5,949 results match your criteria Multiple System Atrophy


5-methylcytosine and 5-hydroxymethylcytosine in brains of patients with multiple system atrophy and patients with Parkinson's disease.

J Chem Neuroanat 2018 Dec 14. Epub 2018 Dec 14.

Department of Neurology, University of Bonn, Germany; German Center for Neurodegenerative Diseases (DZNE), University of Bonn, Germany.

Multiple system atrophy (MSA) is a sporadic neurodegenerative disorder of unknown etiology, characterized pathologically by α-synuclein aggregates preferentially found in oligodendroglial cells. DNA methylation has emerged as a mechanism of regulation of α-synuclein expression. Reduced 5-methylcytosine (5-mC) DNA methylation of α-synuclein has been found in the brains of patients with Parkinson's disease (PD). Read More

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http://dx.doi.org/10.1016/j.jchemneu.2018.12.005DOI Listing
December 2018

The biomarker potential of cell-free microRNA from cerebrospinal fluid in Parkinsonian Syndromes.

Mov Disord 2018 Dec 17. Epub 2018 Dec 17.

Department of Neurology, Bispebjerg University Hospital, Copenhagen, Denmark.

Background: MicroRNAs are small noncoding RNAs involved in the post-transcriptional regulation of protein synthesis. Extracellular microRNAs are accessible in a stable form in biofluids.

Objectives: The aim was to identify individual microRNAs and/or subsets of microRNAs in CSF with biomarker potential and thus identify specific putative pathophysiological pathways. Read More

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http://dx.doi.org/10.1002/mds.27542DOI Listing
December 2018

What Is Behind Cerebellar Vertigo and Dizziness?

Cerebellum 2018 Dec 14. Epub 2018 Dec 14.

Department of Neurology, Ludwig-Maximilians University, Marchioninistrasse 15, 81377, Munich, Germany.

The differential diagnosis of vertigo or dizziness as a result of cerebellar disorders can be difficult as many patients with a cerebellar pathology do not present with the full spectrum of cerebellar signs. The main goal of this study was to describe the typical clinical features of these patients with vertigo or dizziness of a cerebellar origin. We reviewed the medical records of 5400 patients with vertigo and dizziness from our tertiary outpatient clinic for vertigo and balance disorders. Read More

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http://dx.doi.org/10.1007/s12311-018-0992-8DOI Listing
December 2018

Altered structural connectivity of the motor subnetwork in multiple system atrophy with cerebellar features.

Eur Radiol 2018 Dec 14. Epub 2018 Dec 14.

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

Objectives: To investigate the structural connectivity of the motor subnetwork in multiple system atrophy with cerebellar features (MSA-C), a distinct subtype of MSA, characterized by predominant cerebellar symptoms.

Methods: Twenty-three patients with MSA-C and 25 age- and gender-matched healthy controls were recruited for the study. Disease severity was quantified using the Unified Multiple System Atrophy Rating Scale (UMSARS). Read More

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http://link.springer.com/10.1007/s00330-018-5874-4
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http://dx.doi.org/10.1007/s00330-018-5874-4DOI Listing
December 2018
2 Reads

Anesthesia in Patients With Multiple-System Atrophy: A Narrative Review and Practice Guidance.

A A Pract 2018 Dec 13. Epub 2018 Dec 13.

Department of Anesthesiology and Pain Management, University of Texas Southwestern Medical Center, Dallas, Texas.

Multiple-system atrophy, a rare adult-onset neurodegenerative disease, is associated with autonomic dysfunction, features of parkinsonism, and cerebellar ataxia. This article discusses multiple-system atrophy pathophysiology, clinical presentation, and perioperative issues pertinent to anesthesia. Read More

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http://dx.doi.org/10.1213/XAA.0000000000000939DOI Listing
December 2018

Abnormal functional connectivity of the amygdala is associated with depressive symptoms in patients with multiple system atrophy.

Neuropsychiatr Dis Treat 2018 15;14:3133-3142. Epub 2018 Nov 15.

Department of Neurology, The First Affiliated Hospital of China Medical University, Shenyang 110001, China,

Purpose: Depressive symptoms are frequent nonmotor symptoms that occur in multiple system atrophy (MSA) patients. However, possible changes that can present in the amygdala (AMY) functional connectivity (FC) of the brain in MSA patients with depressive symptoms (DMSA patients) remain largely unknown.

Materials And Methods: Resting-state functional magnetic resonance imaging scans were obtained from 29 DMSA patients, 28 MSA patients without depression symptoms (NDMSA patients), and 34 healthy controls (HCs). Read More

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http://dx.doi.org/10.2147/NDT.S178657DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6247974PMC
November 2018

Biochemical and morphological classification of disease-associated alpha-synuclein mutants aggregates.

Biochem Biophys Res Commun 2018 Dec 5. Epub 2018 Dec 5.

Laboratory of Structural Neuropathology, Doshisha University Graduate School of Brain Science, 1-3 Miyakodanitatara, Kyotanabe-shi, Kyoto, 610-0394, Japan. Electronic address:

Alpha-synuclein (a-syn) aggregation in brain is implicated in several synucleinopathies, including Parkinson's disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA). Until date, at least six disease-associated mutations in a-syn (namely A30P, E46K, H50Q, G51D, A53T, and A53E) are known to cause dominantly inherited familial forms of synucleinopathies. Previous studies using recombinant proteins have reported that a subset of disease-associated mutants show higher aggregation propensities and form spectroscopically distinguishable aggregates compared to wild-type (WT). Read More

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http://dx.doi.org/10.1016/j.bbrc.2018.11.200DOI Listing
December 2018

Retinal degeneration is associated with brain volume reduction and prognosis in radiologically isolated syndrome.

Mult Scler 2018 Dec 11:1352458518817987. Epub 2018 Dec 11.

Department of Neurology, Hacettepe University, Ankara, Turkey.

Background:: The extent of neurodegeneration in the earliest stages of central nervous system (CNS) demyelination is not known. Optical coherence tomography (OCT) is a powerful tool to study neurodegeneration in demyelinating disorders.

Objectives:: To study neuroaxonal loss in the retina of individuals with radiologically isolated syndrome (RIS) and investigate whether OCT measurements are associated with brain volumetrics and clinical conversion to multiple sclerosis (MS). Read More

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http://dx.doi.org/10.1177/1352458518817987DOI Listing
December 2018

Modeling Parkinson's Disease and Atypical Parkinsonian Syndromes Using Induced Pluripotent Stem Cells.

Int J Mol Sci 2018 Dec 4;19(12). Epub 2018 Dec 4.

Department of Neurology, Fukuoka University, Fukuoka 814-0180, Japan.

Parkinson's disease (PD) and atypical parkinsonian syndromes are age-dependent multifactorial neurodegenerative diseases, which are clinically characterized by bradykinesia, tremor, muscle rigidity and postural instability. Although these diseases share several common clinical phenotypes, their pathophysiological aspects vary among the disease categories. Extensive animal-based approaches, as well as postmortem studies, have provided important insights into the disease mechanisms and potential therapeutic targets. Read More

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http://dx.doi.org/10.3390/ijms19123870DOI Listing
December 2018
9 Reads

Quantification of brain-derived extracellular vesicles in plasma as a biomarker to diagnose Parkinson's and related diseases.

Parkinsonism Relat Disord 2018 Nov 20. Epub 2018 Nov 20.

Department of Neurology, Kyoto Prefectural University of Medicine, Kyoto, 602-0841, Japan; Department of Molecular Pathobiology of Brain Diseases, Kyoto Prefectural University of Medicine, Kyoto, 602-0841, Japan. Electronic address:

Introduction: There is still a substantial unmet need for blood-based biomarkers to make an objective diagnosis of Parkinson's disease (PD) and the parkinsonism-plus syndromes. This study is aimed to determine whether enumeration of brain-derived exosomes (BDEs) in plasma is informative in the diagnosis of those diseases.

Methods: We have developed a specific method to enumerate the plasma levels of neuron-derived, astrocyte-derived, and oligodendrocyte-derived exosomes (NDEs, ADEs and ODEs, respectively), and quantified them individually in patients with PD (n = 15), multiple system atrophy (MSA, n = 15), progressive supranuclear palsy (PSP, n = 7) and disease controls (n = 15). Read More

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http://dx.doi.org/10.1016/j.parkreldis.2018.11.021DOI Listing
November 2018
1 Read

Exploring bedside clinical features of parkinsonism: A focus on differential diagnosis.

Parkinsonism Relat Disord 2018 Nov 5. Epub 2018 Nov 5.

Center for Neurological Restoration, Cleveland Clinic, Cleveland, OH, USA.

The proper diagnosis of parkinsonian disorders usually involves three steps: identifying core features of parkinsonism; excluding other causes; and collating supportive evidence based on clinical signs or investigations. While the recognition of cardinal parkinsonian features is usually straightforward, the appreciation of clinical features suggestive of specific parkinsonian disorders can be challenging, and often requires greater experience and skills. In this review, we outline the clinical features that are relevant to the differential diagnosis of common neurodegenerative parkinsonian disorders, including Parkinson's disease, multiple system atrophy, progressive supranuclear palsy, and corticobasal degeneration. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S13538020183048
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http://dx.doi.org/10.1016/j.parkreldis.2018.11.005DOI Listing
November 2018
3 Reads

Phenoconversion from Idiopathic Rapid Eye Movement Sleep Behavior Disorder to Lewy Body Disease.

Mov Disord Clin Pract 2018 Sep-Oct;5(5):506-511. Epub 2018 Oct 3.

Center of Sleep Medicine Dokkyo Medical University Hospital Tochigi Japan.

Background: The conversion rate and estimated risk of neurodegenerative diseases vary with idiopathic rapid eye movement sleep behavior disorder (IRBD).

Methods: This retrospective cohort study examined 273 patients (213 men, 60 women) with polysomnographic-confirmed IRBD (192 and 81 patients in the Sleep Center [SC] cohort and Neurological Center [NC] cohort, respectively) who were followed longitudinally. The date of diagnosis was determined as the onset of an overt neurological syndrome. Read More

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http://doi.wiley.com/10.1002/mdc3.12647
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http://dx.doi.org/10.1002/mdc3.12647DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6207131PMC
October 2018
5 Reads

An autopsy case of progressive multifocal leukoencephalopathy after rituximab therapy for malignant lymphoma.

Neuropathology 2018 Dec 3. Epub 2018 Dec 3.

Department of Pathology, Kurume University School of Medicine, Kurume, Japan.

Progressive multifocal leukoencephalopathy (PML) is a rare fatal demyelinating disease of the central nervous system caused by reactivation of the JC virus (JCV), which is named after the initials of the patient from whom the virus was first isolated. JCV is highly prevalent worldwide, infects humans in early childhood, and the infection persists throughout the course of life in latent form. The present paper deals with the second autopsy case report of rituximab-associated PML in Japan. Read More

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http://doi.wiley.com/10.1111/neup.12526
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http://dx.doi.org/10.1111/neup.12526DOI Listing
December 2018
4 Reads

Investigating the neuroprotective effect of AAV-mediated β-synuclein overexpression in a transgenic model of synucleinopathy.

Sci Rep 2018 Dec 3;8(1):17563. Epub 2018 Dec 3.

ANSES (French Agency for Food, Environmental and Occupational Health & Safety), University of Lyon, Lyon, France.

Parkinson's disease (PD) and multiple system atrophy (MSA) are neurodegenerative diseases characterized by inclusions mainly composed of α-synuclein (α-syn) aggregates. The objective of this study was to investigate if β-synuclein (β-syn) overexpression could have beneficial effects by inhibiting the aggregation of α-syn. The M83 transgenic mouse is a model of synucleinopathy, which develops severe motor symptoms associated with aggregation of α-syn. Read More

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http://dx.doi.org/10.1038/s41598-018-35825-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6277436PMC
December 2018
2 Reads

Stridor-related gray matter alterations in multiple system atrophy: A pilot study.

Parkinsonism Relat Disord 2018 Nov 17. Epub 2018 Nov 17.

Department of Biomedical and NeuroMotor Sciences, University of Bologna, Bologna, Italy; IRCCS Istituto delle Scienze Neurologiche di Bologna, Clinica Neurologica, Bologna, Italy.

Introduction: The neuroanatomical substrate of stridor associated with Multiple System Atrophy (MSA) remains unclear. We evaluated stridor-related gray matter (GM) changes in MSA.

Methods: 36 MSA patients underwent standardized nocturnal video-polysomnography and brain MRI. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S13538020183051
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http://dx.doi.org/10.1016/j.parkreldis.2018.11.018DOI Listing
November 2018
5 Reads

Treatment of multiple system atrophy - the past, present and future.

Am J Clin Exp Immunol 2018 5;7(5):88-94. Epub 2018 Oct 5.

Department of Neurology, Liaocheng People's Hospital, Liaocheng Clinical School, Taishan Medical University Liaocheng, PR China.

Multiple system atrophy is a sporadic progressive degenerative disease which is characterized by multiple central nervous systems involved. So far, there is no effective medicine to cure MSA. The main research direction of treatment includes immunization transplantation and cytotherapy. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6261842PMC
October 2018
1 Read

Understanding the role of genetic variability in LRRK2 in Indian population.

Mov Disord 2018 Nov 28. Epub 2018 Nov 28.

Centre for Genetic Epidemiology, Institute for Clinical Epidemiology and Applied Biometry, University of Tübingen, Tübingen, Germany.

Background: Genetic variability in LRRK2 has been unequivocally established as a major risk factor for familial and sporadic forms of PD in ethnically diverse populations.

Objectives: To resolve the role of LRRK2 in the Indian population.

Methods: We performed targeted resequencing of the LRRK2 locus in 288 cases and 298 controls and resolved the haplotypic structure of LRRK2 in a combined cohort of 800 cases and 402 controls in the Indian population. Read More

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http://doi.wiley.com/10.1002/mds.27558
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http://dx.doi.org/10.1002/mds.27558DOI Listing
November 2018
2 Reads

Parkinson's disease and multiple system atrophy have distinct α-synuclein seed characteristics.

J Biol Chem 2018 Nov 26. Epub 2018 Nov 26.

University of Texas Southwestern Medical Center, United States.

Parkinson's disease (PD)§ and multiple system atrophy (MSA) are distinct clinical syndromes characterized by the pathological accumulation of α-synuclein (α-syn) protein fibrils in neurons and glial cells. These disorders and other neurodegenerative diseases may progress via prion-like mechanisms. The prion model of propagation predicts the existence of "strains" that link pathological aggregate structure and neuropathology. Read More

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http://dx.doi.org/10.1074/jbc.RA118.004471DOI Listing
November 2018
4 Reads

Neuroimaging Applications in Chronic Ataxias.

Int Rev Neurobiol 2018 29;143:109-162. Epub 2018 Oct 29.

Nuclear Medicine, "Le Scotte" University Hospital, Siena, Italy.

Magnetic resonance imaging (MRI), single photon emission computed tomography (SPECT) and positron emission tomography (PET) are the main instruments for neuroimaging investigation of patients with chronic ataxia. MRI has a predominant diagnostic role in the single patient, based on the visual detection of three patterns of atrophy, namely, spinal atrophy, cortical cerebellar atrophy and olivopontocerebellar atrophy, which correlate with the aetiologies of inherited or sporadic ataxia. In fact spinal atrophy is observed in Friedreich ataxia, cortical cerebellar atrophy in Ataxia Telangectasia, gluten ataxia and Sporadic Adult Onset Ataxia and olivopontocerebellar atrophy in Multiple System Atrophy cerebellar type. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00747742183011
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http://dx.doi.org/10.1016/bs.irn.2018.09.011DOI Listing
October 2018
2 Reads

Pharmacokinetics and efficacy of PT302, a sustained-release Exenatide formulation, in a murine model of mild traumatic brain injury.

Neurobiol Dis 2018 Nov 21. Epub 2018 Nov 21.

Drug Design and Development Section, Translational Gerontology Branch, Intramural Research Program, National Institutes of Health, National Institute on Aging, Baltimore, MD, USA; Peptron Inc., Yuseong-gu, Daejeon, Republic of Korea.

Traumatic brain injury (TBI) is a neurodegenerative disorder for which no effective pharmacological treatment is available. Glucagon-like peptide 1 (GLP-1) analogues such as Exenatide have previously demonstrated neurotrophic and neuroprotective effects in cellular and animal models of TBI. However, chronic or repeated administration was needed for efficacy. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S09699961183066
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http://dx.doi.org/10.1016/j.nbd.2018.11.023DOI Listing
November 2018
10 Reads

An update on advances in magnetic resonance imaging of multiple system atrophy.

J Neurol 2018 Nov 20. Epub 2018 Nov 20.

Dementia Research Centre, Department of Neurodegenerative Disease, UCL Institute of Neurology, Queen Square, WC1N 3BG, London, UK.

In this review, we describe how different neuroimaging tools have been used to identify novel MSA biomarkers, highlighting their advantages and limitations. First, we describe the main structural MRI changes frequently associated with MSA including the 'hot cross-bun' and 'putaminal rim' signs as well as putaminal, pontine, and middle cerebellar peduncle (MCP) atrophy. We discuss the sensitivity and specificity of different supra- and infratentorial changes in differentiating MSA from other disorders, highlighting those that can improve diagnostic accuracy, including the MCP width and MCP/superior cerebellar peduncle (SCP) ratio on T1-weighted imaging, raised putaminal diffusivity on diffusion-weighted imaging, and increased T2* signal in the putamen, striatum, and substantia nigra on susceptibility-weighted imaging. Read More

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http://dx.doi.org/10.1007/s00415-018-9121-3DOI Listing
November 2018

Anle138b modulates α-synuclein oligomerization and prevents motor decline and neurodegeneration in a mouse model of multiple system atrophy.

Mov Disord 2018 Nov 19. Epub 2018 Nov 19.

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

Background: MSA is a fatal neurodegenerative disease characterized by autonomic failure and severe motor impairment. Its main pathological hallmark is the accumulation of α-synuclein in oligodendrocytes, leading to glial and neuronal dysfunction and neurodegeneration. These features are recapitulated in the PLP-hαSyn mouse model expressing human α-synuclein in oligodendrocytes. Read More

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http://dx.doi.org/10.1002/mds.27562DOI Listing
November 2018
2 Reads
5.680 Impact Factor

Guidelines of the Polish Medical Society of radiology for the routinely used MRI protocol in patients with multiple sclerosis.

Neurol Neurochir Pol 2018 Nov - Dec;52(6):638-642. Epub 2018 Oct 4.

Department of Radiology, Medical Centre for Postgraduate Education, Warsaw, Poland.

Magnetic resonance imaging is widely used in diagnosing multiple sclerosis as a basic method for detecting and monitoring the disease. INTRODUCTION: Polish Medical Society of Radiology presents the second version of the recommendations for the routinely conducted MRI in multiple sclerosis, which include new data and practical remarks for radiographers and radiologists. The recommended protocol aims to improve the imaging procedure and, most importantly, to standardize conducting MRI scans in all MRI departments. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00283843183010
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http://dx.doi.org/10.1016/j.pjnns.2018.09.010DOI Listing
October 2018
2 Reads

Protective and therapeutic role of Bilobalide in cuprizone-induced demyelination.

Int Immunopharmacol 2019 Jan 13;66:69-81. Epub 2018 Nov 13.

The Key Research Laboratory of Benefiting Qi for Acting Blood Circulation Method to Treat Multiple Sclerosis of State Administration of Traditional Chinese Medicine, Shanxi University of Chinese Medicine, Taiyuan 030024, China; The First Clinical College, Shanxi Medical University, Taiyuan 030001, China; Institute of Brain Science, Shanxi Datong University, Datong 037009, China. Electronic address:

Multiple sclerosis (MS) is a chronic demyelinating disease of the central nervous system characterized by recurrent and progressive demyelination, neuroinflammation and oligodendrocyte loss. The cuprizone (CPZ) model is characterized by primary and reversible demyelination, accompanied by oligodendrocyte loss and neuroinflammation. In the current study, we explored the efficiency of Bilobalide in the demyelination and remyelination. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15675769183074
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http://dx.doi.org/10.1016/j.intimp.2018.09.041DOI Listing
January 2019
6 Reads

Oligodendroglial A-synucleinopathy driven neuroinflammation in multiple system atrophy.

Brain Pathol 2018 Nov 16. Epub 2018 Nov 16.

Department of Molecular Neurology, University Hospital Erlangen Friedrich-Alexander-Universität Erlangen- Nürnberg, Schwabachanlage 6, 91054, Erlangen, Germany.

Neuroinflammation and oligodendroglial cytoplasmic α-synuclein (α-syn) inclusions (GCIs) are important neuropathological characteristics of multiple system atrophy (MSA). GCIs are known to interfere with oligodendroglial maturation and consequently result in myelin loss. The neuroinflammatory phenotype in the context of MSA, however, remains poorly understood. Read More

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http://doi.wiley.com/10.1111/bpa.12678
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http://dx.doi.org/10.1111/bpa.12678DOI Listing
November 2018
10 Reads

Autophagy in neurodegeneration and aging.

Aging (Albany NY) 2018 Nov 14. Epub 2018 Nov 14.

Hirosaki University Graduate School of Medicine, Department of Neuropathology, Institute of Brain Science, Hirosaki, Japan.

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http://dx.doi.org/10.18632/aging.101652DOI Listing
November 2018
1 Read

A novel autosomal recessive orthostatic hypotension syndrome: and other updates on recent autonomic research.

Clin Auton Res 2018 Dec 10;28(6):565-567. Epub 2018 Nov 10.

Stanford Neurosciences Health Center, 213 Quarry Road, 2nd Floor, Palo Alto, CA, 94304, USA.

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http://link.springer.com/10.1007/s10286-018-0578-z
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http://dx.doi.org/10.1007/s10286-018-0578-zDOI Listing
December 2018
4 Reads

Plasma levels of soluble NCAM in multiple sclerosis.

J Neurol Sci 2018 Oct 28;396:36-41. Epub 2018 Oct 28.

Department of Biomedical and Specialty Surgical Sciences, University of Ferrara, Italy. Electronic address:

In multiple sclerosis (MS), several adhesion molecules are involved within the central nervous system in inflammatory and neurodegenerative processes that are associated to progressive disability and increasing brain atrophy. The neural cell adhesion molecule (NCAM) has been suggested to participate in the reparative mechanisms and in the remyelination processes, key issues in MS pathology. We aimed at investigating plasma levels of the seldom investigated soluble (s)NCAM, and as comparison those of intercellular adhesion molecule-1 (sICAM-1) and vascular adhesion molecule-1 (sVCAM-1), and their association with clinical and MRI measures of lesion volumes and of global and regional atrophy. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S0022510X183043
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http://dx.doi.org/10.1016/j.jns.2018.10.023DOI Listing
October 2018
5 Reads

Red flags phenotyping: A systematic review on clinical features in atypical parkinsonian disorders.

Parkinsonism Relat Disord 2018 Oct 6. Epub 2018 Oct 6.

Department of Neurology, Santa Maria University Hospital, Terni, Italy.

To establish a clinical diagnosis of a parkinsonian disorder, physicians rely on their ability to identify relevant red flags, in addition to cardinal features, to support or refute their working diagnosis in an individual patient. The term 'red flag', was originally coined in 1989 to define the presence of non-cardinal features that may raise a suspicion of multiple system atrophy (MSA), or at least suggest alternative diagnosis to Parkinson's disease (PD). Since then, the term 'red flag', has been consistently used in the literature to denote the clinical history or signs that may signal to physicians the possibility of an atypical parkinsonian disorder (APD). Read More

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http://dx.doi.org/10.1016/j.parkreldis.2018.10.009DOI Listing
October 2018
1 Read

Structural Imaging in Atypical Parkinsonism.

Int Rev Neurobiol 2018 24;142:67-148. Epub 2018 Sep 24.

Department of Neurology, Medical University Innsbruck, Innsbruck, Austria; Neuroimaging Research Core Facility, Medical University Innsbruck, Innsbruck, Austria. Electronic address:

Qualitative and quantitative structural magnetic resonance imaging offer objective measures of the underlying neurodegeneration in atypical parkinsonism. Regional changes in tissue volume, signal changes and increased deposition of iron as assessed with different structural MRI techniques are surrogate markers of underlying neurodegeneration and may reflect cell loss, microglial proliferation and astroglial activation. Structural MRI has been explored as a tool to enhance diagnostic accuracy in differentiating atypical parkinsonian disorders (APDs). Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00747742183007
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http://dx.doi.org/10.1016/bs.irn.2018.08.010DOI Listing
September 2018
4 Reads

SPECT Molecular Imaging in Atypical Parkinsonism.

Int Rev Neurobiol 2018 29;142:37-65. Epub 2018 Aug 29.

University Hospital and Julius-Maximilians University, Würzburg, Germany. Electronic address:

Atypical parkinsonism is the second most common diagnosis for patients with hypokinetic movement disorders. Beside common parkinsonian symptoms (i.e. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00747742183007
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http://dx.doi.org/10.1016/bs.irn.2018.08.006DOI Listing
August 2018
3 Reads

PET Molecular Imaging in Atypical Parkinsonism.

Int Rev Neurobiol 2018 8;142:3-36. Epub 2018 Oct 8.

Newcastle University Newcastle Magnetic Resonance Centre & Positron Emission Tomography Centre, Newcastle University Campus for Ageing & Vitality, Newcastle upon Tyne, United Kingdom; Department of Clinical Medicine-Positron Emission Tomography Centre, Aarhus University, Aarhus, Denmark. Electronic address:

Multiple System Atrophy, Progressive Supranuclear Palsy, and Corticobasal Degeneration are three neurodegenerative disorders characterized by parkinsonism along with involvement of other brain cortical and subcortical regions. The ante mortem diagnosis of these disorders is extremely challenging with up to a quarter of these patients being misdiagnosed, particularly in the early stages of disease. While highly specific and sensitive imaging biomarkers of individual atypical parkinsonisms have not been identified yet, molecular PET and SPECT imaging have improved our knowledge of the physiopathology and neuropathology of these disorders and are often used as supportive criteria for the differential diagnosis of these conditions. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00747742183010
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http://dx.doi.org/10.1016/bs.irn.2018.09.001DOI Listing
October 2018
2 Reads
1.921 Impact Factor

Functional MRI in Atypical Parkinsonisms.

Int Rev Neurobiol 2018 25;142:149-173. Epub 2018 Oct 25.

Neuroimaging Research Unit, Institute of Experimental Neurology, Division of Neuroscience, San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy; Department of Neurology, Institute of Experimental Neurology, Division of Neuroscience, San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy.

The present chapter reports the current knowledge on the use of functional MRI (fMRI) in patients with atypical parkinsonisms, including Multiple System Atrophy, Corticobasal Syndrome and Progressive Supranuclear Palsy syndrome. Both resting state functional connectivity and task-based brain activity abnormalities are reported in atypical parkinsonisms relative to healthy controls and Parkinson's disease patients. Functional alterations were observed earlier than structural damage and may help to make early diagnosis. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00747742183010
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http://dx.doi.org/10.1016/bs.irn.2018.09.002DOI Listing
October 2018
3 Reads

Abnormal Golgi morphology and decreased COPI function in cells with low levels of SMN.

Brain Res 2018 Nov 5. Epub 2018 Nov 5.

Walther Hall, R3 C636, 980 West Walnut Street, Indianapolis, IN 46202 317-278-7319, United States. Electronic address:

We report here the finding of abnormal Golgi apparatus morphology in motor neuron like cells depleted of SMN as well as Golgi apparatus morphology in SMA patient fibroblasts. Rescue experiments demonstrate that this abnormality is dependent on SMN, but can also be rescued by expression of the COPI coatomer subunit alpha-COP. A motor neuron-like cell line containing an inducible alpha-COP shRNA was created to generate a parallel system to study knockdown of SMN or alpha-COP. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00068993183055
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http://dx.doi.org/10.1016/j.brainres.2018.11.005DOI Listing
November 2018
9 Reads

MIBG myocardial scintigraphy in progressive supranuclear palsy.

J Neurol Sci 2018 Oct 22;396:3-7. Epub 2018 Oct 22.

Division of Respirology, Neurology and Rheumatology, Department of Medicine, Kurume University School of Medicine, 67 Asashi-machi, Kurume 830-0011, Japan.

Background And Objectives: Meta-iodobenzylguanidine (MIBG) myocardial scintigraphy is an effective tool for distinguishing Parkinson's disease (PD) from other diseases accompanied by parkinsonism. Unlike other Parkinsonian diseases, in PD, MIBG accumulation in the heart tends to decrease. However, previous studies have reported that a decrease in MIBG accumulation also occurs in progressive supranuclear palsy (PSP). Read More

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http://dx.doi.org/10.1016/j.jns.2018.10.019DOI Listing
October 2018
1 Read

Cerebrospinal fluid pro-inflammatory cytokines differentiate parkinsonian syndromes.

J Neuroinflammation 2018 Nov 3;15(1):305. Epub 2018 Nov 3.

Department of Neurology, Bispebjerg University Hospital, Bispebjerg Bakke 23, Copenhagen, Denmark.

Introduction: Neuroinflammation has been established to be part of the neuropathological changes in Parkinson's disease (PD) and atypical parkinsonism (APD). Activated microglia play a key role in neuroinflammation by release of cytokines. Evidence of the disparity, if any, in the neuroinflammatory response between PD and APD is sparse. Read More

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http://dx.doi.org/10.1186/s12974-018-1339-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6215346PMC
November 2018
1 Read

Clinics in diagnostic imaging (191). Multiple system atrophy-cerebellar type (MSA-C).

Singapore Med J 2018 Oct;59(10):550-554

Department of Diagnostic Radiology, Singapore General Hospital, Singapore.

A 49-year-old Chinese man was evaluated for progressive uncoordinated movements, dysphagia and urinary symptoms. Magnetic resonance imaging demonstrated a cruciform pattern of T2-weighted hyperintensity within the pons and selective atrophy of the cerebellar hemispheres and pons. The clinical history and radiological findings were consistent with a diagnosis of multiple system atrophy-cerebellar type. Read More

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http://www.smj.org.sg/article/clinics-diagnostic-imaging-191
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http://dx.doi.org/10.11622/smedj.2018128DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6199191PMC
October 2018
7 Reads

Early predictors of mortality in parkinsonism and Parkinson disease: A population-based study.

Neurology 2018 Nov 31;91(22):e2045-e2056. Epub 2018 Oct 31.

From the Department of Pharmacology and Clinical Neuroscience (D.B., M.E.D., J.L., L.F.), Epidemiology and Global Health Unit, Department of Public Health and Clinical Medicine (G.G.), Department of Psychology (M.E.D.), and Department of Radiation Sciences, Diagnostic Radiology and Umeå Center for Functional Brain Imaging (S.J.M., K.R.), Umeå University; Institute of Neuroscience and Physiology (H.Z., K.B.), Department of Psychiatry and Neurochemistry, Sahlgrenska Academy at University of Gothenburg, Mölndal; Clinical Neurochemistry Laboratory (H.Z., K.B.), Sahlgrenska University Hospital, Mölndal, Sweden; Department of Molecular Neuroscience (H.Z.), University College London Institute of Neurology; and UK Dementia Research Institute at UCL (H.Z.), London, UK.

Objective: To examine mortality and associated risk factors, including possible effects of mild cognitive impairment, imaging, and CSF abnormalities, in a community-based population with incident parkinsonism and Parkinson disease.

Methods: One hundred eighty-two patients with new-onset, idiopathic parkinsonism were diagnosed from January 2004 through April 2009, in a catchment area of 142,000 inhabitants in Sweden. Patients were comprehensively investigated according to a multimodal research protocol and followed prospectively for up to 13. Read More

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http://www.neurology.org/lookup/doi/10.1212/WNL.000000000000
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http://dx.doi.org/10.1212/WNL.0000000000006576DOI Listing
November 2018
5 Reads

Clinical characteristics and quality of life in Chinese patients with multiple system atrophy.

Brain Behav 2018 Oct 30:e01135. Epub 2018 Oct 30.

Department of Neurology & The Collaborative Innovation Center for Brain Science, Ruijin Hospital affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China.

Objectives: Multiple system atrophy (MSA) is a progressive neurodegenerative disorder that causes early sustained disability and poor health-related quality of life (HrQoL). The clinical features and their effects on the HrQoL of patients in China have received little attention in the research literature. We evaluated the clinical characteristics and HrQoL of Chinese patients with MSA. Read More

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http://dx.doi.org/10.1002/brb3.1135DOI Listing
October 2018
5 Reads

MRI evaluation of progressive supranuclear palsy: differentiation from Parkinson's disease and multiple system atrophy.

Neurol Res 2018 Oct 30:1-8. Epub 2018 Oct 30.

a Department of Radiology , Ege University Medical Faculty , Izmir , Turkey.

Objectives: To evaluate the magnetic resonance imaging (MRI)-derived parameters in differentiation of patients with progressive supranuclear palsy (PSP) from patients with Parkinson's disease (PD), multiple system atrophy (MSA), and control subjects was aimed.

Methods: Thirty-three patients [mean age, 65.21 ± 4. Read More

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http://dx.doi.org/10.1080/01616412.2018.1541115DOI Listing
October 2018
2 Reads

Immune Thymic Profile of the MOG-Induced Experimental Autoimmune Encephalomyelitis Mouse Model.

Front Immunol 2018 11;9:2335. Epub 2018 Oct 11.

Life and Health Sciences Research Institute (ICVS), School of Medicine, University of Minho, Braga, Portugal.

Multiple sclerosis (MS) is a chronic, immune-mediated, demyelinating disease that affects the neurons of the central nervous system. Activated T cells, specific for myelin epitopes, cross the brain barriers, and react against the myelin sheath, leading to demyelination. Since T cells are generated within the thymus, here we explored, in mice, the alterations occurring in this organ throughout the different phases of the disease. Read More

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https://www.frontiersin.org/article/10.3389/fimmu.2018.02335
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http://dx.doi.org/10.3389/fimmu.2018.02335DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6194318PMC
October 2018
5 Reads

Dynamic Formation of Microvillus Inclusions During Enterocyte Differentiation in -Deficient Intestinal Organoids.

Cell Mol Gastroenterol Hepatol 2018 14;6(4):477-493.e1. Epub 2018 Aug 14.

German Cancer Consortium (Deutsches Konsortium für Translationale Krebsforschung), Heidelberg, Germany.

Background & Aims: Microvillus inclusion disease (MVID) is a congenital intestinal malabsorption disorder caused by defective apical vesicular transport. Existing cellular models do not fully recapitulate this heterogeneous pathology. The aim of this study was to characterize 3-dimensional intestinal organoids that continuously generate polarized absorptive cells as an accessible and relevant model to investigate MVID. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S2352345X183010
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http://dx.doi.org/10.1016/j.jcmgh.2018.08.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6198061PMC
August 2018
7 Reads

Subtle Esophageal Motility Alterations in Parkinsonian Syndromes: Synucleinopathies vs. Tauopathies.

Mov Disord Clin Pract 2018 Jul-Aug;5(4):406-412. Epub 2018 May 15.

Department of Neurology University Hospital of Münster Albert-Schweitzer-Campus 1, D- 48149, Münster Germany.

Background: Esophageal dysfunction is a frequent phenomenon in Parkinson's disease during all disease stages, but data about esophageal involvement in atypical parkinsonian syndromes as well as possible differences between alpha-synucleinopathies and tauopathies, including causative links to the origin of the dysfunction, are lacking so far.

Objective: To describe esophageal alternation patterns in different parkinsonian syndromes and to look for differences supporting the hypothesis of alpha-synuclein aggregation being linked to gastrointestinal impairment in parkinsonian syndromes.

Methods: We performed an analysis and comparison of esophageal high-resolution manometry examination parameters in 10 patients with Parkinson's disease, 10 patients with multiple system atrophy (both alpha-synucleinopathies), 10 patients with progressive supranuclear palsy (tauopathy), and 10 age-matched controls. Read More

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http://doi.wiley.com/10.1002/mdc3.12616
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http://dx.doi.org/10.1002/mdc3.12616DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6174416PMC
May 2018
3 Reads

Sleep-Disordered Breathing in Multiple System Atrophy: Pathophysiology and New Insights for Diagnosis and Treatment.

J Clin Sleep Med 2018 Oct 15;14(10):1641-1642. Epub 2018 Oct 15.

Department of Clinical and Experimental Medicine, Sleep Medicine Center of the Neurophysiopathology and Movement Disorders Unit, University of Messina, Messina, Italy.

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http://dx.doi.org/10.5664/jcsm.7360DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6175796PMC
October 2018

Breathing Irregularity Is Independently Associated With the Severity of Obstructive Sleep Apnea in Patients With Multiple System Atrophy.

J Clin Sleep Med 2018 Oct 15;14(10):1661-1667. Epub 2018 Oct 15.

Department of Neurology and Geriatrics, Gifu University Graduate School of Medicine, Gifu, Japan.

Study Objectives: Multiple system atrophy (MSA) is a neurodegenerative disease characterized by the combination of cerebellar ataxia, parkinsonism, and autonomic disturbance. Patients with MSA frequently have sleep-disordered breathing. In some patients with MSA, central sleep apnea manifests during the disease's natural course or as a treatment effect. Read More

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http://dx.doi.org/10.5664/jcsm.7368DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6175809PMC
October 2018
8 Reads

Multiplex Droplet Digital PCR Method Applicable to Newborn Screening, Carrier Status, and Assessment of Spinal Muscular Atrophy.

Clin Chem 2018 Dec 23;64(12):1753-1761. Epub 2018 Oct 23.

Departments of Laboratory Medicine and Pathology.

Background: Spinal muscular atrophy (SMA) is a progressive neuromuscular disorder with neuronal degeneration leading to muscular atrophy and respiratory failure. SMA is frequently caused by homozygous deletions that include exon 7 of the survival motor neuron gene , and its clinical course is influenced by the copy number of a nearby 5q paralog, . Multiple ligation probe amplification (MLPA) and real-time quantitative PCR (qPCR) can detect deletions. Read More

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http://www.clinchem.org/lookup/doi/10.1373/clinchem.2018.293
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http://dx.doi.org/10.1373/clinchem.2018.293712DOI Listing
December 2018
7 Reads

Eye movement deficits in X-linked dystonia-parkinsonism are related to striatal degeneration.

Parkinsonism Relat Disord 2018 Oct 15. Epub 2018 Oct 15.

Department of Neurology, University of Luebeck, Luebeck, Germany. Electronic address:

Background: X-linked dystonia-parkinsonism (XDP) is characterized by the unique transition of dystonia to parkinsonism and striatal degeneration. Slowing of saccades on clinical examination has been taken as suggestive of a progressive supranuclear palsy (PSP) phenotype.

Objectives: To elucidate whether eye movement abnormalities in XDP patients reflect striatonigral impairment or deficits in the brainstem saccade generator as present in PSP. Read More

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http://dx.doi.org/10.1016/j.parkreldis.2018.10.016DOI Listing
October 2018
1 Read

Neuropsychiatric disturbances in atypical parkinsonian disorders.

Neuropsychiatr Dis Treat 2018 9;14:2643-2656. Epub 2018 Oct 9.

IRCCS Neuromed, Pozzilli, Italy,

Multiple system atrophy (MSA), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD) are the most common atypical parkinsonisms. These disorders are characterized by varying combinations of autonomic, cerebellar and pyramidal system, and cognitive dysfunctions. In this paper, we reviewed the evidence available on the presence and type of neuropsychiatric disturbances in MSA, PSP, and CBD. Read More

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https://www.dovepress.com/neuropsychiatric-disturbances-in-a
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http://dx.doi.org/10.2147/NDT.S178263DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6186304PMC
October 2018
7 Reads

Effects of anodal tDCS on motor and cognitive function in a patient with multiple system atrophy.

Disabil Rehabil 2018 Oct 21:1-5. Epub 2018 Oct 21.

a Department of Neurosurgery , Evangelismos Hospital, National and Kapodistrian University of Athens , Athens , Greece.

Purpose: Multiple system atrophy (MSA) is a progressive neurodegenerative disease characterized by postural instability, autonomic failure, cerebellar ataxia, and cognitive deficits. There is currently no effective cure. Transcranial direct current stimulation (tDCS), offers promise in amendment of motor, and cognitive performance in advanced Parkinson's disease. Read More

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http://dx.doi.org/10.1080/09638288.2018.1510043DOI Listing
October 2018
10 Reads

High Resolution Manofluorographic Study in Patients With Multiple System Atrophy: Possible Early Detection of Upper Esophageal Sphincter and Proximal Esophageal Abnormality.

Front Med (Lausanne) 2018 5;5:286. Epub 2018 Oct 5.

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

Multiple system atrophy (MSA) has detrimental effects on swallowing function. The swallowing function of patients with MSA has not been systematically characterized and the underlying pathophysiological mechanisms of dysphagia remain poorly understood. To investigate the characteristics of swallow function in MSA using high-resolution manofluorography (HRMF). Read More

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https://www.frontiersin.org/article/10.3389/fmed.2018.00286/
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http://dx.doi.org/10.3389/fmed.2018.00286DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6182049PMC
October 2018
3 Reads