keyword
https://read.qxmd.com/read/36859484/alpha-synuclein-in-parkinson-s-disease-and-other-synucleinopathies-from-overt-neurodegeneration-back-to-early-synaptic-dysfunction
#21
REVIEW
Paolo Calabresi, Alessandro Mechelli, Giuseppina Natale, Laura Volpicelli-Daley, Giulia Di Lazzaro, Veronica Ghiglieri
Although the discovery of the critical role of α-synuclein (α-syn) in the pathogenesis of Parkinson's disease (PD) is now twenty-five years old, it still represents a milestone in PD research. Abnormal forms of α-syn trigger selective and progressive neuronal death through mitochondrial impairment, lysosomal dysfunction, and alteration of calcium homeostasis not only in PD but also in other α-syn-related neurodegenerative disorders such as dementia with Lewy bodies, multiple system atrophy, pure autonomic failure, and REM sleep behavior disorder...
March 1, 2023: Cell Death & Disease
https://read.qxmd.com/read/36749600/-d-685-reverses-motor-deficits-and-reduces-accumulation-of-human-%C3%AE-synuclein-protein-in-two-different-parkinson-s-disease-animal-models
#22
JOURNAL ARTICLE
Aloke K Dutta, Christopher Armstrong, Dan Luo, Banibrata Das, Brian Spencer, Robert A Rissman
Aggregation of misfolded α-synuclein (α-syn) protein in the periphery and central nervous system (CNS) gives rise to a group of disorders, which are labeled collectively as synucleinopathies. These clinically distinct disorders are known as pure autonomic failure, Parkinson's disease (PD), Parkinson's disease dementia (PDD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA). In the case of PD, it has been demonstrated that toxic aggregates of α-syn protein not only cause apoptosis of dopamine neurons but its accumulation in the neocortex and limbic area principally contributes to dementia...
February 7, 2023: ACS Chemical Neuroscience
https://read.qxmd.com/read/36653527/seed-amplification-assay-for-the-detection-of-pathologic-alpha-synuclein-aggregates-in-cerebrospinal-fluid
#23
REVIEW
Luis Concha-Marambio, Sandra Pritzkow, Mohammad Shahnawaz, Carly M Farris, Claudio Soto
Misfolded alpha-synuclein (αSyn) aggregates are a hallmark event in Parkinson's disease (PD) and other synucleinopathies. Recently, αSyn seed amplification assays (αSyn-SAAs) have shown promise as a test for biochemical diagnosis of synucleinopathies. αSyn-SAAs use the intrinsic self-replicative nature of misfolded αSyn aggregates (seeds) to multiply them in vitro. In these assays, αSyn seeds circulating in biological fluids are amplified by a cyclical process that includes aggregate fragmentation into smaller self-propagating seeds, followed by elongation at the expense of recombinant αSyn (rec-αSyn)...
January 18, 2023: Nature Protocols
https://read.qxmd.com/read/36409381/imaging-in-autonomic-failure-a-window-into-real-time-physiological-mechanisms-and-other-updates-on-recent-autonomic-research
#24
EDITORIAL
Mitchell G Miglis, Nicholas Larsen, Srikanth Muppidi
No abstract text is available yet for this article.
December 2022: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
https://read.qxmd.com/read/36382590/health-related-quality-of-life-and-burden-for-caregivers-of-individuals-with-neurogenic-orthostatic-hypotension
#25
JOURNAL ARTICLE
Katy Gallop, Ngan Pham, Grant Maclaine, Emma Saunders, Bonnie Black, Lena Hubig, Sarah Acaster
Aim: This study explores the burden of caring for an individual with neurogenic orthostatic hypotension (nOH) and an underlying neurodegenerative disease (Parkinson's disease, multiple system atrophy, pure autonomic failure or dementia with Lewy bodies). Materials & methods: A survey including several validated instruments was conducted with informal caregivers of individuals with Parkinson's disease, multiple system atrophy, pure autonomic failure or dementia with Lewy bodies. Results: Caregivers of patients with nOH (n = 60) reported greater burden across all outcomes compared with those without nOH (n = 60)...
February 2023: Neurodegenerative Disease Management
https://read.qxmd.com/read/36300662/real-time-magnetic-resonance-imaging-to-study-orthostatic-intolerance-mechanisms-in-human-beings-proof-of-concept
#26
JOURNAL ARTICLE
Darius A Gerlach, Andrea Maier, Jorge Manuel, Anja Bach, Alex Hoff, Jan-Niklas Hönemann, Karsten Heusser, Dirk Voit, Jens Frahm, Jens Jordan, Jens Tank
Background Discerning the mechanisms driving orthostatic symptoms in human beings remains challenging. Therefore, we developed a novel approach combining cardiac and cerebral real-time magnetic resonance imaging, beat-to-beat physiological monitoring, and orthostatic stress testing through lower-body negative pressure (LBNP). We conducted a proof-of-concept study in a patient with severe orthostatic hypotension. Methods and Results We included a 46-year-old man with pure autonomic failure. Without and during -30 mmHg LBNP, we obtained 3T real-time magnetic resonance imaging of the cardiac short axis and quantitative flow measurements in the pulmonary trunk and middle cerebral artery...
October 27, 2022: Journal of the American Heart Association
https://read.qxmd.com/read/35936586/pure-autonomic-failure-a-localized-alpha-synucleinopathy-with-a-potential-for-conversion-to-more-extensive-alpha-synucleinopathies
#27
JOURNAL ARTICLE
Shakya Bhattacharjee, Rana Alnasser Alsukhni
Pure autonomic failure (PAF) is an alpha synucleinopathy with predominant involvement of the autonomic ganglia and peripheral nerves. The hallmark clinical feature is orthostatic hypotension. However, genitourinary, sudomotor, and cardiac involvement is also common. Many patients also develop supine hypertension. Almost a quarter of patients can phenoconvert or evolve into Parkinson's disease, multiple system atrophy, and Lewy body dementia in the future. Early severe bladder involvement, higher supine noradrenaline level, early motor involvement, and dream enactment behavior increase the risk of phenoconversion...
May 2022: Annals of Indian Academy of Neurology
https://read.qxmd.com/read/35935025/renal-transplantation-in-pure-autonomic-failure
#28
JOURNAL ARTICLE
Zain Ul Abideen, Anna Rixon, Nirosha D Gunatillake, Elizabeth Househam, Christopher J Mathias, Andrew Connor
No abstract text is available yet for this article.
September 2022: Transplantation Direct
https://read.qxmd.com/read/35908952/-evaluation-of-disopyramide-efficacy-for-refractory-syncope-in-heart-failure-with-preserved-ejection-fraction-using-holter-electrocardiography-a-case-report
#29
JOURNAL ARTICLE
Yuki Asai, Takuya Shintani, Takanori Yamamoto, Yoshiharu Sato
The management of syncope is clinically important for heart failure (HF) patients. We herein describe a case on the efficacy of disopyramide for refractory syncope in HF with preserved ejection fraction (HFpEF). An 82-year-old man was hospitalized for respiratory distress and lower limb edema and was subsequently diagnosed with HFpEF. The use of diuretics improved HF symptoms; however, on day 10 after hospitalization, a rapid decrease in blood pressure and transient loss of consciousness developed. After neurologic examination, he was diagnosed with pure autonomic failure...
2022: Yakugaku Zasshi: Journal of the Pharmaceutical Society of Japan
https://read.qxmd.com/read/35895157/recent-advances-in-establishing-fluid-biomarkers-for-the-diagnosis-and-differentiation-of-alpha-synucleinopathies-a-mini-review
#30
REVIEW
Wolfgang Singer
The clinical differentiation between multiple system atrophy (MSA), Parkinson's disease (PD), dementia with Lewy bodies (DLB), as well as the distinction between these synucleinopathies from other neurodegenerative disorders can be challenging, particularly at early disease stages or when the presentation is atypical. That is also true for predicting the fate of patients with limited or prodromal forms of synucleinopathies such as pure autonomic failure (PAF) or idiopathic REM-sleep behavior disorder (iRBD) which are known to be at risk of developing MSA, PD, or DLB...
August 2022: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
https://read.qxmd.com/read/35790426/diagnosing-premotor-multiple-system-atrophy-natural-history-and-autonomic-testing-in-an-autopsy-confirmed-cohort
#31
JOURNAL ARTICLE
Ekawat Vichayanrat, Fernanda Valerio, Shiwen Koay, Eduardo De Pablo-Fernandez, Jalesh Panicker, Huw Morris, Kailash Bhatia, Viorica Chelban, Henry Houlden, Niall Quinn, Judith Navarro-Otano, Yasuo Miki, Janice Holton, Thomas Warner, Christopher Mathias, Valeria Iodice
BACKGROUND AND OBJECTIVES: Nonmotor features precede motor symptoms in many patients with multiple system atrophy (MSA). However, little is known about differences between the natural history, progression, and prognostic factors for survival in patients with MSA with nonmotor vs motor presentations. We aimed to compare initial symptoms, disease progression, and clinical features at final evaluation and investigate differences in survival and natural history between patients with MSA with motor and nonmotor presentations...
September 13, 2022: Neurology
https://read.qxmd.com/read/35736363/-dizziness-upon-standing-consider-autonomic-dysfunction
#32
JOURNAL ARTICLE
Niels P Riksen, Joost Rutten, Bastiaan R Bloem, Jaap Deinum
Many people, particularly elderly, experience dizziness upon standing. It is important to always measure the blood pressure and heart rate in the supine and standing position in these patients. In case of orthostatic hypotension with an insufficient increase in heart rate, one has to consider autonomic dysfunction. Alpha-synucleopathies are frequent causes of autonomic dysfunction, including Parkinson's disease, Pure Autonomic Failure and Multiple System Atrophy. The quality of life can significantly be improved in patients with autonomic dysfunction due to life-style measures (increased salt and water intake, compressive stockings, head-up sleeping) and medication to increase blood pressure (fludrocortisone, midodrine)...
April 25, 2022: Nederlands Tijdschrift Voor Geneeskunde
https://read.qxmd.com/read/35702060/a-case-of-pure-autonomic-failure-initially-presenting-with-hemihypohidrosis-twelve-year-follow-up
#33
Hiroshi Saito
Although it is generally recognized that pure autonomic failure (PAF) is a progressive neurodegenerative disease selectively involving the autonomic nervous system, its mode of progression remains to be settled. A 57-year-old man presented with sweat reduction on the left side during previous 3 years. The thermoregulatory sweat test revealed left-sided multi-segmental hypohidrosis more markedly on the face. Pharmacological sweating was relatively preserved except for the face. During the subsequent 8 years, he developed erectile dysfunction and overt orthostatic hypotension...
January 2022: Case Reports in Neurology
https://read.qxmd.com/read/35693346/alpha-synuclein-strain-variability-in-body-first-and-brain-first-synucleinopathies
#34
Mie Kristine Just, Hjalte Gram, Vasileios Theologidis, Poul Henning Jensen, K Peter R Nilsson, Mikael Lindgren, Karoline Knudsen, Per Borghammer, Nathalie Van Den Berge
Pathogenic alpha-synuclein (asyn) aggregates are a defining feature of neurodegenerative synucleinopathies, which include Parkinson's disease, Lewy body dementia, pure autonomic failure and multiple system atrophy. Early accurate differentiation between these synucleinopathies is challenging due to the highly heterogeneous clinical profile at early prodromal disease stages. Therefore, diagnosis is often made in late disease stages when a patient presents with a broad range of motor and non-motor symptoms easing the differentiation...
2022: Frontiers in Aging Neuroscience
https://read.qxmd.com/read/34913328/pure-autonomic-failure-the-first-report-in-poland
#35
JOURNAL ARTICLE
Małgorzata Dec-Ćwiek, Karolina Porębska, Magdalena Witkowska, Jakub Antczak, Magdalena Bosak, Marcin Tutaj
No abstract text is available yet for this article.
March 30, 2022: Polish Archives of Internal Medicine
https://read.qxmd.com/read/34880184/-a-case-of-pure-autonomic-failure-that-eventually-developed-into-multiple-system-atrophy-5-years-later
#36
JOURNAL ARTICLE
(no author information available yet)
No abstract text is available yet for this article.
2021: Nihon Ronen Igakkai Zasshi. Japanese Journal of Geriatrics
https://read.qxmd.com/read/34879118/lio-csi-lidar-inertial-odometry-with-loop-closure-combined-with-semantic-information
#37
JOURNAL ARTICLE
Gang Wang, Saihang Gao, Han Ding, Hao Zhang, Hongmin Cai
Accurate and reliable state estimation and mapping are the foundation of most autonomous driving systems. In recent years, researchers have focused on pose estimation through geometric feature matching. However, most of the works in the literature assume a static scenario. Moreover, a registration based on a geometric feature is vulnerable to the interference of a dynamic object, resulting in a decline of accuracy. With the development of a deep semantic segmentation network, we can conveniently obtain the semantic information from the point cloud in addition to geometric information...
2021: PloS One
https://read.qxmd.com/read/34817726/the-role-of-cardiovascular-autonomic-failure-in-the-differential-diagnosis-of-%C3%AE-synucleinopathies
#38
REVIEW
Fabian Leys, Gregor K Wenning, Alessandra Fanciulli
The α-synucleinopathies comprise a group of adult-onset neurodegenerative disorders including Parkinson's disease (PD), multiple system atrophy (MSA), dementia with Lewy bodies (DLB,) and - as a restricted non-motor form - pure autonomic failure (PAF). Neuropathologically, the α-synucleinopathies are characterized by aggregates of misfolded α-synuclein in the central and peripheral nervous system. Cardiovascular autonomic failure is a common non-motor symptom in people with PD, a key diagnostic criterion in MSA, a supportive feature for the diagnosis of DLB and disease-defining in PAF...
January 2022: Neurological Sciences
https://read.qxmd.com/read/34757507/inhibition-of-the-norepinephrine-transporter-to-treat-neurogenic-orthostatic-hypotension-is-this-the-end-of-the-story
#39
EDITORIAL
Guillaume Lamotte, Patricio Millar Vernetti
No abstract text is available yet for this article.
December 2021: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
https://read.qxmd.com/read/34737065/origin-branching-pattern-foraminal-and-intraspinal-distribution-of-the-human-lumbar-sinuvertebral-nerves
#40
JOURNAL ARTICLE
Marcus C Breemer, Martijn J A Malessy, Robbert G E Notenboom
BACKGROUND CONTEXT: The lumbar sinuvertebral nerve (SVN) innervates the outer posterior intervertebral disc (IVD); it is thought to mediate discogenic low-back pain (LBP). Controversy, however, exists on its origins at higher (L1-L2) versus lower (L3-L5) lumbar levels. Additionally, lack of knowledge regarding its foraminal and intraspinal branching patterns and extensions may lead to iatrogenic damage. PURPOSE: To systematically describe the origins of the L2 and L5 SVNs, their morphological variation in the intervertebral foramen (IVF) and intraspinal distribution...
March 2022: Spine Journal: Official Journal of the North American Spine Society
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