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https://www.readbyqxmd.com/read/28091823/skeletal-muscle-inflammation-and-atrophy-in-heart-failure
#1
Kory J Lavine, Oscar L Sierra
Heart failure represents a systemic disease with profound effects on multiple peripheral tissues including skeletal muscle. Within the context of heart failure, perturbations in skeletal muscle physiology, structure, and function strongly contribute to exercise intolerance and the morbidity of this devastating disease. There is growing evidence that chronic heart failure imparts specific pathological changes within skeletal muscle beds resulting in muscle dysfunction and tissue atrophy. Mechanistically, systemic and local inflammatory responses drive critical aspects of this pathology...
January 14, 2017: Heart Failure Reviews
https://www.readbyqxmd.com/read/28090690/the-many-faces-of-autonomic-failure-in-multiple-system-atrophy
#2
EDITORIAL
Wassilios G Meissner, Anne Pavy-Le Traon
No abstract text is available yet for this article.
January 16, 2017: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/28087304/sleep-and-neurodegeneration-a-critical-appraisal
#3
REVIEW
Jagan A Pillai, James B Leverenz
Sleep abnormalities are clearly recognized as distinct clinical symptom of concern in neurodegenerative disorders. Appropriate management of sleep related symptoms has a positive impact on the quality of life of patients with neurodegenerative disorders. This review provides an overview of mechanisms that are currently being considered that tie sleep with neurodegeneration. It appraises the literature regarding specific sleep changes among common neurodegenerative diseases with a focus on Alzheimer's disease and synucleinopathies (i...
January 10, 2017: Chest
https://www.readbyqxmd.com/read/28074911/further-evidence-for-microtubule-independent-dimerization-of-tppp-p25
#4
J Oláh, T Szénási, S Szunyogh, A Szabó, A Lehotzky, J Ovádi
Tubulin Polymerization Promoting Protein (TPPP/p25) is a brain-specific disordered protein that modulates the dynamics and stability of the microtubule network by its assembly promoting, cross-linking and acetylation enhancing activities. In normal brain it is expressed primarily in differentiated oligodendrocytes; however, at pathological conditions it is enriched in inclusions of both neurons and oligodendrocytes characteristic for Parkinson's disease and multiple system atrophy, respectively. The objective of this paper is to highlight a critical point of a recently published Skoufias's paper in which the crucial role of the microtubules in TPPP/p25 dimerization leading to microtubule bundling was suggested...
January 11, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28069058/endosulfine-alpha-inhibits-membrane-induced-%C3%AE-synuclein-aggregation-and-protects-against-%C3%AE-synuclein-neurotoxicity
#5
Daniel Ysselstein, Benjamin Dehay, Isabel M Costantino, George P McCabe, Matthew P Frosch, Julia M George, Erwan Bezard, Jean-Christophe Rochet
Neuropathological and genetic findings suggest that the presynaptic protein α-synuclein (aSyn) is involved in the pathogenesis of synucleinopathy disorders, including Parkinson's disease (PD), dementia with Lewy bodies (DLB) and multiple system atrophy. Evidence suggests that the self-assembly of aSyn conformers bound to phospholipid membranes in an aggregation-prone state plays a key role in aSyn neurotoxicity. Accordingly, we hypothesized that protein binding partners of lipid-associated aSyn could inhibit the formation of toxic aSyn oligomers at membrane surfaces...
January 10, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28062667/smn-deficiency-negatively-impacts-red-pulp-macrophages-and-spleen-development-in-mouse-models-of-spinal-muscular-atrophy
#6
Marie-Therese Khairallah, Jacob Astroski, Sarah K Custer, Elliot J Androphy, Craig L Franklin, Christian L Lorson
Spinal muscular atrophy (SMA) is a progressive neurodegenerative disease that is the leading genetic cause of infantile death. It is caused by severe deficiency of the ubiquitously expressed Survival Motor Neuron (SMN) protein. SMA is characterized by α-lower motor neuron loss and muscle atrophy, however, there is a growing list of tissues impacted by SMN deficiency beyond motor neurons. The non-neuronal defects are observed in the most severe Type I SMA patients and most of the widely used SMA mouse models, however, as effective therapeutics are developed, it is unclear whether additional symptoms will be uncovered in longer lived patients...
January 5, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28060892/the-utility-of-post-void-residual-volume-versus-sphincter-electromyography-to-distinguish-between-multiple-system-atrophy-and-parkinson-s-disease
#7
Tatsuya Yamamoto, Masato Asahina, Yoshitaka Yamanaka, Tomoyuki Uchiyama, Shigeki Hirano, Miki Fuse, Yasuko Koga, Ryuji Sakakibara, Satoshi Kuwabara
OBJECTIVE: To determine the ability of sphincter electromyography (EMG) and post-void residual urine volume (PVR) during a free-flow study and a pressure-flow study (PFS) for distinguishing multiple system atrophy (MSA) from Parkinson's disease (PD). METHODS: We retrospectively reviewed 241 case records; both urodynamic study and sphincter EMG were performed in patients with MSA (n = 147) and PD (n = 94). RESULTS: There was a statistically significant difference (p < 0...
2017: PloS One
https://www.readbyqxmd.com/read/28059646/irreversible-changes-occurring-in-long-term-denervated-schwann-cells-affect-delayed-nerve-repair
#8
Giulia Ronchi, Michele Cillino, Giovanna Gambarotta, Benedetta Elena Fornasari, Stefania Raimondo, Pierfrancesco Pugliese, Pierluigi Tos, Adriana Cordova, Francesco Moschella, Stefano Geuna
OBJECTIVE Multiple factors may affect functional recovery after peripheral nerve injury, among them the lesion site and the interval between the injury and the surgical repair. When the nerve segment distal to the lesion site undergoes chronic degeneration, the ensuing regeneration (when allowed) is often poor. The aims of the current study were as follows: 1) to examine the expression changes of the neuregulin 1/ErbB system during long-term nerve degeneration; and 2) to investigate whether a chronically denervated distal nerve stump can sustain nerve regeneration of freshly axotomized axons...
January 6, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28057080/combination-of-alpha-synuclein-immunotherapy-with-anti-inflammatory-treatment-in-a-transgenic-mouse-model-of-multiple-system-atrophy
#9
Elvira Valera, Brian Spencer, Jerel A Fields, Ivy Trinh, Anthony Adame, Michael Mante, Edward Rockenstein, Paula Desplats, Eliezer Masliah
Multiple system atrophy (MSA) is a fatal neurodegenerative disorder characterized by the pathological accumulation of alpha-synuclein (α-syn) in oligodendrocytes. Therapeutic efforts to stop or delay the progression of MSA have yielded suboptimal results in clinical trials, and there are no efficient treatments currently available for MSA patients. We hypothesize that combining therapies targeting different aspects of the disease may lead to better clinical outcomes. To test this hypothesis, we combined the use of a single-chain antibody targeting α-syn modified for improved central nervous system penetration (CD5-D5) with an unconventional anti-inflammatory treatment (lenalidomide) in the myelin basic protein (MBP)-α-syn transgenic mouse model of MSA...
January 5, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28050656/the-recommendations-of-a-consensus-panel-for-the-screening-diagnosis-and-treatment-of-neurogenic-orthostatic-hypotension-and-associated-supine-hypertension
#10
REVIEW
Christopher H Gibbons, Peter Schmidt, Italo Biaggioni, Camille Frazier-Mills, Roy Freeman, Stuart Isaacson, Beverly Karabin, Louis Kuritzky, Mark Lew, Phillip Low, Ali Mehdirad, Satish R Raj, Steven Vernino, Horacio Kaufmann
Neurogenic orthostatic hypotension (nOH) is common in patients with neurodegenerative disorders such as Parkinson's disease, multiple system atrophy, pure autonomic failure, dementia with Lewy bodies, and peripheral neuropathies including amyloid or diabetic neuropathy. Due to the frequency of nOH in the aging population, clinicians need to be well informed about its diagnosis and management. To date, studies of nOH have used different outcome measures and various methods of diagnosis, thereby preventing the generation of evidence-based guidelines to direct clinicians towards 'best practices' when treating patients with nOH and associated supine hypertension...
January 3, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28034624/determinants-of-denervation-independent-depletion-of-putamen-dopamine-in-parkinson-s-disease-and-multiple-system-atrophy
#11
David S Goldstein, Patti Sullivan, Courtney Holmes, Deborah C Mash, Irwin J Kopin, Yehonatan Sharabi
BACKGROUND: Severe putamen dopamine depletion characterizes Parkinson's disease (PD) and multiple system atrophy (MSA). The extent of the depletion is greater than can be accounted for by loss of nigrostriatal dopaminergic terminals alone. We used putamen tissue levels and ratios of cysteinyl and parent catechols to explore possible denervation-independent abnormalities of dopamine synthesis and fate in PD and MSA. 5-S-Cysteinyldopa (Cys-DOPA) is produced from spontaneous oxidation of DOPA and 5-S-cysteinyldopamine (Cys-DA) from spontaneous oxidation of DA...
December 15, 2016: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/28031995/prevalence-of-progressive-supranuclear-palsy-in-yonago-change-throughout-a-decade
#12
Hiroshi Takigawa, Michio Kitayama, Kenji Wada-Isoe, Hisanori Kowa, Kenji Nakashima
BACKGROUND: Progressive supranuclear palsy (PSP) is a neurodegenerative disorder that is sometimes confused with Parkinson's disease, multiple system atrophy, and other disorders. The typical clinical features are categorized as Richardson's syndrome (RS), but other clinical subtypes include PSP-parkinsonism (PSP-P) and PSP-pure akinesia with gait freezing (PSP-PAGF). In this study, we determined the prevalence of PSP in a Japanese rural area compared to our previous 1999 report. METHODS: We collected data in Yonago City from 2009 to 2014 using a service-based study of PSP...
December 2016: Brain and Behavior
https://www.readbyqxmd.com/read/28017424/predictors-of-long-term-visual-outcome-in-intermediate-uveitis
#13
Rachael L Niederer, Lazha Sharief, Asaf Bar, Sue L Lightman, Oren Tomkins-Netzer
PURPOSE: To describe factors that predict visual loss and complications in intermediate uveitis. DESIGN: Cross-sectional study. PARTICIPANTS: Subjects with intermediate uveitis were identified from a database of 1254 uveitis patients seen in the clinic of a single consultant (S.L.L.) between 2011 and 2013. METHODS: Information was gathered from the clinical notes of all subjects examined in clinic. MAIN OUTCOME MEASURES: Best-corrected visual acuity (BCVA), moderate visual loss (MVL; ≤20/50), severe visual loss (SVL; ≤20/200)...
December 22, 2016: Ophthalmology
https://www.readbyqxmd.com/read/28017129/kynurenine-system-and-multiple-sclerosis-pathomechanism-and-drug-targets-with-an-emphasis-on-laquinimod
#14
Zsófia Majláth, Ádám Annus, László Vécsei
Multiple sclerosis is a common chronic, disabling autoimmune neurological disease affecting mainly young adults. In its pathomechanism, neurodegenerative and acute inflammatory characteristics are both involved. Disease-modifying therapies aim to reduce relapse-rate and slow down the deterioration in neurological functions. The currently available therapies fail to exert neuroprotective effects and most of them are associated with potentially toxic side-effects, therefore, ongoing research aims to develop novel drug candidates to cover these therapeutic gaps...
December 23, 2016: Current Drug Targets
https://www.readbyqxmd.com/read/28011746/the-pharmacology-of-autonomic-failure-from-hypotension-to-hypertension
#15
REVIEW
Italo Biaggioni
Primary neurodegenerative autonomic disorders are characterized clinically by loss of autonomic regulation of blood pressure. The clinical picture is dominated by orthostatic hypotension, but supine hypertension is also a significant problem. Autonomic failure can result from impairment of central autonomic pathways (multiple system atrophy) or neurodegeneration of peripheral postganglionic autonomic fibers (pure autonomic failure, Parkinson's disease). Pharmacologic probes such as the ganglionic blocker trimethaphan can help us in the understanding of the underlying pathophysiology and diagnosis of these disorders...
January 2017: Pharmacological Reviews
https://www.readbyqxmd.com/read/28011164/impact-of-sleep-apnea-syndrome-on-survival-in-patients-with-multiple-system-atrophy
#16
Olivier Flabeau, Imad Ghorayeb, Paul Perez, Aline Maillard, Jacques Taillard, Pierre Philip, Alexandra Foubert-Samier, François Tison, Wassilios G Meissner
INTRODUCTION: Sleep apnea is very frequent in multiple system atrophy (MSA) and may contribute to the poor prognosis. The aim of the present study was to prospectively assess the relation between sleep apnea and survival in 30 consecutive MSA patients recruited at the French Reference Center for MSA. METHODS: Patients with "probable" MSA according to current consensus diagnosis criteria were enrolled in this prospective cohort study. All patients received full polysomnography at baseline and were then followed for up to 4...
December 16, 2016: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/28004201/autonomic-disturbances-including-impaired-hand-thermoregulation-in-multiple-system-atrophy-and-parkinson-s-disease
#17
Sarunas Augustis, Viktoras Saferis, Wolfgang H Jost
Autonomic dysfunction in multiple system atrophy (MSA) comprises cardinal symptoms of orthostatic hypotension (OH) and urinary incontinence. Additionally, cardiovagal and sudomotor abnormalities can be present. Previous studies compared hand skin temperature and its response to cooling in subjects with probable MSA and Parkinson's disease (PD). Significant differences were found indicating that disturbed thermoregulation belongs to MSA autonomic features and could be helpful in differentiation of MSA from PD...
December 21, 2016: Journal of Neural Transmission
https://www.readbyqxmd.com/read/27993522/non-motor-symptoms-and-the-quality-of-life-in-multiple-system-atrophy-with-different-subtypes
#18
LingYu Zhang, Bei Cao, RuWei Ou, Qian-Qian Wei, Bi Zhao, Jing Yang, Ying Wu, HuiFang Shang
BACKGROUND: The differences in non-motor symptoms (NMS) and quality of life (QOL) between MSA patients with different subtypes remain unknown, so do the determinants of poor QOL in both subtypes. METHODS: A total of 172 MSA patients were enrolled in the study. NMS of patients with MSA were assessed using the non-motor symptoms scale (NMSS) and Parkinson's Disease Questionnaire-39 item version (PDQ-39) was used to evaluate the QOL of patients with MSA. RESULTS: The most prevalent NMS domain was urinary (91...
December 13, 2016: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/27987553/molecular-imaging-of-extrapyramidal-movement-disorders
#19
REVIEW
Kirk A Frey
Extrapyramidal movement disorders including Parkinson disease, multiple systems atrophy, progressive supranuclear palsy, and corticobasal degeneration are neurodegenerative syndromes with distinct neuropathological changes, indicating differing underlying etiologies. Clinical features that may distinguish among these conditions are often absent, particularly early after the onset of symptoms. Therapy is presently limited, and there are no established disease-modifying or neuroprotective interventions. Advances in therapeutics will depend on the early and accurate diagnostic classification of patients...
January 2017: Seminars in Nuclear Medicine
https://www.readbyqxmd.com/read/27983999/wdr73-missense-mutation-causes-infantile-onset-intellectual-disability-and-cerebellar-hypoplasia-in-a-consanguineous-family
#20
Chen Jiang, Nan Gai, Yongyi Zou, Yu Zheng, Ruiyu Ma, Xianda Wei, Desheng Liang, Lingqian Wu
Galloway-Mowat syndrome (GMS) is a very rare autosomal-recessive disorder characterized by nephrotic syndrome associated with microcephaly, and various central nervous system abnormalities, mostly cerebral hypoplasia or cerebellar atrophy, intellectual disability and neural-migration defects. WDR73 is the only gene known to cause GMS, and has never been implicated in other disease. Here we present a Chinese consanguineous family with infantile onset intellectual disability and cerebellar hypoplasia but no microcephaly...
January 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
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