jian Chen, Le-Meng Yao, Lu-Yong Zhang, Zhi-Qi Yin, Hong Liao
Injuries and diseases that occur in the nervous system are common and have few effective treatments. Previous studies have shown that quercetin has a therapeutic effect on nervous system injuries, but its potential effects on and mechanisms of action related to behavioral recovery and axonal regrowth have not been investigated. Here, we showed that quercetin administration promotes behavioral recovery following sciatic nerve-crush injury in mice. Long-term evaluation showed that mice administered 20 mg·kg(-1)·day(-1) quercetin for 35 days had a greater sensorimotor recovery compared with all other treatment groups...
April 17, 2017: Journal of Nutritional Biochemistry
https://www.readbyqxmd.com/read/28440890/fluorescence-and-autoradiographic-evaluation-of-tau-pet-ligand-pbb3-to-%C3%AE-synuclein-pathology
#15
Shunsuke Koga, Maiko Ono, Naruhiko Sahara, Makoto Higuchi, Dennis W Dickson
BACKGROUND: The tau PET ligand 2-((1E,3E)-4-(6-([(11) C]methylamino)pyridin-3-yl)buta-1,3-dienyl)benzo[d]thiazol-6-ol ([(11) C]PBB3) binds to a wide range of tau pathology; however, binding property of PBB3 to non-tau inclusions remains unknown. To clarify whether [(11) C]PBB3 binds to α-synuclein pathology, reactivity of PBB3 was assessed by in vitro fluorescence and autoradiographic labeling of brain sections from α-synucleinopathies patients. METHOD: Of 10 pure Lewy body disease and 120 multiple system atrophy (MSA) cases in the Mayo Clinic brain bank, we selected 3 Lewy body disease and 4 MSA cases with a range of α-synuclein severity based on the quantitative analysis of α-synuclein burden...
April 25, 2017: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/28438224/early-strong-intrathecal-inflammation-in-cerebellar-type-multiple-system-atrophy-by-cerebrospinal-fluid-cytokine-chemokine-profiles-a-case-control-study
#16
Ryo Yamasaki, Hiroo Yamaguchi, Takuya Matsushita, Takayuki Fujii, Akio Hiwatashi, Jun-Ichi Kira
BACKGROUND: The pathology of multiple system atrophy cerebellar-type (MSA-C) includes glial inflammation; however, cerebrospinal fluid (CSF) inflammatory cytokine profiles have not been investigated. In this study, we determined CSF cytokine/chemokine/growth factor profiles in MSA-C and compared them with those in hereditary spinocerebellar ataxia (SCA). METHODS: We collected clinical data and CSF from 20 MSA-C patients, 12 hereditary SCA patients, and 15 patients with other non-inflammatory neurological diseases (OND), and measured 27 cytokines/chemokines/growth factors using a multiplexed fluorescent bead-based immunoassay...
April 24, 2017: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/28432997/serum-uric-acid-level-is-linked-to-the-disease-progression-rate-in-male-patients-with-multiple-system-atrophy
#17
Jiro Fukae, Shinsuke Fujioka, Shosaburo Yanamoto, Akio Mori, Takahiro Nomi, Taku Hatano, Kousuke Fukuhara, Shinji Ouma, Nobutaka Hattori, Yoshio Tsuboi
OBJECTIVES: Multiple system atrophy (MSA) is a progressive neurodegenerative disorder that may be caused in part by oxidative stress. Uric acid (UA) protects neurons in neurodegenerative disorders via antioxidative effects. The aim of this study was to investigate the relationship between the serum UA concentration and disease progression in MSA patients. PATIENTS AND METHODS: A total of 53 Japanese MSA patients were enrolled in this study. The disease progression rate was estimated by the rate of global disability scale change per year...
April 3, 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28420950/metallothionein-copper-and-alpha-synuclein-in-alpha-synucleinopathies
#18
REVIEW
Yuho Okita, Alexandre N Rcom-H'cheo-Gauthier, Michael Goulding, Roger S Chung, Peter Faller, Dean L Pountney
Metallothioneins (MTs) are proteins that function by metal exchange to regulate the bioavailability of metals, such as zinc and copper. Copper functions in the brain to regulate mitochondria, neurotransmitter production, and cell signaling. Inappropriate copper binding can result in loss of protein function and Cu(I)/(II) redox cycling can generate reactive oxygen species. Copper accumulates in the brain with aging and has been shown to bind alpha-synuclein and initiate its aggregation, the primary aetiological factor in Parkinson's disease (PD), and other alpha-synucleinopathies...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28419566/perirhinal-accumulation-of-neuronal-alpha-synuclein-in-a-multiple-system-atrophy-patient-with-dementia
#19
Mari Saito, Makoto Hara, Momoko Ebashi, Akihiko Morita, Kyoko Okada, Taku Homma, Masahiko Sugitani, Kentaro Endo, Toshiki Uchihara, Satoshi Kamei
We report the case of a 79-year-old Japanese woman who developed cerebellar ataxia followed by rigidity, dysautonomia and cognitive disorders, and was thus clinically diagnosed as having possible MSA with dementia. Neuropathological findings demonstrated not only olivopontocerebellar and striatonigral degeneration with frequent glial cytoplasmic inclusions (GCIs), but also degenerative changes in the parahippocampal region, accentuated in the anterior portion of perirhinal cortex, where neuronal cytoplasmic inclusions (NCIs) and NFTs were numerous while GCIs were limited...
April 16, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28395742/reporting-on-methods-to-generate-and-purify-rodent-and-human-oligodendrocytes-from-different-sources
#20
Mehdi Djelloul, Carla Azevedo, Yuriy Pomeshchik, Anna Hammarberg, Laurent Roybon
Oligodendrocytes are part of the glial cells located in the central nervous system, capable of providing trophic support to neurons and ensheathing their axons. These cells can become dysfunctional under pathologic condition. Rodent and human pluripotent stem cells are inexhaustible sources for producing oligodendrocytes that can be used for disease modeling and cell replacement therapy studies. They also offer many opportunities to model the contribution of oligodendrocytes in non-genetic disorders such as multiple system atrophy...
April 2017: Stem Cell Research
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