6,549 results match your criteria Multiple System Atrophy


Infections or Sepsis Preceding Clinically Diagnosed α-Synucleinopathies: A Case-Control Study.

Mov Disord 2020 Jun 1. Epub 2020 Jun 1.

Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.

Background: Several studies have proposed a role for infections to induce an inflammatory response triggering Parkinson's disease. This remains controversial and the influence of severe infections on other α-synucleinopathies (Dementia with Lewy Bodies, Parkinson's disease dementia, and Multiple System Atrophy) has not been adequately investigated.

Objectives: To assess the association between hospitalization-required infections or sepsis and risk of clinically diagnosed α-synucleinopathies. Read More

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http://dx.doi.org/10.1002/mds.28111DOI Listing

Cerebellar Atrophy in Multiple System Atrophy (Cerebellar Type) and Its Implication for Network Connectivity.

Cerebellum 2020 May 29. Epub 2020 May 29.

Department of Radiology, Dongfang Hospital, Beijing University of Chinese Medicine, Beijing, 100078, China.

We sought to assess structural and functional patterns of cerebellum in multiple system atrophy (cerebellar type), and investigate the associations of structural and functional cerebellar gray matter abnormalities. We collected magnetic resonance imaging data of 18 patients with multiple system atrophy (cerebellar type) and 18 health control subjects. The gray matter loss across the motor and cognitive cerebellar territories in patients was assessed using voxel-based morphometry. Read More

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http://dx.doi.org/10.1007/s12311-020-01144-4DOI Listing

Propagation of Pathological α-Synuclein from the Urogenital Tract to the Brain Initiates MSA-like Syndrome.

iScience 2020 May 15;23(6):101166. Epub 2020 May 15.

Department of Neurology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan 450052, China; Institute of Parkinson and Movement Disorder, Zhengzhou University, Zhengzhou, Henan 450052, China. Electronic address:

The neuropathological feature of multiple system atrophy (MSA), a fatal adult-onset disorder without effective therapy, is the accumulation of pathological α-synuclein (α-Syn) in the central nervous system (CNS). Here we show that pathological α-Syn exists in nerve terminals in detrusor and external urethral sphincter (EUS) of patients with MSA. Furthermore, α-Syn-preformed fibrils (PFFs) injected in the EUS or detrusor in TgM83 mice initiated the transmission of pathological α-Syn from the urogenital tract to brain via micturition reflex pathways, and these mice developed widespread phosphorylated α-Syn inclusion pathology together with phenotypes. Read More

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http://dx.doi.org/10.1016/j.isci.2020.101166DOI Listing

Application of the p9NORM correction method to timed neuropsychological tests in Parkinson's disease and multiple system atrophy.

Neurol Sci 2020 May 28. Epub 2020 May 28.

Department of Neurosciences and Reproductive and Odontostomatological Sciences, University "Federico II", Via Pansini, 5, 80131, Napoli, NA, Italy.

Objective: Timed neuropsychological tests do not take into account physical impairment during scoring procedures. Dysarthria and upper limb impairment can be easily measured with the PATA rate test (PRT) and the nine-hole pegboard test (9HPT). We recently validated a normalization method for timed neuropsychological tests using the PRT and 9HPT (p9NORM). Read More

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http://dx.doi.org/10.1007/s10072-020-04478-3DOI Listing

Structures of α-synuclein filaments from multiple system atrophy.

Nature 2020 May 27. Epub 2020 May 27.

MRC Laboratory of Molecular Biology, Cambridge, UK.

Synucleinopathies, which include multiple system atrophy (MSA), Parkinson's disease, Parkinson's disease with dementia and dementia with Lewy bodies (DLB), are human neurodegenerative diseases. Existing treatments are at best symptomatic. These diseases are characterized by the presence of, and believed to be caused by the formation of, filamentous inclusions of α-synuclein in brain cells. Read More

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http://dx.doi.org/10.1038/s41586-020-2317-6DOI Listing

Generation and Regeneration of Thymic Epithelial Cells.

Front Immunol 2020 7;11:858. Epub 2020 May 7.

Institute for Immunology and Immunotherapy, University of Birmingham, Birmingham, United Kingdom.

The thymus is unique in its ability to support the maturation of phenotypically and functionally distinct T cell sub-lineages. Through its combined production of MHC-restricted conventional CD4 and CD8, and Foxp3 regulatory T cells, as well as non-conventional CD1d-restricted iNKT cells and invariant γδT cells, the thymus represents an important orchestrator of immune system development and control. It is now clear that thymus function is largely determined by the availability of stromal microenvironments. Read More

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http://dx.doi.org/10.3389/fimmu.2020.00858DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7221188PMC

Clinical and Imaging Markers of Prodromal Parkinson's Disease.

Front Neurol 2020 8;11:395. Epub 2020 May 8.

Department of Neurology, St. Olavs Hospital, Trondheim, Norway.

The diagnosis of Parkinson's disease (PD) relies on the clinical effects of dopamine deficiency, including bradykinesia, rigidity and tremor, usually manifesting asymmetrically. Misdiagnosis is common, due to overlap of symptoms with other neurodegenerative disorders such as multiple system atrophy and progressive supranuclear palsy, and only autopsy can definitively confirm the disease. Motor deficits generally appear when 50-60% of dopaminergic neurons in the substantia nigra are already lost, limiting the effectiveness of potential neuroprotective therapies. Read More

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http://dx.doi.org/10.3389/fneur.2020.00395DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7225301PMC

Cerebrovascular pathology and misdiagnosis of multiple system atrophy: An autopsy study.

Parkinsonism Relat Disord 2020 May 16;75:34-40. Epub 2020 May 16.

Department of Neuroscience, Mayo Clinic, Jacksonville, FL, 32224, USA. Electronic address:

Background: Multiple system atrophy (MSA) is a progressive neurodegenerative disease characterized by a combination of dysautonomia, parkinsonism, and cerebellar ataxia. Other disorders can mimic MSA, but it is unknown whether cerebrovascular pathology, so-called "vascular parkinsonism," can mimic MSA. This study aimed to determine the clinicopathological features and red flags for vascular parkinsonism masquerading as MSA. Read More

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http://dx.doi.org/10.1016/j.parkreldis.2020.05.018DOI Listing

Overview of sleep disturbances and their management in Parkinson plus disorders.

J Neurol Sci 2020 May 8;415:116891. Epub 2020 May 8.

Department of Neurology, National Institute of Mental Health & Neurosciences (NIMHANS), Hosur Road, Bangalore, Karnataka 560029, India. Electronic address:

Sleep disturbance is one of the commonly reported non-motor symptoms in patients with Parkinson's disease (PD) as well as in Parkinson plus disorders such as multiple system atrophy (MSA), dementia with Lewy bodies (DLB), progressive supranuclear palsy (PSP), and corticobasal syndrome (CBS). Although there is a wealth of literature on sleep disturbances in PD, the same is not robust on the Parkinson plus disorders. This article aims to comprehensively review the sleep disturbances in Parkinson plus disorders. Read More

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http://dx.doi.org/10.1016/j.jns.2020.116891DOI Listing

The Role of Mitochondria in Neurodegenerative Diseases: the Lesson from Alzheimer's Disease and Parkinson's Disease.

Mol Neurobiol 2020 May 22. Epub 2020 May 22.

Division of Neuroscience and Laboratory of Neurogenetics, National Institute on Aging, National Institute of Health, Bethesda, MD, USA.

Although the pathogenesis of neurodegenerative diseases is still widely unclear, various mechanisms have been proposed and several pieces of evidence are supportive for an important role of mitochondrial dysfunction. The present review provides a comprehensive and up-to-date overview about the role of mitochondria in the two most common neurodegenerative disorders: Alzheimer's disease (AD) and Parkinson's disease (PD). Mitochondrial involvement in AD is supported by clinical features like reduced glucose and oxygen brain metabolism and by numerous microscopic and molecular findings, including altered mitochondrial morphology, impaired respiratory chain function, and altered mitochondrial DNA. Read More

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http://dx.doi.org/10.1007/s12035-020-01926-1DOI Listing

An update on MSA: premotor and non-motor features open a window of opportunities for early diagnosis and intervention.

J Neurol 2020 May 20. Epub 2020 May 20.

Department of Neuromuscular Diseases, Queen Square Institute of Neurology, University College London, London, WC1N 3BG, UK.

In this review, we describe the wide clinical spectrum of features that can be seen in multiple system atrophy (MSA) with a focus on the premotor phase and the non-motor symptoms providing an up-to-date overview of the current understanding in this fast-growing field. First, we highlight the non-motor features at disease onset when MSA can be indistinguishable from pure autonomic failure or other chronic neurodegenerative conditions. We describe the progression of clinical features to aid the diagnosis of MSA early in the disease course. Read More

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http://dx.doi.org/10.1007/s00415-020-09881-6DOI Listing

The importance of brain banking for dementia practice: the first experience of Turkey.

Cell Tissue Bank 2020 May 20. Epub 2020 May 20.

Division of Neuropathology, Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, AR, USA.

This study reports the results of the first brain tissue banking experience of Turkey in the Unit for Aging Brain and Dementia at Dokuz Eylul University, Department of Geriatric Medicine, Izmir. Here, we have briefly described our efforts on brain banking in our country, which consist of six brains from autopsies that had at least two years of clinical follow-up in the 2015-2017 period. The evaluation led to the diagnosis of two Alzheimer's disease (AD) with cerebral amyloid angiopathy, one AD with dementia with Lewy bodies, one corticobasal degeneration, one multiple system atrophy, one vascular dementia. Read More

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http://dx.doi.org/10.1007/s10561-020-09835-2DOI Listing

Gut Microbiome-Modified Polyphenolic Compounds Inhibit α-Synuclein Seeding and Spreading in α-Synucleinopathies.

Front Neurosci 2020 4;14:398. Epub 2020 May 4.

Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY, United States.

Misfolding, aggregation and deposition of α-synuclein (α-syn) are major pathologic characteristics of Parkinson's disease (PD) and the related synucleinopathy, multiple system atrophy (MSA). The spread of α-syn pathology across brain regions is thought to play a key role in the onset and progression of clinical phenotypes. Thus, there is increasing interest in developing strategies that target and attenuate α-syn aggregation and spread. Read More

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http://dx.doi.org/10.3389/fnins.2020.00398DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7212829PMC

Reduced oligodendrocyte exosome secretion in multiple system atrophy involves SNARE dysfunction.

Brain 2020 May 18. Epub 2020 May 18.

Department of Pathology, University of Washington School of Medicine, 325 9th Ave, HMC Box 359635, Seattle, WA 98104, USA.

Transportation of key proteins via extracellular vesicles has been recently implicated in various neurodegenerative disorders, including Parkinson's disease, as a new mechanism of disease spreading and a new source of biomarkers. Extracellular vesicles likely to be derived from the brain can be isolated from peripheral blood and have been reported to contain higher levels of α-synuclein (α-syn) in Parkinson's disease patients. However, very little is known about extracellular vesicles in multiple system atrophy, a disease that, like Parkinson's disease, involves pathological α-syn aggregation, though the process is centred around oligodendrocytes in multiple system atrophy. Read More

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http://dx.doi.org/10.1093/brain/awaa110DOI Listing

Fibrillar form of α-synuclein-specific scFv antibody inhibits α-synuclein seeds induced aggregation and toxicity.

Sci Rep 2020 May 18;10(1):8137. Epub 2020 May 18.

Neurological Disorder Research Center, Qatar Biomedical Research Institute (QBRI), Hamad Bin Khalifa University (HBKU), Qatar Foundation, Doha, Qatar.

Synucleinopathies including Parkinson's disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA) are characterized by pathological accumulation of α-synuclein (α-syn). Amongst the various approaches attempting to tackle the pathological features of synucleinopathies, antibody-based immunotherapy holds much promise. However, the large size of antibodies and corresponding difficulty in crossing the blood-brain barrier has limited development in this area. Read More

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http://dx.doi.org/10.1038/s41598-020-65035-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7235225PMC

The emerging role of α-synuclein truncation in aggregation and disease.

J Biol Chem 2020 05 18. Epub 2020 May 18.

University of Florida, United States.

α-synuclein (αsyn) is an abundant, brain neuronal protein that can misfold and polymerize to form toxic fibrils coalescing into pathologic inclusions in neurodegenerative diseases including Parkinson's disease (PD), Lewy body dementia (LBD), and multiple system atrophy (MSA). These fibrils may induce further αsyn misfolding and propagation of pathologic fibrils in a prion-like process. It is unclear why αsyn initially misfolds, but a growing body of literature suggests a critical role of partial proteolytic processing resulting in various truncations of the highly charged and flexible carboxy-terminal region. Read More

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http://dx.doi.org/10.1074/jbc.REV120.011743DOI Listing

Central autonomic dysfunction in multiple system atrophy: can we measure it with MRI?

Clin Auton Res 2020 Jun 16;30(3):185-187. Epub 2020 May 16.

Department of Neurology, University of Cologne, Cologne, Germany.

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http://dx.doi.org/10.1007/s10286-020-00695-0DOI Listing

Mesial Frontal Lobe Infarction Presenting as Pisa Syndrome.

J Stroke Cerebrovasc Dis 2020 May 13:104882. Epub 2020 May 13.

Department of Neurology, Juntendo University Shizuoka Hospital, 1129 Nagaoka, Izunokuni, Shizuoka 410-2295, Japan.

Pisa syndrome is usually seen in patients with Alzheimer's disease treated with a cholinesterase inhibitor, dementia with Lewy bodies, Parkinson's disease, or atypical parkinsonism including multiple system atrophy. An 86-year-old woman presented with an acute onset of lateral flexion of her trunk to the left side, i.e. Read More

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http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2020.104882DOI Listing

Which Autonomic Features Distinguish Multiple System Atrophy and When.

Mov Disord 2020 May;35(5):902-903

Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria.

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http://dx.doi.org/10.1002/mds.28050DOI Listing
May 2020
5.680 Impact Factor

Does Urinary Retention Discriminate Multiple System Atrophy From Parkinson's Disease?

Mov Disord 2020 May;35(5):901-902

Department of Neurology and Movement Disorder Center, College of Medicine, Seoul National University, Seoul, Korea.

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http://dx.doi.org/10.1002/mds.28049DOI Listing

Cardiovascular autonomic function testing in multiple system atrophy and Parkinson's disease: an expert-based blinded evaluation.

Clin Auton Res 2020 Jun 15;30(3):255-263. Epub 2020 May 15.

Division of Clinical Neurobiology, Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria.

Purpose: Multiple system atrophy (MSA) and Parkinson's disease (PD) are sporadic neurodegenerative diseases characterized by an accumulation of misfolded α-synuclein. Cardiovascular autonomic failure develops in both MSA and PD, although studies indicate different sites of autonomic nervous system lesion. However, it is unclear whether this could potentially aid the differential diagnosis of these diseases. Read More

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http://dx.doi.org/10.1007/s10286-020-00691-4DOI Listing
June 2020
1.864 Impact Factor

Colorectal Cancer Invasion and Atrophy of the Enteric Nervous System: Potential Feedback and Impact on Cancer Progression.

Int J Mol Sci 2020 May 11;21(9). Epub 2020 May 11.

Department of Histology, Medical University of Gdansk, 80-210 Gdansk, Poland.

Colorectal cancer (CRC) invasion within the large intestine wall results in the replacement of normal tissue architecture by tumour mass. Cancer cells digest the extracellular matrix (ECM) by the release of proteolytic enzymes. The disintegration of matrix ground substance activates several deposited growth factors which stimulate cell proliferation. Read More

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http://dx.doi.org/10.3390/ijms21093391DOI Listing

DCTN1 mutation analysis in Italian patients with PSP, MSA, and DLB.

Neurobiol Aging 2020 Apr 15. Epub 2020 Apr 15.

Institute of Molecular Bioimaging and Physiology, National Research Council, Section of Germaneto, Catanzaro, Italy.

DCTN1 encodes the largest subunit of dynactin complex essential in the retrograde axonal transport and cytoplasmic transport of vesicles; mutations in DCTN1 have been reported predominantly in individuals with Perry syndrome and, recently, in patients with progressive supranuclear palsy. Our genetic screening of DCTN1 in 79 patients with progressive supranuclear palsy, 100 patients with multiple system atrophy, and 28 patients with dementia with Lewy bodies from Italy revealed only synonymous and intronic variants, suggesting that DCTN1 mutations do not have a key role in the development of atypical parkinsonism in the Italian population. Read More

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http://dx.doi.org/10.1016/j.neurobiolaging.2020.04.006DOI Listing

Asymmetry index of Blink Reflex Recovery Cycle differentiates Parkinson's disease from atypical Parkinsonian syndromes.

J Neurol 2020 May 11. Epub 2020 May 11.

Department of Medical, Surgical Sciences and Advanced Technologies GF Ingrassia, University of Catania, Via Santa Sofia 78, 95123, Catania, Italy.

Background: Differential diagnosis between Parkinson's disease (PD) and atypical Parkinsonian syndromes (APS), such as multiple system atrophy (MSA) and progressive supranuclear palsy (PSP), is often difficult because of overlap of common clinical features. We evaluated R2 Blink Reflex Recovery Cycle (R2BRRC) in drug-naive PD patients and in MSA and PSP patients to differentiate early PD from APS.

Methods: We investigated 43 patients: 15 drug-naive PD patients, 16 MSA patients, and 12 PSP patients. Read More

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http://dx.doi.org/10.1007/s00415-020-09900-6DOI Listing

[12th Post-ECTRIMS Meeting: review of the novelties from the 2019 ECTRIMS Congress (I)].

Rev Neurol 2020 May;70(10):379-390

Hospital de Cruces, Baracaldo, España.

Introduction: Like every year, after the ECTRIMS Congress, renowned Spanish neurologists who are experts in multiple sclerosis presented the main novelties in research in this field at the Post-ECTRIMS Meeting.

Aim: To summarise the content presented at the 12th edition of the Post-ECTRIMS Meeting, which took place in September 2019 in Sevilla and is presented in two parts.

Development: This first part addresses the latest studies on vitamin D deficiency and the discrepancies that currently exist regarding its treatment. Read More

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http://dx.doi.org/10.33588/rn.7010.2020121DOI Listing

Hippocampal α-synuclein pathology correlates with memory impairment in multiple system atrophy.

Brain 2020 May 8. Epub 2020 May 8.

Queen Square Brain Bank for Neurological Disorders, UCL Queen Square Institute of Neurology, 1 Wakefield Street, London WC1N 1PJ, UK.

Recent post-mortem studies reported 22-37% of patients with multiple system atrophy can develop cognitive impairment. With the aim of identifying associations between cognitive impairment including memory impairment and α-synuclein pathology, 148 consecutive patients with pathologically proven multiple system atrophy were reviewed. Among them, 118 (79. Read More

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http://dx.doi.org/10.1093/brain/awaa126DOI Listing

Lower urinary tract dysfunction in common neurological diseases.

Turk J Urol 2020 Apr 30. Epub 2020 Apr 30.

Department of Urology/General Surgery, Areteion Hospital, Athens, Greece.

The lower urinary tract has the main function of urine storage and voiding. The integrity of the lower urinary tract nerve supply is necessary for its proper function. Neurological disorders can lead to lower urinary tract dysfunction (LUTD) and cause lower urinary tract symptoms (LUTS). Read More

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http://dx.doi.org/10.5152/tud.2020.20092DOI Listing

Swallow tail sign on susceptibility map-weighted imaging (SMWI) for disease diagnosing and severity evaluating in parkinsonism.

Acta Radiol 2020 May 7:284185120920793. Epub 2020 May 7.

Department of Radiology, Huashan Hospital, Fudan University, Shanghai, PR China.

Background: Loss of swallow tail sign (STS) on iron-sensitive magnetic resonance imaging (MRI) has been recognized as an imaging feature in parkinsonism (PS).

Purpose: To investigate the diagnostic and differential diagnostic value of STS scale on susceptibility map-weighted imaging (SMWI) in PS, including Parkinson's disease (PD), progressive supranuclear palsy syndrome (PSP), and multiple system atrophy (MSA), and to evaluate its correlation with disease severity.

Material And Methods: Ninety-nine patients (55 PD, 29 PSP, and 15 MSA) and 47 healthy controls (HC) were prospectively recruited and scanned using quantitative susceptibility mapping (QSM). Read More

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http://dx.doi.org/10.1177/0284185120920793DOI Listing

Is There a Difference in Autonomic Dysfunction Between Multiple System Atrophy Subtypes?

Mov Disord Clin Pract 2020 May 9;7(4):405-412. Epub 2020 Apr 9.

Department of Neurology All India Institute of Medical Sciences New Delhi India.

Background: Autonomic dysfunction forms the diagnostic cornerstone in MSA. Data are limited on autonomic dysfunction differences between the two subtypes, MSA-C and MSA-P.

Objectives: To assess autonomic dysfunction in MSA subtypes and Parkinson's disease (PD) and compare it to healthy controls. Read More

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http://dx.doi.org/10.1002/mdc3.12936DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7197320PMC

Late onset multiple sclerosis - multiparametric MRI characteristics.

Neurol Neurochir Pol 2020 May 5. Epub 2020 May 5.

Department of Neurology, Medical University of Lodz, Lodz, Poland.

Introduction: Multiple sclerosis (MS) is a demyelinating disease of the central nervous system (CNS) with heterogenic character. Typical age of onset is between 20 and 35 years. Clinically definite multiple sclerosis (CDMS) can occur also in patients older than 50 years. Read More

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http://dx.doi.org/10.5603/PJNNS.a2020.0036DOI Listing

Neuropathological findings in multiple system atrophy with cognitive impairment.

Authors:
Kurt A Jellinger

J Neural Transm (Vienna) 2020 May 4. Epub 2020 May 4.

Institute of Clinical Neurobiology, Alberichgasse 5/13, 1150, Vienna, Austria.

Cognitive impairment (CI), previously considered an exclusion criterium for the diagnosis of multiple system atrophy (MSA) according to the second consensus criteria, is not uncommon in MSA. Mild cognitive impairment (MCI) has been reported in up to 47% of MSA patients, while severe dementia is rare. We related clinical CI with neuropathological findings in 48 autopsy-proven cases of MSA. Read More

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http://dx.doi.org/10.1007/s00702-020-02201-2DOI Listing

Sleep-Induced Glottis Closure in Multiple System Atrophy Evaluated by Four-Dimensional Computed Tomography.

Front Med (Lausanne) 2020 17;7:132. Epub 2020 Apr 17.

Department of Otolaryngology, The University of Tokyo, Tokyo, Japan.

Multiple system atrophy (MSA) is a progressive neurodegenerative disorder. Since patients with MSA often have sleep-related respiratory disorders including upper-airway obstruction and/or central sleep disturbance, appropriate evaluation of the upper airway especially during sleep may be indispensable. Fiberoptic laryngoscopy during diazepam-induced sleep has been reported for upper-airway obstruction verification. Read More

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http://dx.doi.org/10.3389/fmed.2020.00132DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7180743PMC

A novel neurodegenerative spectrum disorder in patients with MLKL deficiency.

Cell Death Dis 2020 May 1;11(5):303. Epub 2020 May 1.

Oxford Centre for Neuroinflammation, Nuffield Department of Clinical Neurosciences, Division of Clinical Neurology, John Radcliffe Hospital, University of Oxford, Oxford, OX3 9DS, UK.

Mixed lineage kinase domain-like (MLKL) is the main executor of necroptosis, an inflammatory form of programmed cell death. Necroptosis is implicated in combating infections, but also in contributing to numerous other clinical conditions, including cardiovascular diseases and neurodegenerative disorders. Inhibition of necroptosis is therefore of therapeutic interest. Read More

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http://dx.doi.org/10.1038/s41419-020-2494-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7195448PMC

Shared Metabolic Profile of Caffeine in Parkinsonian Disorders.

Mov Disord 2020 May 1. Epub 2020 May 1.

Department of Neurology, Juntendo University School of Medicine, Tokyo, Japan.

Objective: The objective of this study was to determine comprehensive metabolic changes of caffeine in the serum of patients with parkinsonian disorders including Parkinson's disease (PD), progressive supranuclear palsy (PSP), and multiple system atrophy (MSA) and to compare this with healthy control serum.

Methods: Serum levels of caffeine and its 11 downstream metabolites from independent double cohorts consisting of PD (n = 111, 160), PSP (n = 30, 19), MSA (n = 23, 17), and healthy controls (n = 43, 31) were examined by liquid chromatography-mass spectrometry. The association of each metabolite with clinical parameters and medication was investigated. Read More

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http://dx.doi.org/10.1002/mds.28068DOI Listing

Magnetic Resonance Imaging and Neurofilament Light in the Differentiation of Parkinsonism.

Mov Disord 2020 May 1. Epub 2020 May 1.

Laboratory for Rehabilitation Neuroscience, Department of Applied Physiology and Kinesiology, University of Florida, Gainesville, Florida, USA.

Objective: Accurate diagnosis is particularly challenging in Parkinson's disease (PD), multiple system atrophy (MSAp), and progressive supranuclear palsy (PSP). We compare the utility of 3 promising biomarkers to differentiate disease state and explain disease severity in parkinsonism: the Automated Imaging Differentiation in Parkinsonism (AID-P), the Magnetic Resonance Parkinsonism Index (MRPI), and plasma-based neurofilament light chain protein (NfL).

Methods: For each biomarker, the area under the curve (AUC) of receiver operating characteristic curves were quantified for PD versus MSAp/PSP and MSAp versus PSP and statistically compared. Read More

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http://dx.doi.org/10.1002/mds.28060DOI Listing
May 2020
5.680 Impact Factor

The structural differences between patient-derived α-synuclein strains dictate characteristics of Parkinson's disease, multiple system atrophy and dementia with Lewy bodies.

Acta Neuropathol 2020 Jun 30;139(6):977-1000. Epub 2020 Apr 30.

Laboratory for Neurobiology and Gene Therapy, Department of Neurosciences, KU Leuven, Leuven, Belgium.

Synucleinopathies, such as Parkinson's disease (PD), multiple system atrophy (MSA), and dementia with Lewy bodies (DLB), are defined by the presence of α-synuclein (αSYN) aggregates throughout the nervous system but diverge from one another with regard to their clinical and pathological phenotype. The recent generation of pure fibrillar αSYN polymorphs with noticeable differences in structural and phenotypic traits has led to the hypothesis that different αSYN strains may be in part responsible for the heterogeneous nature of synucleinopathies. To further characterize distinct αSYN strains in the human brain, and establish a structure-pathology relationship, we pursued a detailed comparison of αSYN assemblies derived from well-stratified patients with distinct synucleinopathies. Read More

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http://dx.doi.org/10.1007/s00401-020-02157-3DOI Listing

Various Motor and Non-Motor Symptoms in Early Multiple System Atrophy.

Neurodegener Dis 2020 Apr 29:1-6. Epub 2020 Apr 29.

Department of Neurology, Seoul National University Hospital, College of Medicine, Seoul National University, Seoul, Republic of Korea.

Background: Multiple system atrophy (MSA) patients pre-sent a variety of symptoms other than autonomic dysfunctions, parkinsonism, and cerebellar ataxia. The aim of this study was to evaluate the frequency of various motor and non-motor symptoms including so-called "red flags" in patients with early MSA and to determine whether the frequency of these symptoms was different between the parkinsonian (MSA-P) and cerebellar (MSA-C) subtypes.

Methods: Sixty-one probable or possible MSA patients with disease duration of 3 years or less were included. Read More

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http://dx.doi.org/10.1159/000507292DOI Listing

Ultrasensitive RT-QuIC assay with high sensitivity and specificity for Lewy body-associated synucleinopathies.

Acta Neuropathol 2020 Apr 27. Epub 2020 Apr 27.

IRCCS, Istituto Delle Scienze Neurologiche di Bologna, Bologna, Italy.

The clinical diagnosis of synucleinopathies, including Parkinson's disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA), is challenging, especially at an early disease stage, due to the heterogeneous and often non-specific clinical manifestations. The discovery of reliable specific markers for synucleinopathies would consequently be of great aid to the diagnosis and management of these disorders. Real-Time Quaking-Induced Conversion (RT-QuIC) is an ultrasensitive technique that has been previously used to detect self-templating amyloidogenic proteins in the cerebrospinal fluid (CSF) and other biospecimens in prion disease and synucleinopathies. Read More

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http://dx.doi.org/10.1007/s00401-020-02160-8DOI Listing

Alpha-synuclein Levels in the Differential Diagnosis of Lewy Bodies Dementia and Other Neurodegenerative Disorders: A Meta-analysis.

Alzheimer Dis Assoc Disord 2020 Apr 24. Epub 2020 Apr 24.

Third Department of Neurology, Aristotle University of Thessaloniki, Thessaloniki, Greece.

Subjectives: Lewy body dementia (LBD) is the second most common type of neurodegenerative dementia after Alzheimer disease (AD). It is characterized by the accumulation of Lewy bodies and Lewy neurites which are composed of aggregated phosphorylated alpha-synuclein, which is a presynaptic neuronal protein genetically and neuropathologically linked to Parkinson disease and to LBD. Alpha-synuclein is thought to contribute to LBD pathogenesis and to linked to disruption of cellular homeostasis and neuronal death, through effects on various intracellular targets, including synaptic function. Read More

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http://dx.doi.org/10.1097/WAD.0000000000000381DOI Listing

Vertical pons hyperintensity and hot cross bun sign in cerebellar-type multiple system atrophy and spinocerebellar ataxia type 3.

BMC Neurol 2020 Apr 27;20(1):157. Epub 2020 Apr 27.

Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan.

Background: The "hot cross bun" (HCB) sign, a cruciform hyperintensity in the pons on magnetic resonance imaging (MRI), has gradually been identified as a typical finding in multiple system atrophy, cerebellar-type (MSA-C). Few reports have evaluated the sensitivity of an HCB, including a cruciform hyperintensity and vertical line in the pons, which precedes a cruciform hyperintensity, in the early stages of MSA-C. Moreover, the difference in frequency and timing of appearance of an HCB between MSA-C and spinocerebellar ataxia type 3 (SCA3) has not been fully investigated. Read More

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http://dx.doi.org/10.1186/s12883-020-01738-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7184719PMC

Differential diagnosis of parkinsonian syndromes: a comparison of clinical and automated - metabolic brain patterns' based approach.

Eur J Nucl Med Mol Imaging 2020 Apr 27. Epub 2020 Apr 27.

Department of Neurology, UMC Ljubljana, Zaloška cesta 2, 1000, Ljubljana, Slovenia.

Purpose: Differentiation among parkinsonian syndromes may be clinically challenging, especially at early disease stages. In this study, we used F-FDG-PET brain imaging combined with an automated image classification algorithm to classify parkinsonian patients as Parkinson's disease (PD) or as an atypical parkinsonian syndrome (APS) at the time when the clinical diagnosis was still uncertain. In addition to validating the algorithm, we assessed its utility in a "real-life" clinical setting. Read More

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http://dx.doi.org/10.1007/s00259-020-04785-zDOI Listing

RFC1 Intronic Repeat Expansions Absent in Pathologically Confirmed Multiple Systems Atrophy.

Mov Disord 2020 Apr 24. Epub 2020 Apr 24.

Department of Neuromuscular Disease, Queen's Square Institute of Neurology, London, United Kingdom.

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http://dx.doi.org/10.1002/mds.28074DOI Listing

Possible "Premotor" Multiple System Atrophy-Cerebellar Form.

Eur Neurol 2020 22;83(1):80-86. Epub 2020 Apr 22.

Department of Uro-Neurology, The National Hospital for Neurology and Neurosurgery, Queen Square, London, United Kingdom.

We report the case of a 52-year-old Japanese man who, while he had no cerebellar ataxia or parkinsonism, was revealed to have silent cerebellar hypoperfusion/mild cerebellar atrophy and sacral autonomic disorder. His sacral autonomic disorder was urinary retention without marked prostate hyperplasia. Urodynamics-sphincter electromyography revealed detrusor hyperactivity with impaired contraction and neurogenic changes of the sphincter motor unit potentials. Read More

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http://dx.doi.org/10.1159/000506983DOI Listing

Lower Vitamin B12 Level at Multiple System Atrophy Diagnosis Is Associated With Shorter Survival.

Mov Disord 2020 Apr 22. Epub 2020 Apr 22.

Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.

Background: Multiple system atrophy (MSA) is a neurodegenerative disorder from α-synuclein aggregation. in vitro studies suggest vitamin B12 may interrupt α-synuclein-mediated neurodegeneration. The objective of this study was to determine whether serum vitamin B12 level at MSA diagnosis is associated with survival. Read More

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http://dx.doi.org/10.1002/mds.28070DOI Listing

Newly Diagnosed Amyotrophic Lateral Sclerosis in a Patient with Multiple-System Atrophy.

J Clin Neurol 2020 Apr;16(2):324-326

Department of Neurology, Keimyung University School of Medicine, Daegu, Korea.

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http://dx.doi.org/10.3988/jcn.2020.16.2.324DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7174119PMC

Early Impairment of Chopsticks Skills in Parkinsonism Suggests Progressive Supranuclear Palsy.

J Clin Neurol 2020 Apr;16(2):254-260

Department of Neurology, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, Korea.

Background And Purpose: Chopsticks are a primary eating utensil in East Asia, but systematic assessments of chopsticks skills in parkinsonian disorders is lacking. We aimed to identify any differences in chopsticks skills in the early stages of Parkinson's disease (PD) and atypical parkinsonism (AP), including progressive supranuclear palsy (PSP), multiple system atrophy (MSA), and corticobasal syndrome (CBS).

Methods: We consecutively recruited 111 patients with PD and 74 with AP (40 with PSP, 30 with MSA, and 4 with CBS) who were in a drug-naïve state. Read More

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http://dx.doi.org/10.3988/jcn.2020.16.2.254DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7174121PMC

Analysis of Protein Conformational Strains-A Key for New Diagnostic Methods of Human Diseases.

Authors:
Andrei Surguchov

Int J Mol Sci 2020 Apr 17;21(8). Epub 2020 Apr 17.

Department of Neurology, University of Kansas Medical Center, Kansas City, KS 66160, USA.

α-Synuclein is a naturally unfolded protein which easily aggregates and forms toxic inclusions and deposits. It is associated with several neurodegenerative diseases, including Parkinson's disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA). These diseases, called synucleinopathies, have overlapping symptoms but require different methods of treatment. Read More

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http://dx.doi.org/10.3390/ijms21082801DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7215537PMC

Current Symptomatic and Disease-Modifying Treatments in Multiple System Atrophy.

Int J Mol Sci 2020 Apr 16;21(8). Epub 2020 Apr 16.

Department of Molecular Neurology, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-Nürnberg, 91054 Erlangen, Germany.

Multiple system atrophy (MSA) is a rare, severe, and rapidly progressive neurodegenerative disorder categorized as an atypical parkinsonian syndrome. With a mean life expectancy of 6-9 years after diagnosis, MSA is clinically characterized by parkinsonism, cerebellar ataxia, autonomic failure, and poor l-Dopa responsiveness. Aside from limited symptomatic treatment, there is currently no disease-modifying therapy available. Read More

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http://dx.doi.org/10.3390/ijms21082775DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7215736PMC

Isolated stridor without any other sleeping breathing disorder diagnosed using drug-induced sleep endoscopy in a patient with multiple system atrophy: A case report.

Medicine (Baltimore) 2020 Apr;99(16):e19745

Department of Physical Medicine and Rehabilitation, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Republic of Korea.

Rationale: Multiple system atrophy (MSA) is a rare neurodegenerative disease characterized by Parkinsonism and autonomic dysfunction or cerebellar ataxia. MSA can be accompanied by stridor caused by laryngeal stenosis secondary to vocal cord dysfunction.

Patient Concern: A 60-year-old woman with MSA, complaining of difficulty in breathing during sleep. Read More

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http://dx.doi.org/10.1097/MD.0000000000019745DOI Listing