6,975 results match your criteria Multiple System Atrophy


Multisegmental versus monosegmental intramedullary spinal cord ependymomas: perioperative neurological functions and surgical outcomes.

Neurosurg Rev 2021 May 14. Epub 2021 May 14.

Department of Neurosurgery, Peking University Third Hospital, Peking University, Beijing, China.

Multiple factors, such as tumor size, lateralization, tumor location, accompanying syringomyelia, and regional spinal cord atrophy, may affect the resectability and clinical prognosis of intramedullary spinal cord ependymomas. However, whether long-segmental involvement of the spinal cord may impair functional outcomes remains unclear. This study was aimed to compare perioperative neurological functions and long-term surgical outcomes between multisegmental ependymomas and their monosegmental counterparts. Read More

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The Isolated Form of Rapid Eye Movement Sleep Behavior Disorder: The Upcoming Challenges.

Sleep Med Clin 2021 Jun 15;16(2):335-348. Epub 2021 Apr 15.

Instituto de Investigaciones del Sueño, Calle Padre Damián, 44, Madrid 28036, Spain; Hospital Universitario Puerta de Hierro, Majadahonda, Spain.

The diagnosis of rapid eye movement (REM) sleep behavior disorder (SBD) requires videopolysomnography detection of excessive electromyographic activity during REM sleep, which is time consuming and difficult. An easier, faster, reliable, and reproducible methodology is needed for its diagnosis. The isolated form of RBD represents an early manifestation of the synucleinopathies Parkinson disease and dementia with Lewy bodies. Read More

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Cardiac F-Dopamine PET Distinguishes PD with Orthostatic Hypotension from Parkinsonian MSA.

Mov Disord Clin Pract 2021 May 23;8(4):582-586. Epub 2021 Mar 23.

Autonomic Medicine Section, National Institute of Neurological Disorders and Stroke (NINDS) National Institutes of Health (NIH) Bethesda Maryland USA.

Background: Parkinson's disease with orthostatic hypotension (PD + OH) can be difficult to distinguish clinically from the parkinsonian form of multiple system atrophy (MSA-P). Previous studies examined cardiac sympathetic neuroimaging to differentiate PD from MSA but without focusing specifically on PD + OH versus MSA-P, which often is the relevant differential diagnostic issue.

Objective: To investigate the utility of cardiac sympathetic neuroimaging by F-dopamine positron emission tomographic (PET) scanning for separating PD + OH from MSA-P. Read More

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Multiple system atrophy-associated oligodendroglial protein p25α stimulates formation of novel α-synuclein strain with enhanced neurodegenerative potential.

Acta Neuropathol 2021 May 12. Epub 2021 May 12.

DANDRITE, Danish Research Institute of Translational Neuroscience & Department of Biomedicine, Aarhus University, 8000, Aarhus C, Denmark.

Pathology consisting of intracellular aggregates of alpha-Synuclein (α-Syn) spread through the nervous system in a variety of neurodegenerative disorders including Parkinson's disease, dementia with Lewy bodies, and multiple system atrophy. The discovery of structurally distinct α-Syn polymorphs, so-called strains, supports a hypothesis where strain-specific structures are templated into aggregates formed by native α-Syn. These distinct strains are hypothesised to dictate the spreading of pathology in the tissue and the cellular impact of the aggregates, thereby contributing to the variety of clinical phenotypes. Read More

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Motor Dysfunction in REM Sleep Behavior Disorder: A Rehabilitation Framework for Prodromal Synucleinopathy.

Neurorehabil Neural Repair 2021 May 12:15459683211011238. Epub 2021 May 12.

University of Minnesota, Minneapolis, MN, USA.

Parkinson disease (PD) and other related diseases with α-synuclein pathology are associated with a long prodromal or preclinical stage of disease. Predictive models based on diagnosis of idiopathic rapid eye movement (REM) sleep behavior disorder (iRBD) make it possible to identify people in the prodromal stage of synucleinopathy who have a high probability of future disease and provide an opportunity to implement neuroprotective therapies. However, rehabilitation providers may be unaware of iRBD and the motor abnormalities that indicate early motor system dysfunction related to α-synuclein pathology. Read More

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Identifying miRNAs in multiple sclerosis gray matter lesions that correlate with atrophy measures.

Ann Clin Transl Neurol 2021 May 12. Epub 2021 May 12.

Department of Neurosciences, Cleveland Clinic, Cleveland, Ohio, USA.

Objective: Multiple sclerosis (MS) is an inflammatory, demyelinating and neurodegenerative disease of the central nervous system (CNS). Though MS was initially considered to be a white matter demyelinating disease, myelin loss in cortical gray matter has been reported in all disease stages. We previously identified microRNAs (miRNAs) in white matter lesions (WMLs) that are detected in serum from MS patients. Read More

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Norepinephrine reuptake blockade to treat neurogenic orthostatic hypotension.

Clin Auton Res 2021 May 11. Epub 2021 May 11.

Autonomic Medicine Section, Clinical Neurosciences Program, Division of Intramural Research, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA.

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The Cryo-EM Effect: Structural Biology of Neurodegenerative Disease Aggregates.

J Neuropathol Exp Neurol 2021 May 10. Epub 2021 May 10.

Neurogenerative diseases are characterized by diverse protein aggregates with a variety of microscopic morphologic features. Although ultrastructural studies of human neurodegenerative disease tissues have been conducted since the 1960s, only recently have near-atomic resolution structures of neurodegenerative disease aggregates been described. Solid-state nuclear magnetic resonance spectroscopy and X-ray crystallography have provided near-atomic resolution information about in vitro aggregates but pose logistical challenges to resolving the structure of aggregates derived from human tissues. Read More

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Co-registration Analysis of Fluorodopa and Fluorodeoxyglucose Positron Emission Tomography for Differentiating Multiple System Atrophy Parkinsonism Type From Parkinson's Disease.

Front Aging Neurosci 2021 20;13:648531. Epub 2021 Apr 20.

Department of Neurology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China.

It is difficult to differentiate between Parkinson's disease and multiple system atrophy parkinsonian subtype (MSA-P) because of the overlap of their signs and symptoms. Enormous efforts have been made to develop positron emission tomography (PET) imaging to differentiate these diseases. This study aimed to investigate the co-registration analysis of F-fluorodopa and F-flurodeoxyglucose PET images to visualize the difference between Parkinson's disease and MSA-P. Read More

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Potential Fluid Biomarkers and a Prediction Model for Better Recognition Between Multiple System Atrophy-Cerebellar Type and Spinocerebellar Ataxia.

Front Aging Neurosci 2021 20;13:644699. Epub 2021 Apr 20.

Department of Laboratory Medicine, West China Hospital of Sichuan University, Chengdu, China.

Objective: This study screened potential fluid biomarkers and developed a prediction model based on the easily obtained information at initial inspection to identify ataxia patients more likely to have multiple system atrophy-cerebellar type (MSA-C).

Methods: We established a retrospective cohort with 125 ataxia patients from southwest China between April 2018 and June 2020. Demographic and laboratory variables obtained at the time of hospital admission were screened using Least Absolute Shrinkage and Selection Operator (LASSO) regression and logistic regression to construct a diagnosis score. Read More

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Optic disc morphology and peripapillary atrophic changes in diabetic children and adults without diabetic retinopathy or visual impairment.

Acta Ophthalmol 2021 May 5. Epub 2021 May 5.

Shanghai Engineering Center for Precise Diagnosis and Treatment of Eye Diseases, National Clinical Research Center for Eye Diseases, Shanghai Key laboratory of Ocular Fundus Diseases, Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.

Purpose: To investigate the changes in optic disc morphology and peripapillary atrophy (PPA) in diabetic children and adults without diabetic retinopathy (DR) or visual impairment (VI).

Methods: This cross-sectional study included two groups of subjects. One group included 91 children with type 1 diabetes mellitus (T1DM) and 86 healthy children, and the other group included 444 adults with T2DM and 442 healthy controls. Read More

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Radiomics on routine T1-weighted MRI can delineate Parkinson's disease from multiple system atrophy and progressive supranuclear palsy.

Eur Radiol 2021 May 4. Epub 2021 May 4.

Symbiosis Center for Medical Image Analysis and Symbiosis Institute of Technology, Symbiosis International University, Lavale, Mulshi, Pune, Maharashtra, 412115, India.

Objectives: This study aimed to explore the feasibility of radiomics features extracted from T1-weighted MRI images to differentiate Parkinson's disease (PD) from atypical parkinsonian syndromes (APS).

Methods: Radiomics features were computed from T1 images of 65 patients with PD, 61 patients with APS (31: progressive supranuclear palsy and 30: multiple system atrophy), and 75 healthy controls (HC). These features were extracted from 19 regions of interest primarily from subcortical structures, cerebellum, and brainstem. Read More

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Robust α-synuclein pathology in select brainstem neuronal populations is a potential instigator of multiple system atrophy.

Acta Neuropathol Commun 2021 May 3;9(1):80. Epub 2021 May 3.

Department of Neuroscience, College of Medicine, University of Florida, BMS J483/CTRND, 1275 Center Drive, Gainesville, FL, 32610, USA.

Multiple system atrophy (MSA) is an insidious middle age-onset neurodegenerative disease that clinically presents with variable degrees of parkinsonism and cerebellar ataxia. The pathological hallmark of MSA is the progressive accumulation of glial cytoplasmic inclusions (GCIs) in oligodendrocytes that are comprised of α-synuclein (αSyn) aberrantly polymerized into fibrils. Experimentally, MSA brain samples display a high level of seeding activity to induce further αSyn aggregation by a prion-like conformational mechanism. Read More

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Plasma Short-Chain Fatty Acids Differences in Multiple System Atrophy from Parkinson's Disease.

J Parkinsons Dis 2021 Apr 30. Epub 2021 Apr 30.

Department of Neurology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, P.R. China.

Background: Multiple system atrophy (MSA) and Parkinson's disease (PD) have overlapping symptoms, making diagnosis challenging. Short-chain fatty acids (SCFAs) are produced exclusively by gut microbiota and were reduced in feces of MSA patients. However, plasma SCFA concentrations in MSA patients have not been investigated. Read More

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Phytochemicals as Regulators of Genes Involved in Synucleinopathies.

Biomolecules 2021 Apr 22;11(5). Epub 2021 Apr 22.

Section of Otolaryngology, Department of Surgery, Yale School of Medicine, Yale University, New Haven, CT 06520, USA.

Synucleinopathies are a group of neurodegenerative diseases characterized by the accumulation of α-synuclein aggregates in neurons, nerve fibers or glial cells. Three main types of diseases belong to the synucleinopathies: Parkinson's disease, dementia with Lewy bodies, and multiple system atrophy. All of them develop as a result of an interplay of genetic and environmental factors. Read More

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Placenta-Expanded Stromal Cell Therapy in a Rodent Model of Simulated Weightlessness.

Cells 2021 Apr 19;10(4). Epub 2021 Apr 19.

Space Biosciences Division, NASA Ames Research Center, Moffett Field, CA 94035, USA.

Long duration spaceflight poses potential health risks to astronauts during flight and re-adaptation after return to Earth. There is an emerging need for NASA to provide successful and reliable therapeutics for long duration missions when capability for medical intervention will be limited. Clinically relevant, human placenta-derived therapeutic stromal cells (PLX-PAD) are a promising therapeutic alternative. Read More

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Camptocormia in patients with multiple system atrophy at different disease durations: frequency and related factors.

BMC Neurol 2021 Apr 28;21(1):181. Epub 2021 Apr 28.

Department of Neurology, Laboratory of Neurodegenerative Disorders, Rare Diseases Center, West China Hospital, Sichuan University, Chengdu, 610041, Sichuan, China.

Background: Camptocormia is common in patients with multiple system atrophy (MSA). The current study was aimed at assessing the frequency of camptocormia and its related factors in MSA patients with different disease durations. Also, the impact of camptocormia on disability was evaluated. Read More

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Intraocular pressure and choroidal thickness postural changes in multiple system atrophy and Parkinson's disease.

Sci Rep 2021 Apr 26;11(1):8936. Epub 2021 Apr 26.

Department of Medicine, Surgery, and Dentistry "Scuola Medica Salernitana", University of Salerno, via S. Allende - 84081 - Baronissi, Salerno, Italy.

To evaluate intraocular pressure (IOP) and choroidal thickness (ChT) postural changes in multiple system atrophy (MSA), Parkinson's disease (PD) patients and healthy controls (HC). 20 MSA patients, 21 PD patients and 14 HC, were examined. All subjects underwent a complete examination, including corneal thickness, ChT, IOP and axial length (AL) measurements. Read More

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Homozygous SCN1B variants causing early infantile epileptic encephalopathy 52 affect voltage-gated sodium channel function.

Epilepsia 2021 Apr 26. Epub 2021 Apr 26.

Faculty of Medicine and Health Sciences, Department of Basic and Applied Medical Sciences, Ghent University, Ghent, Belgium.

We identified nine patients from four unrelated families harboring three biallelic variants in SCN1B (NM_001037.5: c.136C>T; p. Read More

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Differential abnormalities of cerebrospinal fluid dopaminergic vs. noradrenergic indices in synucleinopathies.

J Neurochem 2021 Apr 24. Epub 2021 Apr 24.

Autonomic Medicine Section, Clinical Neurosciences Program, Division of Intramural Research, National Institute of Neurological, Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA.

The synucleinopathies Parkinson disease (PD), multiple system atrophy (MSA), and pure autonomic failure (PAF) are characterized by intra-cytoplasmic deposition of the protein alpha-synuclein and by catecholamine depletion. PAF, which manifests with neurogenic orthostatic hypotension (nOH) and no motor signs of central neurodegeneration, can evolve into PD+nOH. Cerebrospinal fluid (CSF) levels of catecholamine metabolites may indicate central catecholamine deficiency in these synucleinopathies, but the literature is inconsistent and incomplete. Read More

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Early atypical signs and insula hypometabolism predict survival in multiple system atrophy.

J Neurol Neurosurg Psychiatry 2021 Apr 23. Epub 2021 Apr 23.

Department of Neurology and Movement Disorders, University Hospital La Timone, Marseille, France.

Objective: We aim to search for predictors of survival among clinical and brain F-FDG positron emission tomography (PET) metabolic features in our cohort of patients with multiple system atrophy (MSA).

Methods: We included patients with a 'probable' MSA diagnosis for whom a clinical evaluation and a brain PET were performed early in the course of the disease (median 3 years, IQR 2-5). A retrospective analysis was conducted using standardised data collection. Read More

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Gene Therapy in Movement Disorders: A Systematic Review of Ongoing and Completed Clinical Trials.

Front Neurol 2021 6;12:648532. Epub 2021 Apr 6.

Department of Neurological Surgery, College of Medicine, The Ohio State University, Columbus, OH, United States.

We sought to provide an overview of the published and currently ongoing movement disorders clinical trials employing gene therapy, defined as a technology aiming to modulate the expression of one or more genes to achieve a therapeutic benefit. We systematically reviewed movement disorders gene therapy clinical trials from PubMed and ClinicalTrials.gov using a searching strategy that included Parkinson disease (PD), Huntington disease (HD), amino acid decarboxylase (AADC) deficiency, multiple system atrophy (MSA), progressive supranuclear palsy (PSP), dystonia, tremor, ataxia, and other movement disorders. Read More

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Alpha-Synuclein Oligomers and Neurofilament Light Chain Predict Phenoconversion of Pure Autonomic Failure.

Ann Neurol 2021 Apr 21. Epub 2021 Apr 21.

Department of Neurology, Mayo Clinic, Rochester, MN.

Objective: To explore the role of alpha-synuclein (αSyn) oligomers and neurofilament light chain (NfL) in cerebrospinal fluid (CSF) of patients with pure autonomic failure (PAF) as markers of future phenoconversion to multiple system atrophy (MSA).

Methods: Well-characterized patients with PAF (n = 32) were enrolled between June 2016 and February 2019 at Mayo Clinic Rochester and followed prospectively with annual visits to determine future phenoconversion to MSA, Parkinson's disease (PD), or dementia with Lewy bodies (DLB). ELISA was utilized to measure NfL and protein misfolding cyclic amplification (PMCA) to detect αSyn oligomers in CSF collected at baseline. Read More

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Gut Microbiome in Progressive Multiple Sclerosis.

Ann Neurol 2021 Apr 19. Epub 2021 Apr 19.

Ann Romney Center for Neurologic Diseases, Harvard Medical School, Brigham and Women's Hospital, Boston, MA.

Objective: This study was undertaken to investigate the gut microbiome in progressive multiple sclerosis (MS) and how it relates to clinical disease.

Methods: We sequenced the microbiota from healthy controls and relapsing-remitting MS (RRMS) and progressive MS patients and correlated the levels of bacteria with clinical features of disease, including Expanded Disability Status Scale (EDSS), quality of life, and brain magnetic resonance imaging lesions/atrophy. We colonized mice with MS-derived Akkermansia and induced experimental autoimmune encephalomyelitis (EAE). Read More

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Effects of COVID -19 pandemic and lockdown on people with multiple system atrophy participating in a therapeutic education program.

Parkinsonism Relat Disord 2021 Mar 29;86:78-80. Epub 2021 Mar 29.

Parkinson's Disease & Movement Disorders Unit, Neurology Service, Clinical Institute of Neuroscience (ICN), Hospital Clínic de Barcelona / IDIBAPS / CIBERNED (CB06/05/0018-ISCIII) / European Reference Network for Rare Neurological Diseases (ERN-RND), Institut de Neurociències de la Universitat de Barcelona (Maria de Maeztu's Excellence Center), Catalonia, Spain.

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Cerebral Metabolism Related to Cognitive Impairments in Multiple System Atrophy.

Front Neurol 2021 1;12:652059. Epub 2021 Apr 1.

Department of Neurology and National Clinical Research Center for Aging and Medicine, Huashan Hospital, Fudan University, Shanghai, China.

We aimed to characterize the cognitive profiles in multiple system atrophy (MSA) and explore the cerebral metabolism related to the cognitive decline in MSA using F-fluorodeoxyglucose (F-FDG) Positron Emission Tomography (PET). In this study, 105 MSA patients were included for cognitive assessment and 84 of them were enrolled for F-FDG PET analysis. The comprehensive neuropsychological tests covered five main domains including execution, attention, memory, language, and visuospatial function. Read More

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[Inherited Creutzfeldt-Jakob disease with four-octapeptide repeat insertional mutation in the prion gene].

Rinsho Shinkeigaku 2021 Apr 17. Epub 2021 Apr 17.

Department of Neurology and Geriatrics, Kagoshima University Graduate School of Medical and Dental Sciences.

We report a case of a 60-year-old man who presented with symptoms of memory loss, gait disorder, and sluggish movement. We considered both Parkinson's disease and multiple system atrophy as possible diagnoses and consequently hospitalized the patient owing to the worsening symptoms and the development of consciousness disorder. During the course of the disease, dementia, loss of consciousness, and movement disorders worsened rapidly within one year after admission, and the patient eventually developed mutism. Read More

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Adult-onset Alexander disease mimicking multiple system atrophy predominant cerebellar ataxia.

J Clin Neurosci 2021 May 23;87:150-152. Epub 2021 Mar 23.

Department of Neurology, Dokkyo Medical University, Tochigi, Japan. Electronic address:

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High prevalence of serum anti-NH-terminal of α-enolase antibodies in patients with multiple system atrophy and corticobasal syndrome.

J Neurol 2021 Apr 15. Epub 2021 Apr 15.

Department of Neurology, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, Miyagi, 980-8574, Japan.

Background: Hashimoto's encephalopathy with serum anti-NH-terminal of α-enolase (NAE) antibodies occasionally displays clinical symptoms such as cerebellar ataxia and parkinsonism. We studied the frequency of anti-NAE antibodies in patients with Parkinson-plus syndrome.

Methods: We examined the positive rates of anti-NAE antibodies in 47 patients with multiple system atrophy (MSA), 29 patients with Parkinson's disease (PD), eight patients with corticobasal syndrome (CBS), and 18 patients with progressive supranuclear palsy (PSP) using conventional immunoblot analysis. Read More

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Alpha-synuclein seeds in olfactory mucosa of patients with isolated REM sleep behaviour disorder.

Brain 2021 May;144(4):1118-1126

Department of Neurosciences, Biomedicine and Movement Sciences University of Verona, Verona, Italy.

Isolated REM sleep behaviour disorder (RBD) is an early-stage α-synucleinopathy in most, if not all, affected subjects. Detection of pathological α-synuclein in peripheral tissues of patients with isolated RBD may identify those progressing to Parkinson's disease, dementia with Lewy bodies or multiple system atrophy, with the ultimate goal of testing preventive therapies. Real-time quaking-induced conversion (RT-QuIC) provided evidence of α-synuclein seeding activity in CSF and olfactory mucosa of patients with α-synucleinopathies. Read More

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