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Multiple systems atrophy

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https://www.readbyqxmd.com/read/28440890/fluorescence-and-autoradiographic-evaluation-of-tau-pet-ligand-pbb3-to-%C3%AE-synuclein-pathology
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
https://www.readbyqxmd.com/read/28395279/serum-lymphocyte-associated-cytokine-concentrations-change-more-rapidly-over-time-in-multiple-system-atrophy-compared-to-parkinson-disease
#7
Keri Csencsits-Smith, Jessika Suescun, Kan Li, Sheng Luo, Diane L Bick, Mya Schiess
OBJECTIVE: Chronic inflammatory processes contribute to the eventual death of motor neurons and the development of symptoms in both idiopathic Parkinson disease (PD) and multiple system atrophy (MSA). Given the faster rate of progression and more severe symptoms associated with MSA, we hypothesized that markers of inflammation would be more evident in the peripheral blood of MSA than PD patients, and that evidence of this inflammation might assist early diagnosis of MSA versus PD. METHODS: We performed multiplex analysis to determine the concentrations of 37 immune-associated cytokines and chemokines isolated from the plasma of patients with PD (n = 25) and MSA (n = 14) and compared our results to those of age-matched controls (n = 15)...
April 11, 2017: Neuroimmunomodulation
https://www.readbyqxmd.com/read/28394902/correlation-of-hippocampal-atrophy-with-hyperhomocysteinemia-in-hemodialysis-patients-an-exploratory-pilot-study
#8
Kyoko Maesato, Takayasu Ohtake, Yasuhiro Mochida, Kunihiro Ishioka, Machiko Oka, Hidekazu Moriya, Sumi Hidaka, Shuzo Kobayashi
BACKGROUND: Cognitive impairment is one of the important critical issues in hemodialysis (HD) patients. However, the associating factors of brain atrophy in HD patients have not been fully elucidated. PURPOSE AND METHODS: Brain magnetic resonance imaging (MRI) was performed in 34 of total 72 HD outpatients in our dialysis center. These MRI images were analyzed by an application software; Voxel-based Specific Regional Analysis System for Alzheimer's Disease (VSRAD)...
2017: PloS One
https://www.readbyqxmd.com/read/28394031/degeneration-of-rapid-eye-movement-sleep-circuitry-underlies-rapid-eye-movement-sleep-behavior-disorder
#9
REVIEW
Dillon McKenna, John Peever
During healthy rapid eye movement sleep, skeletal muscles are actively forced into a state of motor paralysis. However, in rapid eye movement sleep behavior disorder-a relatively common neurological disorder-this natural process is lost. A lack of motor paralysis (atonia) in rapid eye movement sleep behavior disorder allows individuals to actively move, which at times can be excessive and violent. At first glance this may sound harmless, but it is not because rapid eye movement sleep behavior disorder patients frequently injure themselves or the person they sleep with...
April 10, 2017: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/28394027/changes-in-the-cell-population-in-brain-white-matter-in-multiple-system-atrophy
#10
Charlotte Havelund Nykjaer, Tomasz Brudek, Lisette Salvesen, Bente Pakkenberg
BACKGROUND: Multiple system atrophy (MSA) is a sporadic progressive neurodegenerative disorder with adult onset and unknown etiology. Clinically it is characterized by autonomic failure, cerebellar ataxia, parkinsonism, and corticospinal dysfunction in any combination and with varying severity. OBJECTIVES AND METHODS: To establish the extent of involvement of the white matter in the disease, we have used stereology to quantify the total number of neurons and glial cells (oligodendrocytes, astrocytes, and microglia) in the brains from 10 MSA patients and 11 controls...
April 10, 2017: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/28392299/corrigendum-to-determinants-of-denervation-independent-depletion-of-putamen-dopamine-in-parkinson-s-disease-and-multiple-system-atrophy-parkinsonism-relat-disord-35-2017-88-91
#11
David S Goldstein, Patti Sullivan, Courtney Holmes, Deborah C Mash, Irwin J Kopin, Yehonatan Sharabi
No abstract text is available yet for this article.
April 6, 2017: Parkinsonism & related Disorders
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
#12
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/28385128/retrograde-trans-synaptic-visual-pathway-degeneration-in-multiple-sclerosis-a-case-series
#13
Omar Al-Louzi, Julia Button, Scott D Newsome, Peter A Calabresi, Shiv Saidha
BACKGROUND: Trans-synaptic degeneration (TSD) describes the propagation of neuronal injury through synaptic pathways in the human nervous system and may be linked to the accelerated retinal atrophy seen in multiple sclerosis (MS). RESULTS: We report six cases where homonymous, hemi-macular ganglion cell + inner plexiform (GCIP) thickness reduction was seen in conjunction with posterior visual pathway lesions. Macular microcystoid changes of the inner nuclear layer (INL) were seen in a subset of three subjects...
April 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28385127/retinal-nerve-fiber-layer-thickness-and-neuropsychiatric-manifestations-in-systemic-lupus-erythematosus
#14
S Shulman, R Shorer, J Wollman, G Dotan, D Paran
Background Cognitive impairment is frequent in systemic lupus erythematosus. Atrophy of the corpus callosum and hippocampus have been reported in patients with systemic lupus erythematosus, and diffusion tensor imaging studies have shown impaired white matter integrity, suggesting that white matter damage in systemic lupus erythematosus may underlie the cognitive impairment as well as other neuropsychiatric systemic lupus erythematosus manifestations. Retinal nerve fiber layer thickness, as assessed by optical coherence tomography, has been suggested as a biomarker for white matter damage in neurologic disorders such as multiple sclerosis, Alzheimer's disease and Parkinson's disease...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28382615/a-case-of-multiple-system-atrophy-with-preexisting-alzheimer-s-disease-and-predating-the-hot-cross-bun-sign
#15
Chi-Wei Lin, Chi-Yu Tseng, Chung-Ping Lo, Min-Chien Tu
PURPOSE: Synucleinopathy, tauopathy and amyloidopathy were classified as distinct clinical and pathological entities in traditional classification systems, and their interactions have been studied on neuropathology and molecular genetics recently. CASE REPORT: In this report, we present a 69-year-old male patient who had been diagnosed with probable Alzheimer's disease (AD) dementia due to progressive forgetfulness in February 2013. His Mini- Mental State Examination score was 21/30, and his Cognitive Abilities Screening Instrument score was 78/100, resulted from profound deficits in recent memory and abstract thinking domains...
December 15, 2016: Acta Neurologica Taiwanica
https://www.readbyqxmd.com/read/28378615/the-utility-of-fdg-pet-in-the-differential-diagnosis-of-parkinsonism
#16
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/28378233/multiple-system-atrophy-state-of-the-art
#17
REVIEW
Brice Laurens, Sylvain Vergnet, Miguel Cuina Lopez, Alexandra Foubert-Samier, François Tison, Pierre-Olivier Fernagut, Wassilios G Meissner
Multiple system atrophy (MSA) is a rare and fatal neurodegenerative disorder that is characterized by a variable combination of parkinsonism, cerebellar impairment, and autonomic dysfunction. Some symptomatic treatments are available while neuroprotection or disease-modification remain unmet treatment needs. The pathologic hallmark is the accumulation of aggregated alpha-synuclein (α-syn) in oligodendrocytes forming glial cytoplasmic inclusions, which qualifies MSA as synucleinopathy together with Parkinson's disease and dementia with Lewy bodies...
May 2017: Current Neurology and Neuroscience Reports
https://www.readbyqxmd.com/read/28374506/g-csf-and-cognitive-dysfunction-in-elderly-diabetic-mice-with-cerebral-small-vessel-disease-preventive-intervention-effects-and-underlying-mechanisms
#18
Zhu-Fei Guan, Ying-Hong Tao, Xiao-Ming Zhang, Qi-Lin Guo, Ying-Chao Liu, Yu Zhang, Yan-Mei Wang, Gang Ji, Guo-Feng Wu, Na-Na Wang, Hao Yang, Zhong-Yu Yu, Jing-Chun Guo, Hou-Guang Zhou
AIMS: Although cognitive dysfunction is a common neurological complication in elderly patients with diabetes, the mechanisms underlying this relationship remain unclear, and effective preventive interventions have yet to be developed. Thus, this study investigated the preventive effects and mechanisms of action associated with granulocyte colony-stimulating factor (G-CSF) on cognitive dysfunction in elderly diabetic mice with cerebral small vessel disease. METHODS: This study included 40 male db/db diabetic and wild-type (WT) mice that were categorized into the following four groups at the age of 3 weeks: db/db group (DG), db/db+G-CSF group (DGG), WT group (WG), and WT+G-CSF group (WGG)...
April 4, 2017: CNS Neuroscience & Therapeutics
https://www.readbyqxmd.com/read/28374232/monitoring-the-course-of-ms-with-optical-coherence-tomography
#19
REVIEW
Alexander U Brandt, Elena H Martinez-Lapiscina, Rachel Nolan, Shiv Saidha
Retinae of patients with multiple sclerosis (MS), as part of the central nervous system (CNS), display inflammatory and neurodegenerative changes. There is increasing evidence suggesting that retinal changes, and in particular neurodegeneration, mirror global CNS alterations in MS. Spectral domain optical coherence tomography (SD-OCT) is an inexpensive, rapid, non-invasive, and reproducible imaging technique that generates high-resolution images of tissues such as the retina. An advantage of SD-OCT over magnetic resonance imaging techniques in the assessment of neurodegeneration may be its sensitivity to capture changes at the individual patient level...
April 2017: Current Treatment Options in Neurology
https://www.readbyqxmd.com/read/28369467/muscle-specific-expression-of-the-rna-binding-protein-staufen1-induces-progressive-skeletal-muscle-atrophy-via-regulation-of-phosphatase-tensin-homolog
#20
Tara E Crawford Parks, Aymeric Ravel-Chapuis, Emma Bondy-Chorney, Jean-Marc Renaud, Jocelyn Côté, Bernard J Jasmin
Converging lines of evidence have now highlighted the key role for post-transcriptional regulation in the neuromuscular system. In particular, several RNA-binding proteins are known to be misregulated in neuromuscular disorders including myotonic dystrophy type 1, spinal muscular atrophy and amyotrophic lateral sclerosis. In this study, we focused on the RNA-binding protein Staufen1, which assumes multiple functions in both skeletal muscle and neurons. Given our previous work that showed a marked increase in Staufen1 expression in various physiological and pathological conditions including denervated muscle, in embryonic and undifferentiated skeletal muscle, in rhabdomyosarcomas as well as in myotonic dystrophy type 1 muscle samples from both mouse models and humans, we investigated the impact of sustained Staufen1 expression in postnatal skeletal muscle...
March 24, 2017: Human Molecular Genetics
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