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Multiple systems atrophy

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https://www.readbyqxmd.com/read/28632692/improved-123i-ioflupane-binding-after-immunotherapy-in-anti-nae-antibody-positive-hashimoto-encephalopathy-that-clinically-mimicked-multiple-system-atrophy
#1
Juuri Otsuka, Ayumi Hida, Kamiyu Ogyu, Ryogo Minamimoto, Sousuke Takeuchi
We describe an 84-year-old man with anti-NH2-terminal of α-enolase antibody-positive Hashimoto encephalopathy that clinically mimicked multiple system atrophy who underwent investigation by dopamine transporter SPECT before and after immunotherapy. Before treatment, dopamine transporter SPECT showed reduced striatal I-ioflupane binding, with a mean specific binding ratio of 2.42, even though he had no apparent parkinsonism. After immunotherapy, mean specific binding ratio was improved to 3.22. Dopamine transporter SPECT was useful in this case to detect subclinical striatal dysfunction, and evaluation both before and after immunotherapy helped to distinguish between neurodegenerative disease and neuroimmunological disorder...
June 19, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28632098/utility-and-accuracy-of-perceptual-voice-and-speech-distinctions-in-the-diagnosis-of-parkinson-s-disease-psp-and-msa-p
#2
Nick Miller, Uma Nath, Emma Noble, David Burn
AIM: To determine if perceptual speech measures distinguish people with Parkinson's disease (PD), multiple system atrophy with predominant parkinsonism (MSA-P) and progressive supranuclear palsy (PSP). METHODS: Speech-language therapists blind to patient characteristics employed clinical rating scales to evaluate speech/voice in 24 people with clinically diagnosed PD, 17 with PSP and 9 with MSA-P, matched for disease duration (mean 4.9 years, standard deviation 2...
June 20, 2017: Neurodegenerative Disease Management
https://www.readbyqxmd.com/read/28628270/serum-klotho-vitamin-d-and-homocysteine-in-combination-predict-the-outcomes-of-chinese-patients-with-multiple-system-atrophy
#3
Yue Guo, Xiao-Dong Zhuang, Wen-Biao Xian, Ling-Ling Wu, Ze-Na Huang, Xun Hu, Xiang-Song Zhang, Ling Chen, Xin-Xue Liao
AIMS: Neuroinflammation contributed to the pathogenesis of multiple system atrophy (MSA). We aimed to detect the correlation between inflammatory mediators, such as Klotho (Klt), vitamin D (25(OH)D) and homocysteine (Hcy), and disease severity among MSA patients. METHODS: A total of 53 MSA patients, 65 PD patients, and 62 normal subjects were recruited in our cross-sectional study. Serum Klotho (Klt), vitamin D (25(OH)D), and homocysteine (Hcy) levels were measured...
June 19, 2017: CNS Neuroscience & Therapeutics
https://www.readbyqxmd.com/read/28622212/a-retrospective-study-of-pimavanserin-use-in-a-movement-disorders-clinic
#4
Joseph H Friedman
Pimavanserin, a 5-HT2A inverse agonist, was commercially released in the United States in April 2016 for the treatment of Parkinson disease psychosis. No "naturalistic" treatment results have yet been published. Charts from the movement disorders clinic were reviewed for all patients who received this drug as treatment for psychosis associated with primary parkinsonism due to α-synucleinopathies. Data of 10 patients with idiopathic Parkinson disease, including 1 with a long history of schizophrenia, 4 with dementia with Lewy bodies, and 1 with multiple-system atrophy, were reviewed...
June 16, 2017: Clinical Neuropharmacology
https://www.readbyqxmd.com/read/28619074/novel-oligodendroglial-alpha-synuclein-viral-vector-models-of-multiple-system-atrophy-studies-in-rodents-and-nonhuman-primates
#5
Ronald J Mandel, David J Marmion, Deniz Kirik, Yaping Chu, Clifford Heindel, Thomas McCown, Steven J Gray, Jeffrey H Kordower
Multiple system atrophy (MSA) is a horrible and unrelenting neurodegenerative disorder with an uncertain etiology and pathophysiology. MSA is a unique proteinopathy in which alpha-synuclein (α-syn) accumulates preferentially in oligodendroglia rather than neurons. Glial cytoplasmic inclusions (GCIs) of α-syn are thought to elicit changes in oligodendrocyte function, such as reduced neurotrophic support and demyelination, leading to neurodegeneration. To date, only a murine model using one of three promoters exist to study this disease...
June 16, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28611571/exosomes-and-homeostatic-synaptic-plasticity-are-linked-to-each-other-and-to-huntington-s-parkinson-s-and-other-neurodegenerative-diseases-by-database-enabled-analyses-of-comprehensively-curated-datasets
#6
James K T Wang, Peter Langfelder, Steve Horvath, Michael J Palazzolo
Huntington's disease (HD) is a progressive and autosomal dominant neurodegeneration caused by CAG expansion in the huntingtin gene (HTT), but the pathophysiological mechanism of mutant HTT (mHTT) remains unclear. To study HD using systems biological methodologies on all published data, we undertook the first comprehensive curation of two key PubMed HD datasets: perturbation genes that impact mHTT-driven endpoints and therefore are putatively linked causally to pathogenic mechanisms, and the protein interactome of HTT that reflects its biology...
2017: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/28604565/droxidopa-for-symptomatic-neurogenic-hypotension
#7
Nadia Ferguson-Myrthil
Droxidopa is a first-in-class, orally available, synthetic amino acid precursor of norepinephrine that received accelerated Food and Drug Administration approval in February 2014 after Orphan Drug status for a debilitating condition known as symptomatic neurogenic orthostatic hypotension. Neurogenic disorders often lead to postural hypotension as a result of poor norepinephrine release from its storage sites. Clinical data suggest increases in standing systolic blood pressure and improvements in many other markers for subjective relief in patients with symptomatic neurogenic hypotension who received droxidopa therapy over 1-2 weeks...
May 4, 2017: Cardiology in Review
https://www.readbyqxmd.com/read/28603516/iodine-123-meta-iodobenzylguanidine-myocardial-scintigraphy-in-isolated-autonomic-failure-potential-red-flag-for-future-multiple-system-atrophy
#8
Francesca Baschieri, Giovanna Calandra-Buonaura, Annagrazia Cecere, Giorgio Barletta, Manuela Contin, Piero Parchi, Pietro Cortelli
Pure autonomic failure is challenging as it can be the presenting feature of a central nervous system syncleinopathy such as Parkinson's disease (PD) or multiple system atrophy (MSA). Because the prognosis of MSA and PD is so different, predictive features for a possible conversion can be extremely valuable. In this paper, we report three cases (two with autopsy-proven diagnosis) that had isolated AF for many years before converting to MSA or PD. Of all the tests that were performed during the premotor stage, Iodine-123-meta-iodobenzylguanidine (MIBG) myocardial scintigraphy was predictive of the conversion to MSA...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28602509/neurochip-an-updated-version-of-the-neurox-genotyping-platform-to-rapidly-screen-for-variants-associated-with-neurological-diseases
#9
Cornelis Blauwendraat, Faraz Faghri, Lasse Pihlstrom, Joshua T Geiger, Alexis Elbaz, Suzanne Lesage, Jean-Christophe Corvol, Patrick May, Aude Nicolas, Yevgeniya Abramzon, Natalie A Murphy, J Raphael Gibbs, Mina Ryten, Raffaele Ferrari, Jose Bras, Rita Guerreiro, Julie Williams, Rebecca Sims, Steven Lubbe, Dena G Hernandez, Kin Y Mok, Laurie Robak, Roy H Campbell, Ekaterina Rogaeva, Bryan J Traynor, Ruth Chia, Sun Ju Chung, John A Hardy, Alexis Brice, Nicholas W Wood, Henry Houlden, Joshua M Shulman, Huw R Morris, Thomas Gasser, Rejko Krüger, Peter Heutink, Manu Sharma, Javier Simón-Sánchez, Mike A Nalls, Andrew B Singleton, Sonja W Scholz
Genetics has proven to be a powerful approach in neurodegenerative diseases research, resulting in the identification of numerous causal and risk variants. Previously, we introduced the NeuroX Illumina genotyping array, a fast and efficient genotyping platform designed for the investigation of genetic variation in neurodegenerative diseases. Here, we present its updated version, named NeuroChip. The NeuroChip is a low-cost, custom-designed array containing a tagging variant backbone of about 306,670 variants complemented with a manually curated custom content comprised of 179,467 variants implicated in diverse neurological diseases, including Alzheimer's disease, Parkinson's disease, Lewy body dementia, amyotrophic lateral sclerosis, frontotemporal dementia, progressive supranuclear palsy, corticobasal degeneration, and multiple system atrophy...
May 17, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28601572/corpus-callosum-atrophy-as-a-marker-of-clinically-meaningful-cognitive-decline-in-secondary-progressive-multiple-sclerosis-impact-on-employment-status
#10
Athanasios Papathanasiou, Lambros Messinis, Petros Zampakis, Panagiotis Papathanasopoulos
Cognitive impairment in Multiple Sclerosis (MS) is more frequent and pronounced in secondary progressive MS (SPMS). Cognitive decline is an important predictor of employment status in patients with MS. Magnetic Resonance Imaging (MRI) markers have been used to associate tissue damage with cognitive dysfunction. The aim of the study was to designate the MRI marker that predicts cognitive decline in SPMS and explore its effect on employment status. 30 SPMS patients and 30 healthy participants underwent neuropsychological assessment using the Trail Making Test (TMT) parts A and B, semantic and phonological verbal fluency task and a computerized cognitive screening battery (Central Nervous System Vital Signs)...
June 7, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28601278/laquinimod-has-no-effects-on-brain-volume-or-cellular-cns-composition-in-the-f1-3xtg-ad-c3h-mouse-model-of-alzheimer-s-disease
#11
Rehana Z Hussain, William A Miller-Little, Doris Lambracht-Washington, Tom C Jaramillo, Masaya Takahashi, Shanrong Zhang, Min Fu, Gary R Cutter, Liat Hayardeny, Craig M Powell, Roger N Rosenberg, Olaf Stüve
BACKGROUND: Laquinimod is an anti-inflammatory agent with good central nervous system (CNS) bioavailability, and neuroprotective and myelorestorative properties. A clinical trial in patients with multiple sclerosis demonstrated that laquinimod significantly reduced loss of brain volume. The cellular substrate or molecular events underlying that treatment effect are unknown. In this study, we aimed to explore laquinimod's potential effects on brain volume, animal behavior, cellular numbers and composition of CNS-intrinsic cells and mononuclear cells within the CNS, amyloid beta (Aβ) accumulation and tau phosphorylation in the F1 3xTg-AD/C3H mouse model of Alzheimer's disease...
August 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28596752/the-retina-in-multiple-system-atrophy-systematic-review-and-meta-analysis
#12
REVIEW
Carlos E Mendoza-Santiesteban, Iñigo Gabilondo, Jose Alberto Palma, Lucy Norcliffe-Kaufmann, Horacio Kaufmann
BACKGROUND: Multiple system atrophy (MSA) is a rare, adult-onset, rapidly progressive fatal synucleinopathy that primarily affects oligodendroglial cells in the brain. Patients with MSA only rarely have visual complaints, but recent studies of the retina using optical coherence tomography (OCT) showed atrophy of the peripapillary retinal nerve fiber layer (RNFL) and to a lesser extent the macular ganglion cell layer (GCL) complex. METHODS: We performed a literature review and meta-analysis according to the preferred reporting items for systematic reviews and meta-analyses guidelines for studies published before January 2017, identified through PubMed and Google Scholar databases, which reported OCT-related outcomes in patients with MSA and controls...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28595550/new-tracers-and-new-perspectives-for-molecular-imaging-in-lewy-body-diseases
#13
Matteo Bauckneht, Dario Arnaldi, Flavio Nobili, Dag Aarsland, Silvia Morbelli
The term Lewy body diseases (LBDs) refers to a subset of neurodegenerative disorders that share the accumulation of the so-called Lewy bodies (LB) including: Parkinson's disease (PD), dementia with Lewy bodies (DLB), and PD later characterized by the occurrence of dementia (PDD). Moreover, multiple system atrophy (MSA) and idiopatic Rem Sleeping behaviour disorders (RBD) complete the group of synucleinopathies and have also common symptoms with respect to LBDs. The clinical diagnosis of LBDs can be challenging for physicians, particularly in the early stages of disease...
June 8, 2017: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/28592329/autoimmune-antibody-decline-in-parkinson-s-disease-and-multiple-system-atrophy-a-step-towards-immunotherapeutic-strategies
#14
Tomasz Brudek, Kristian Winge, Jonas Folke, Søren Christensen, Karina Fog, Bente Pakkenberg, Lars Østergaard Pedersen
BACKGROUND: Parkinson's' disease (PD) and Multiple System Atrophy (MSA) are progressive brain disorders characterized by intracellular accumulations of α-synuclein and nerve cell loss in specific brain areas. This loss causes problems with movement, balance and/or autonomic functions. Naturally occurring autoantibodies (NAbs) play potentially an important role in clearing or/and blocking circulating pathological proteins. Little is known about the functional properties of anti-α-synuclein NAbs in PD and MSA, and there have been opposing reports regarding their plasma concentrations in these disorders...
June 7, 2017: Molecular Neurodegeneration
https://www.readbyqxmd.com/read/28585223/a-molecular-tweezer-ameliorates-motor-deficits-in-mice-overexpressing-%C3%AE-synuclein
#15
Franziska Richter, Sudhakar R Subramaniam, Iddo Magen, Patrick Lee, Jane Hayes, Aida Attar, Chunni Zhu, Nicholas R Franich, Nicholas Bove, Krystal De La Rosa, Jacky Kwong, Frank-Gerrit Klärner, Thomas Schrader, Marie-Françoise Chesselet, Gal Bitan
Aberrant accumulation and self-assembly of α-synuclein are tightly linked to several neurodegenerative diseases called synucleinopathies, including idiopathic Parkinson's disease, dementia with Lewy bodies, and multiple system atrophy. Deposition of fibrillar α-synuclein as insoluble inclusions in affected brain cells is a pathological hallmark of synucleinopathies. However, water-soluble α-synuclein oligomers may be the actual culprits causing neuronal dysfunction and degeneration in synucleinopathies. Accordingly, therapeutic approaches targeting the toxic α-synuclein assemblies are attractive for these incurable disorders...
June 5, 2017: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
https://www.readbyqxmd.com/read/28572021/death-certificates-data-and-causes-of-death-in-patients-with-parkinsonism
#16
Mariana Moscovich, Gabriela Boschetti, Adriana Moro, Helio A G Teive, Anhar Hassan, Renato P Munhoz
INTRODUCTION: Assessment of variables related to mortality in Parkinson disease (PD) and other parkinsonian syndromes relies, among other sources, on accurate death certificate (DC) documentation. We assessed the documentation of the degenerative disorder on DCs and evaluated comorbidities and causes of death among parkinsonian patients. METHODS: Demographic and clinical data were systematically and prospectively collected on deceased patients followed at a tertiary movement disorder clinic...
May 26, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/28566176/effects-of-age-and-glucose-levels-on-lactate-levels-in-cerebrospinal-fluid-examination-of-neurodegenerative-diseases
#17
Fumihito Nakano, Ken Sakushima, Reona Umeki, Ichiro Yabe, Akira Endoh, Hidenao Sasaki
Despite recent studies examining the association between neurodegenerative diseases and mitochondrial dysfunction, there are not sufficient data on factors that influence cerebrospinal fluid (CSF) lactate levels. Thus, we investigated factors that affect CSF lactate levels in neurodegenerative diseases. We extracted laboratory findings, including CSF lactate, glucose, and protein levels, and demographic and background information, including age and gender, from the electronic medical records of patients with neurodegenerative diseases in order to explore factors that have an impact CSF lactate levels...
July 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28566154/usefulness-of-the-superior-cerebellar-peduncle-for-differential-diagnosis-of-progressive-supranuclear-palsy-a-meta-analysis
#18
Wataru Sako, Nagahisa Murakami, Yuishin Izumi, Ryuji Kaji
Previous studies have reported the usefulness of superior cerebellar peduncle (SCP) abnormalities in diagnosing progressive supranuclear palsy. However, the results of these studies were heterogeneous. In the present meta-analysis, we aimed to establish more robust evidence of SCP abnormalities in progressive supranuclear palsy, and to determine the cause of the previously reported heterogeneity. We identified six studies on SCP size and three studies on apparent diffusion coefficient. Key features of each study were extracted and standardized differences in size and apparent diffusion coefficient values were calculated...
July 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28564592/alteration-of-mitochondrial-protein-pdha1-in-lewy-body-disease-and-park14
#19
Yasuo Miki, Kunikazu Tanji, Fumiaki Mori, Akiyoshi Kakita, Hitoshi Takahashi, Koichi Wakabayashi
The histopathological hallmark of Parkinson's disease (PD) and dementia with Lewy bodies (DLB) is the occurrence of insoluble fibrillary aggregates known as Lewy bodies. Mitochondria play a vital role in energy production, and the pathogenesis of PD is associated with altered cellular metabolism due to mitochondrial dysfunction. The pyruvate dehydrogenase (PDH) complex provides a primary step in aerobic glucose metabolism by catalyzing the oxidative decarboxylation of pyruvate to acetyl CoA. Pyruvate dehydrogenase alpha 1 (PDHA1) forms the core structure of the PDH complex...
May 28, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28561779/transcriptomics-nf-%C3%AE%C2%BAb-pathway-and-their-potential-spaceflight-related-health-consequences
#20
REVIEW
Ye Zhang, Maria Moreno-Villanueva, Stephanie Krieger, Govindarajan T Ramesh, Srujana Neelam, Honglu Wu
In space, living organisms are exposed to multiple stress factors including microgravity and space radiation. For humans, these harmful environmental factors have been known to cause negative health impacts such as bone loss and immune dysfunction. Understanding the mechanisms by which spaceflight impacts human health at the molecular level is critical not only for accurately assessing the risks associated with spaceflight, but also for developing effective countermeasures. Over the years, a number of studies have been conducted under real or simulated space conditions...
May 31, 2017: International Journal of Molecular Sciences
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