6,004 results match your criteria Multiple System Atrophy


Abnormal α-synuclein deposits in skin nerves: intra- and inter-laboratory reproducibility.

Eur J Neurol 2019 Feb 15. Epub 2019 Feb 15.

Philipps University, Marburg, Germany.

Background: Visualization of phosphorylated α-synuclein at serine 129 (p-syn) in skin nerves is a promising test for the in vivo diagnosis of synucleinopathies. Here we aimed to establish the intra- and inter-laboratory reproducibility of quantification of intraneural p-syn immunoreactivity in two laboratories with a major expertise (Würzburg and Bologna).

Methods: We enrolled 43 patients affected by Parkinson's disease (PD: 21 patients), Dementia with Lewy Body (DLB: 1), REM sleep behavior disorder (RBD: 11), Multiple System Atrophy (MSA-P: 4) and small fiber neuropathy (SFN: 6). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/ene.13939DOI Listing
February 2019

Transmission of α-synuclein seeds in neurodegenerative disease: recent developments.

Lab Invest 2019 Feb 13. Epub 2019 Feb 13.

Department of Pathology and Laboratory Medicine, Institute on Aging and Center for Neurodegenerative Disease Research, University of Pennsylvania School of Medicine, Philadelphia, PA, 19104, USA.

Cell-to-cell transmission of proteopathic alpha-synuclein (α-syn) seeds is increasingly thought to underlie the progression of neurodegenerative diseases including Parkinson's disease, dementia with Lewy bodies, multiple system atrophy, and related synucleinopathies. As such, it is important to understand the chemical and biological relationships between cells and pathological aggregates of α-syn. This brief review updates our understanding of the templated spread of α-syn pathology in neurodegenerative disease from the perspective of proteopathic α-syn seeds, including how these seeds are processed by cells as well as their effects on cellular function. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1038/s41374-019-0195-zDOI Listing
February 2019

The neutrophil-to-lymphocyte and monocyte-to-lymphocyte ratios are independently associated with neurological disability and brain atrophy in multiple sclerosis.

BMC Neurol 2019 Feb 12;19(1):23. Epub 2019 Feb 12.

Department of Neurology, Harvard Medical School, Boston, MA, USA.

Background: Serum hematological indices such as the neutrophil-lymphocyte ratio (NLR) or monocyte-lymphocyte ratio (MLR) have been used as biomarkers of pathogenic inflammation and prognostication in multiple areas of medicine; recent evidence shows correlation with psychological parameters as well.

Objectives/aims: To characterize clinical, neuroimaging, and psycho-neuro-immunological associations with NLR and MLR in persons with multiple sclerosis (MS).

Methods: We identified a large cohort of clinically well-defined patients from our longitudinal database that included MS-related outcomes, disease-modifying therapy, patient-reported outcome (PRO) measures, and quantified cerebral MRI at 1. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1186/s12883-019-1245-2DOI Listing
February 2019

Dopamine transporter imaging for the diagnosis of multiple system atrophy cerebellar type.

Parkinsonism Relat Disord 2019 Feb 5. Epub 2019 Feb 5.

Service de Neurologie, CRMR Atrophie Multisystématisée, CHU Bordeaux, 33000, Bordeaux, France; Univ. de Bordeaux, Institut des Maladies Neurodégénératives, UMR 5293, 33000, Bordeaux, France; CNRS, Institut des Maladies Neurodégénératives, UMR 5293, 33000, Bordeaux, France; Dept. of Medicine, University of Otago, Christchurch, and New Zealand Brain Research Institute, Christchurch, New Zealand. Electronic address:

Introduction: The added value of dopamine transporter SPECT (DAT-SPECT) for the diagnosis of "possible" multiple system atrophy of the cerebellar type (MSA-C) remains unknown.

Methods: We reviewed retrospectively the charts of 128 consecutive patients with a clinical diagnosis of MSA-C who were seen between 2007 and 2016 at the French Reference Center for MSA. The main objective was to evaluate the proportion of patients for whom the diagnosis of "possible" MSA-C was made because of a positive DAT-SPECT. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.parkreldis.2019.02.006DOI Listing
February 2019

Frontal lobe function, behavioral changes and quality of life in patients with multiple system atrophy.

Restor Neurol Neurosci 2019 Feb 5. Epub 2019 Feb 5.

Department of Neurology and National Clinical Research Center for Geriatrics (West China Hospital), West China Hospital, Sichuan University, Chengdu, Sichuan, China.

Background: Cognitive impairment is an important and common symptom in patients with multiple system atrophy (MSA).

Objective: The objective of the study was to explore the potential relationships among frontal lobe function, behavioral changes and quality of life (QoL) in patients with MSA.

Methods: A total of 203 MSA patients were enrolled and evaluated using the Frontal Assessment Battery (FAB), the Frontal Behavioral Inventory (FBI) and the Parkinson's disease Questionnaire-39 item version (PDQ-39). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3233/RNN-180862DOI Listing
February 2019

Multimodal MRI evaluation of parkinsonian limbic pathologies.

Neurobiol Aging 2019 Jan 16;76:194-200. Epub 2019 Jan 16.

Department of Neurology, Pennsylvania State University-Milton S. Hershey Medical Center, Hershey, PA, USA; Department of Pharmacology, Pennsylvania State University-Milton S. Hershey Medical Center, Hershey, PA, USA; Department of Radiology, Pennsylvania State University-Milton S. Hershey Medical Center, Hershey, PA, USA; Department of Neurosurgery, Pennsylvania State University-Milton S. Hershey Medical Center, Hershey, PA, USA; Department of Kinesiology, Penn State University-Milton S. Hershey Medical Center, Hershey, PA, USA. Electronic address:

Previous multimodal magnetic resonance imaging (MRI) studies of parkinsonian syndromes have focused primarily on motor-related basal ganglia structures. The present study investigated MRI changes in nonmotor-related limbic structures in 35 Parkinson's disease, 16 multiple system atrophy parkinsonian subtype, 17 progressive supranuclear palsy, and 37 control subjects. Mean diffusivity (MD), fractional anisotropy, transverse relaxation rate (R2*), quantitative susceptibility mapping, and volume measurements were obtained from the amygdala, hippocampus, and nucleus accumbens (NAc) to examine differences between groups and to test for associations with clinical scores. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.neurobiolaging.2019.01.004DOI Listing
January 2019
1 Read

Dissecting α-synuclein inclusion pathology diversity in multiple system atrophy: implications for the prion-like transmission hypothesis.

Lab Invest 2019 Feb 8. Epub 2019 Feb 8.

Department of Pathology, University of Florida, Gainesville, FL, 32610, USA.

Synucleinopathies are a group of neurodegenerative diseases characterized by the accumulation of insoluble, aggregated α-synuclein (αS) pathological inclusions. Multiple system atrophy (MSA) presents with extensive oligodendroglial αS pathology and additional more limited neuronal inclusions while most of the other synucleinopathies, such as Parkinson's disease and dementia with Lewy bodies (DLB), develop αS pathology primarily in neuronal cell populations. αS biochemical alterations specific to MSA have been described but thorough examination of these unique and disease-specific protein deposits is further warranted especially given recent findings implicating the prion-like nature of synucleinopathies perhaps with distinct strain-like properties. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1038/s41374-019-0198-9DOI Listing
February 2019
1 Read

Droxidopa for the treatment of neurogenic orthostatic hypotension in neurodegenerative diseases.

Expert Opin Pharmacother 2019 Feb 7:1-11. Epub 2019 Feb 7.

c Services de Pharmacologie Clinique et Neurosciences, Centre d'Investigation Clinique CIC 1436, NS-Park/FCRIN Network, NeuroToul COEN Center , Université de Toulouse UPS, CHU de Toulouse, INSERM , Toulouse , France.

Introduction: L-threo-3,4-dihydroxyphenylserine (droxidopa), a pro-drug metabolized to norepinephrine in nerve endings and other tissues, has been commercially available in Japan since 1989 for treating orthostatic hypotension symptoms in Parkinson's disease (PD) patients with a Hoehn & Yahr stage III rating, as well as patients with Multiple System Atrophy (MSA), familial amyloid polyneuropathy, and hemodialysis. Recently, the FDA has approved its use in symptomatic neurogenic orthostatic hypotension (NOH). Areas covered: The authors review the effects of droxidopa in NOH with a focus on the neurodegenerative diseases PD, MSA, and pure autonomic failure (PAF). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1080/14656566.2019.1574746DOI Listing
February 2019
1 Read

Therapeutic delivery: industry update covering October 2018.

Authors:
Iain Simpson

Ther Deliv 2019 Feb;10(2):91-97

Consultant, Cambridge, UK.

This industry update covers the period from 1 to 31 October 2018 and is based on information sourced from company press releases, scientific literature, patents and various news websites. With the expiry in Europe of AbbVie's (IL, USA) principal patent on Humira this month, the first biosimilar versions of the drug have been launched. AstraZeneca (Cambridge, UK) announced that is has out-licensed two none core drugs to Grunenthal (Aachen, German), while Pfizer (NY, USA) announced the creation of a new company, set up in collaboration with Bain Capital (MA, USA) to exploit Pfizer's clinical and preclinical assets in the neuroscience field. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.4155/tde-2018-0071DOI Listing
February 2019

Widespread microglial activation in multiple system atrophy.

Mov Disord 2019 Feb 6. Epub 2019 Feb 6.

Departments of Nulcear Medicine and Geriatric Medicine, University Hospital Essen, Germany.

Background: The pattern and role of microglial activation in multiple system atrophy is largely unclear. The objective of this study was to use [ C](R)-PK11195 PET to determine the extent and correlation of activated microglia with clinical parameters in MSA patients.

Methods: Fourteen patients with the parkinsonian phenotype of MSA (MSA-P) with a mean disease duration of 2. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/mds.27620DOI Listing
February 2019
1 Read

The Diagnostic Scope of Sensor-Based Gait Analysis in Atypical Parkinsonism: Further Observations.

Front Neurol 2019 22;10. Epub 2019 Jan 22.

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

Differentiating idiopathic Parkinson's disease (IPD) from atypical Parkinsonian disorders (APD) is challenging, especially in early disease stages. Postural instability and gait difficulty (PIGD) are substantial motor impairments of IPD and APD. Clinical evidence implies that patients with APD have larger PIGD impairment than IPD patients. Read More

View Article

Download full-text PDF

Source
https://www.frontiersin.org/article/10.3389/fneur.2019.00005
Publisher Site
http://dx.doi.org/10.3389/fneur.2019.00005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6349719PMC
January 2019
2 Reads

Wide distribution of alpha-synuclein oligomers in multiple system atrophy brain detected by proximity ligation.

Acta Neuropathol 2019 Feb 5. Epub 2019 Feb 5.

Division of Neurology/Molecular Brain Science, Kobe University Graduate School of Medicine, 7-5-1, Kusunoki-cho, Chuo-ku, Kobe, 650-0017, Japan.

Multiple system atrophy (MSA) is a fatal adult-onset neurodegenerative disease that is characterized by varying degrees of cerebellar dysfunction and Parkinsonism. The neuropathological hallmark of MSA is alpha-synuclein (AS)-positive glial cytoplasmic inclusions (GCIs). Although severe neuronal loss (NL) is also observed in MSA, neuronal inclusions (NIs) are rare compared to GCIs, such that the pathological mechanism of NL in MSA is unclear. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00401-019-01961-wDOI Listing
February 2019

Moving forward the in vivo diagnosis of the synucleinopathies.

Authors:
Dolores Vilas

Clin Auton Res 2019 Feb 5. Epub 2019 Feb 5.

Movement Disorders Unit, Neurology Service, Hospital Universitari Germans Trias I Pujol, Badalona, Barcelona, Spain.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s10286-019-00595-yDOI Listing
February 2019
2 Reads

Non-aneurysmal Subarachnoid Hemorrhaging: A Rare Cause of Death in a Patient with Multiple System Atrophy.

Intern Med 2019 Feb 1. Epub 2019 Feb 1.

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

Sudden death as a result of multiple system atrophy (MSA) is usually attributed to vocal cord paralysis or disruption of breathing owing to the degeneration of the brainstem respiratory centers. However, the exact pathophysiology of sudden death is still unclear. In addition, specific causes of sudden death are not always investigated by an autopsy. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.2169/internalmedicine.1567-18DOI Listing
February 2019
2 Reads

Self-reported urinary impairment identifies 'fast progressors' in terms of neuronal loss in multiple system atrophy.

Auton Neurosci 2019 Mar 14;217:1-6. Epub 2018 Dec 14.

Facultad de Medicina, Universidad de Chile, Santos Dummont 999, Santiago, Chile.

Introduction: MSA is an adult-onset, sporadic, progressive parkinsonian syndrome characterised by the presence of akinesia, cerebellar dysfunction, autonomic failure and pyramidal signs. Annualized-whole-brain atrophy rate (a-WBAR) is an informative way to quantify disease progression. In this longitudinal work we investigate the correlations of a-WBAR with clinical scales for motor impairment, autonomic disability and cognitive decline in MSA and explore how atrophy progresses within the brain. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.autneu.2018.12.003DOI Listing
March 2019
2 Reads

Early perfusion and dopamine transporter imaging using F-FP-CIT PET/CT in patients with parkinsonism.

Am J Nucl Med Mol Imaging 2018 20;8(6):360-372. Epub 2018 Dec 20.

Department of Nuclear Medicine, Kyungpook National University Hospital Daegu, Republic of Korea.

Combined use of F-N-(3-fluoropropyl)-2β-carboxymethoxy-3β-(4-iodophenyl)nortropane (FP-CIT) for dopamine transporter imaging and F-fludeoxyglucose (FDG) for glucose metabolism shows good diagnostic performance for differential diagnosis of Parkinson disease (PD) and Parkinson plus syndrome (multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration, and dementia with Lewy bodies). A recent study showed that F-FP-CIT positron emission tomography (PET) with early perfusion imaging is useful for the differential diagnosis of PD and Parkinson plus syndrome with lower radiation exposure, time, and cost. In this review, we summarize the advantages of using F-FP-CIT PET for perfusion and dopamine transporter imaging, as well as clinical features useful for the differential diagnosis of PD and Parkinson plus syndrome. Read More

View Article

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6334208PMC
December 2018
2 Reads

Multiple system atrophy prions retain strain specificity after serial propagation in two different Tg(SNCA*A53T) mouse lines.

Acta Neuropathol 2019 Jan 28. Epub 2019 Jan 28.

Institute for Neurodegenerative Diseases, Weill Institute for Neurosciences, University of California, San Francisco, CA, USA.

Previously, we reported that intracranial inoculation of brain homogenate from multiple system atrophy (MSA) patient samples produces neurological disease in the transgenic (Tg) mouse model TgM83, which uses the prion protein promoter to express human α-synuclein harboring the A53T mutation found in familial Parkinson's disease (PD). In our studies, we inoculated MSA and control patient samples into Tg mice constructed using a P1 artificial chromosome to express wild-type (WT), A30P, and A53T human α-synuclein on a mouse α-synuclein knockout background [Tg(SNCA)Nbm, Tg(SNCA*A30P)Nbm, and Tg(SNCA*A53T)Nbm]. In contrast to studies using TgM83 mice, motor deficits were not observed by 330-400 days in any of the Tg(SNCA)Nbm mice after inoculation with MSA brain homogenates. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00401-019-01959-4DOI Listing
January 2019

Continuous glucose monitoring can disclose glucose fluctuation in advanced Parkinsonian syndromes.

Authors:
Hiroyuki Todo

Neurol Int 2018 Dec 20;10(4):7921. Epub 2018 Dec 20.

Department of Neurology, National Hospital Organization Hyogo-Chuo National Hospital, Sanda, Japan.

Continuous glucose monitoring (CGM) is a method to examine glucose concentration in subcutaneous interstitial fluid sequentially. CGM can disclose glucose fluctuation (GF), which can be unrecognized in routine blood tests. A limited number of studies suggest advanced Parkinsonian syndromes (PS) is at risk of GF, however, the report of CGM in PS is scarce. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.4081/ni.2018.7921DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6322050PMC
December 2018
1 Read

Analysis of α-Synuclein Pathology in PINK1 Knockout Rat Brains.

Front Neurosci 2018 9;12:1034. Epub 2019 Jan 9.

Center for Neurodegeneration and Experimental Therapeutics, Department of Neurology, The University of Alabama at Birmingham, Birmingham, AL, United States.

Mutations in PTEN induced kinase 1 (PINK1) cause autosomal recessive Parkinson's disease (PD). The main pathological hallmarks of PD are loss of dopaminergic neurons in the substantia nigra pars compacta and the formation of protein aggregates containing α-synuclein. Previous studies of PINK1 knockout (PINK1-/-) rats have reported mitochondrial dysfunction, locomotor behavioral deficits, loss of neurons in the substantia nigra and α-synuclein aggregates in various brain regions. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3389/fnins.2018.01034DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6333903PMC
January 2019
1 Read

Altered Functional Connectivity of Cerebello-Cortical Circuit in Multiple System Atrophy (Cerebellar-Type).

Front Neurosci 2018 4;12:996. Epub 2019 Jan 4.

Department of Neurology, Dongfang Hospital, Beijing University of Chinese Medicine, Beijing, China.

Multiple system atrophy (MSA) is regarded as a progressive neurodegenerative disease mainly divided into MSA-p type with Parkinsonism and MSA-c type with cerebellar ataxia as the main symptom. However, its neural mechanism is still unclear. In this study, we only focus on the MSA-c type. Read More

View Article

Download full-text PDF

Source
https://www.frontiersin.org/article/10.3389/fnins.2018.00996
Publisher Site
http://dx.doi.org/10.3389/fnins.2018.00996DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6328464PMC
January 2019
3 Reads

Hydroxyfasudil alleviates demyelination through the inhibition of MOG antibody and microglia activation in cuprizone mouse model.

Clin Immunol 2019 Jan 17. Epub 2019 Jan 17.

The First Clinical College, Shanxi Medical University, Taiyuan 030001, China; 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 Traditional Chinese Medicine, Taiyuan 030024, China; Institute of Brain Science, Shanxi Datong University, Datong 037009, China. Electronic address:

Multiple sclerosis (MS) is an immune-mediated demyelinating disease of the central nervous system characterized by oligodendrocyte loss and progressive neurodegeneration. The cuprizone (CPZ)-induced demyelination is widely used to investigate the demyelination/remyelination. Here, we explored the therapeutic effects of Hydroxyfasudil (HF), an active metabolite of Fasudil, in CPZ model. Read More

View Article

Download full-text PDF

Source
https://linkinghub.elsevier.com/retrieve/pii/S15216616183046
Publisher Site
http://dx.doi.org/10.1016/j.clim.2019.01.006DOI Listing
January 2019
5 Reads

Differentiation Between Multiple System Atrophy and Other Spinocerebellar Degenerations Using Diffusion Kurtosis Imaging.

Acad Radiol 2019 Jan 15. Epub 2019 Jan 15.

Division of Ultrahigh Field MRI, Institute for Biomedical Sciences, Iwate Medical University, 2-1-1 Nishitokuta, Yahaba, Iwate, Japan.

Rationale And Objective: Differentiation between multiple system atrophy (MSA) and other spinocerebellar degenerations showing cerebellar ataxia is often difficult. Hence, we investigated whether magnetic resonance diffusion kurtosis imaging (DKI) could detect pathological changes that occur in these patients and be used for differential diagnosis.

Methods: Thirty-six subjects (12 patients with MSA accompanied by predominant cerebellar ataxia [MSA-C], 10 patients with spinocerebellar ataxias [SCAs] or sporadic adult-onset ataxia of unknown etiology [SAOA], and 14 healthy controls) were examined using 1. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.acra.2018.12.015DOI Listing
January 2019
1 Read

Elevated serum ferritin level as a predictor of reduced survival in patients with sporadic amyotrophic lateral sclerosis in China: a retrospective study.

Amyotroph Lateral Scler Frontotemporal Degener 2019 Jan 17:1-6. Epub 2019 Jan 17.

a College of Medicine , Nankai University , Tianjin , China.

Objective: The objective of this study was to compare iron metabolic variables in the serum and cerebrospinal fluid (CSF) of patients with sporadic amyotrophic lateral sclerosis (sALS) with those of patients with multiple system atrophy (MSA) and control subjects. We also assessed the correlations of these variables with sALS progression and severity and estimated their roles in predicting prognosis.

Methods: We retrospectively collected iron metabolic parameters, including serum levels of iron, ferritin, transferrin levels and total iron binding capacity and the CSF level of ferritin, from 435 sALS patients, 176 MSA patients and 431 control subjects. Read More

View Article

Download full-text PDF

Source
https://www.tandfonline.com/doi/full/10.1080/21678421.2018.1
Publisher Site
http://dx.doi.org/10.1080/21678421.2018.1555599DOI Listing
January 2019
4 Reads
2.591 Impact Factor

Cell Responses to Extracellular α-Synuclein.

Molecules 2019 01 15;24(2). Epub 2019 Jan 15.

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

Synucleins are small naturally unfolded proteins involved in neurodegenerative diseases and cancer. The family contains three members: α-, β-, and -synuclein. α-Synuclein is the most thoroughly investigated because of its close association with Parkinson's disease (PD), dementia with Lewy bodies and multiple system atrophy. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3390/molecules24020305DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6359176PMC
January 2019
1 Read

Omega-3 multiple effects increasing glucocorticoid-induced muscle atrophy: autophagic, AMPK and UPS mechanisms.

Physiol Rep 2019 Jan;7(1):e13966

Department of Neurology, Faculdade de Medicina FMUSP, Universidade de Sao Paulo, SP, Brazil.

Muscle atrophy occurs in many conditions, including use of glucocorticoids. N-3 (omega-3) is widely consumed due its healthy properties; however, concomitant use with glucocorticoids can increase its side effects. We evaluated the influences of N-3 on glucocorticoid atrophy considering IGF-1, Myostatin, MEK/ERK, AMPK pathways besides the ubiquitin-proteasome system (UPS) and autophagic/lysosomal systems. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.14814/phy2.13966DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6333722PMC
January 2019

Neuroimaging of Rapid Eye Movement Sleep Behavior Disorder.

Int Rev Neurobiol 2019 8;144:185-210. Epub 2018 Nov 8.

Department of Psychology, Université du Québec à Montréal, Montreal, QC, Canada; Center for Advanced Research in Sleep Medicine, CIUSSS-NÎM-Hôpital du Sacré-Cœur de Montréal, Montreal, QC, Canada; Research Center, Institut universitaire de gériatrie de Montréal, Montreal, QC, Canada. Electronic address:

Idiopathic rapid eye movement sleep behavior disorder (iRBD) is a parasomnia characterized by the loss of muscle atonia and the presence of undesirable motor manifestations during rapid eye movement sleep. Research findings have shown that iRBD is a prodromal stage of synucleinopathies such as Parkinson's disease, dementia with Lewy bodies, and multiple system atrophy. A wide array of neuroimaging techniques have improved our understanding of the prodromal stage of these diseases in patients with iRBD, and identified potential biomarkers. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/bs.irn.2018.10.006DOI Listing
November 2018
1 Read

Occurrence of Stridor During Sleep in a Patient With Spinocerebellar Ataxia Type 17.

J Clin Sleep Med 2019 Jan 15;15(1):153-155. Epub 2019 Jan 15.

Department of Nuclear Medicine, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea.

Abstract: Autosomal dominant spinocerebellar ataxia type 17 (SCA17) is known to have various manifestations. Previously, ataxia and autonomic dysfunction have been reported with this disorder, making an overlap with multiple systemic atrophy (MSA). However, respiratory complications, especially stridor during sleep, have not yet been reported with SCA17, in contrast to MSA, in which stridor occurs during sleep and is associated with an increased risk of death. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.5664/jcsm.7592DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6329553PMC
January 2019
5 Reads

Spinal cord involvement in Lewy body-related α-synucleinopathies.

J Spinal Cord Med 2019 Jan 8:1-14. Epub 2019 Jan 8.

b Department of Neurology, Christian Doppler Klinik , Paracelsus Medical University , Salzburg , Austria.

Context: Lewy body (LB)-related α-synucleinopathy (LBAS) is the neuropathological hallmark of several neurodegenerative diseases such as Parkinson disease (PD), but it is also found in neurologically asymptomatic subjects. An abnormal accumulation of α-synuclein has been reported also in the spinal cord, but extent and significance of the spinal cord involvement are still poorly defined.

Objective: We aimed to review the studies addressing the spinal cord involvement of LBAS in healthy subjects and in patients with PD or other neurodegenerative diseases. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1080/10790268.2018.1557863DOI Listing
January 2019
1 Read
1.878 Impact Factor

Electrophysiological and clinical assessment of dysautonomia in multiple system atrophy (MSA) and progressive supranuclear palsy (PSP): a comparative study.

Neurol Neurochir Pol 2019 Jan 8. Epub 2019 Jan 8.

Department of Neurology, Medical University of Warsaw, 8 Kondratowicza str, 03-242 Warsaw, Poland.

Clinical Rationale For The Study: Autonomic nervous system (ANS) involvement in different parkinsonian syndromes has been frequently discussed. It is well established in multiple system atrophy (MSA), whereas it is less evident in progressive supranuclear palsy (PSP).

Aims Of The Study: The aims were to assess the presence and pattern of ANS involvement in MSA and PSP using noninvasive tests i. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.5603/PJNNS.a2019.0005DOI Listing
January 2019
1 Read

Distinct Incidence of Takotsubo Syndrome Between Amyotrophic Lateral Sclerosis and Synucleinopathies: A Cohort Study.

Front Neurol 2018 13;9:1099. Epub 2018 Dec 13.

Department of Clinical Neuroscience, Institute of Biomedical Sciences, Tokushima University Graduate School, Tokushima, Japan.

Takotsubo syndrome (TTS) is an acute cardiac syndrome characterized by regional left ventricular dysfunction with a peculiar circumferential pattern, which typically results in apical ballooning. Evidence indicates a pivotal role of catecholamines in TTS, and researchers have discussed multiple hypotheses on the etiology, including multivessel coronary spasm, myocardial stunning, excessive transient ventricular afterload, and cardiac sympathetic overactivity with local noradrenaline spillover. Although central nervous system disorders, such as stroke and epilepsy, are known to trigger TTS, the incidence and clinical features of TTS in neurodegenerative disorders are poorly understood. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3389/fneur.2018.01099DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6300466PMC
December 2018
2 Reads

A Meta-Analysis of α-Synuclein Multiplication in Familial Parkinsonism.

Front Neurol 2018 11;9:1021. Epub 2018 Dec 11.

Department of Medical Genetics, Centre for Applied Neurogenetics, University of British Columbia, Vancouver, BC, Canada.

Chronic alpha-synuclein () overexpression is a relatively homogenous and well-defined cause of parkinsonism and dementia. Parkinson's disease (PD), PD with dementia, dementia with Lewy bodies and multiple system atrophy all manifest in multiplication families. Herein we summarize genealogic, clinical and genetic data from 59 families (25 not previously published) with parkinsonism caused by multiplications. Read More

View Article

Download full-text PDF

Source
https://www.frontiersin.org/article/10.3389/fneur.2018.01021
Publisher Site
http://dx.doi.org/10.3389/fneur.2018.01021DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6297377PMC
December 2018
6 Reads

Hyperglycemia Induced by Chronic Restraint Stress in Mice Is Associated With Nucleus Tractus Solitarius Injury and Not Just the Direct Effect of Glucocorticoids.

Front Neurosci 2018 19;12:983. Epub 2018 Dec 19.

Department of Histology, Embryology and Neurobiology, West China School of Basic Medical Sciences & Forensic Medicine, Sichuan University, Chengdu, China.

Chronic restraint stress (CRS) can affect hypothalamic-pituitary-adrenal (HPA) axis activity and increase glucocorticoid levels. Glucocorticoids are stress hormones that regulate multiple aspects of energy homeostasis. Stress also impairs glucose tolerance. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3389/fnins.2018.00983DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6305899PMC
December 2018

Oxygen consumption rate for evaluation of COQ2 variants associated with multiple system atrophy.

Neurogenetics 2019 Jan 7. Epub 2019 Jan 7.

Department of Neurology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s10048-018-0563-7DOI Listing
January 2019
1 Read

Living in Promiscuity: The Multiple Partners of Alpha-Synuclein at the Synapse in Physiology and Pathology.

Int J Mol Sci 2019 Jan 2;20(1). Epub 2019 Jan 2.

Division of Pharmacology, Department of molecular and Translational Medicine, University of Brescia, Viale Europa 11, 25123, Brescia, Italy.

Alpha-synuclein (α-syn) is a small protein that, in neurons, localizes predominantly to presynaptic terminals. Due to elevated conformational plasticity, which can be affected by environmental factors, in addition to undergoing disorder-to-order transition upon interaction with different interactants, α-syn is counted among the intrinsically disordered proteins (IDPs) family. As with many other IDPs, α-syn is considered a hub protein. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3390/ijms20010141DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6337145PMC
January 2019
1 Read

Brain morphological alteration and cognitive dysfunction in multiple system atrophy.

Quant Imaging Med Surg 2018 Nov;8(10):1030-1038

Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing 100053, China.

Multiple system atrophy (MSA) is a progressive neurodegenerative disease in adults, manifesting various clinical symptoms including autonomic nerve dysfunction, Parkinson's syndrome, cerebellar ataxia, and pyramidal sign. The clinical diagnosis and classification of MSA are mainly dependent on motion and non-motion symptoms, such as autonomic nerve dysfunction. In addition, an increasing amount of clinical and pathological evidence has shown that about half of the MSA patients exhibit distinct types and levels of cognitive dysfunction. Read More

View Article

Download full-text PDF

Source
http://qims.amegroups.com/article/view/22382/21888
Publisher Site
http://dx.doi.org/10.21037/qims.2018.11.02DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6288059PMC
November 2018
3 Reads

Association of autonomic symptoms with disease progression and survival in progressive supranuclear palsy.

J Neurol Neurosurg Psychiatry 2018 Dec 31. Epub 2018 Dec 31.

Reta Lila Weston Institute of Neurological Studies, UCL Queen Square Institute of Neurology, London, UK

Background: Development of autonomic failure is associated with more rapid disease course and shorter survival in patients with Parkinson's disease and multiple system atrophy. However, autonomic symptoms have not been specifically assessed as a prognostic factor in progressive supranuclear palsy (PSP). We evaluated whether development of autonomic symptoms is associated with disease progression and survival in PSP. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1136/jnnp-2018-319374DOI Listing
December 2018
3 Reads

Motor and Nonmotor Symptoms of Parkinson's Disease: Antagonistic Pleiotropy Phenomena Derived from -Synuclein Evolvability?

Parkinsons Dis 2018 22;2018:5789424. Epub 2018 Nov 22.

Tokyo Metropolitan Institute of Medical Science, 2-1-6 Kamikitazawa, Setagaya-ku, Tokyo, Japan.

Lewy body diseases, such as Parkinson's disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA), are associated with a wide range of nonmotor symptoms (NMS), including cognitive impairment, depression and anxiety, sleep disorders, gastrointestinal symptoms, and autonomic failure. The reason why such diverse and disabling NMS have not been weeded out but have persisted across evolution is unknown. As such, one possibility would be that the NMS might be somehow beneficial during development and/or reproductive stages, a possibility consistent with our recent view as to the evolvability of amyloidogenic proteins (APs) such as -synuclein (S) and amyloid- (A) in the brain. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1155/2018/5789424DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6282124PMC
November 2018
1 Read

Similarities and differences in cerebellar grey matter volume and disrupted functional connectivity in idiopathic Parkinson's disease and multiple system atrophy.

Neuropsychologia 2019 Feb 24;124:125-132. Epub 2018 Dec 24.

The First Affiliated Hospital of China Medical University, Department of Radiology, China. Electronic address:

Objective: This study investigated the potential differences in changes to cerebellar grey matter volume (GMV) and functional connectivity (FC) and the association between them, which might elucidate cerebellar function in multiple system atrophy (MSA) and idiopathic Parkinson's disease (IPD).

Methods: Resting-state functional magnetic resonance imaging and three-dimensional T1 scans were obtained from 38 IPD, 62 MSA, and 59 healthy controls (HC). Seed-based connectivity analysis and voxel-based morphometry analysis were performed to assess the changes of cerebellar FC and GMV between the patient groups. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.neuropsychologia.2018.12.019DOI Listing
February 2019
1 Read

Young-onset multiple system atrophy.

Authors:
Kurt A Jellinger

Mov Disord 2018 Dec;33(12):1974-1975

Institute of Clinical Neurobiology, Vienna, Austria.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/mds.27567DOI Listing
December 2018

Unraveling gut microbiota in Parkinson's disease and atypical parkinsonism.

Mov Disord 2018 Dec 21. Epub 2018 Dec 21.

Parkinson Institute, Azienda Socio Sanitaria Territoriale (ASST) Gaetano Pini-CTO, Milan, Italy.

Background: Although several studies have suggested that abnormalities in gut microbiota may play a critical role in the pathogenesis of PD, data are still extremely heterogeneous.

Methods: 16S gene ribosomal RNA sequencing was performed on fecal samples of 350 individuals, subdivided into idiopathic PD (n = 193, of whom 39 were drug naïve) stratified by disease duration, PSP (n = 22), MSA (n = 22), and healthy controls (HC; n = 113). Several confounders were taken into account, including dietary habits. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/mds.27581DOI Listing
December 2018
9 Reads

Extracellular Interactions of Alpha-Synuclein in Multiple System Atrophy.

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

School of Medical Science, Griffith University, Gold Coast 4222, Australia.

Multiple system atrophy, characterized by atypical Parkinsonism, results from central nervous system (CNS) cell loss and dysfunction linked to aggregates of the normally pre-synaptic α-synuclein protein. Mostly cytoplasmic pathological α-synuclein inclusion bodies occur predominantly in oligodendrocytes in affected brain regions and there is evidence that α-synuclein released by neurons is taken up preferentially by oligodendrocytes. However, extracellular α-synuclein has also been shown to interact with other neural cell types, including astrocytes and microglia, as well as extracellular factors, mediating neuroinflammation, cell-to-cell spread and other aspects of pathogenesis. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3390/ijms19124129DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6320782PMC
December 2018
1 Read

The 'Hot Cross Bun' Sign Is Not Always Multiple System Atrophy: Etiologies of 11 Cases.

J Mov Disord 2019 Jan 19;12(1):27-30. Epub 2018 Dec 19.

Parkinson's Institute and Clinical Center, Sunnyvale, CA.

Objective: To clarify the specificity of the 'hot cross bun' sign (HCBS) for multiple system atrophy (MSA) in adult cerebellar ataxia or parkinsonism.

Methods: The radiologic information systems at an academic center and affiliated veterans' hospital were queried using the keywords 'hot cross bun,' 'pontocerebellar,' 'cruciate,' 'cruciform,' 'MSA,' 'multiple system atrophy,' and 'multisystem atrophy.' Scans were reviewed by a neurologist and neuroradiologist to identify the HCBS. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.14802/jmd.18031DOI Listing
January 2019
1 Read

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

J Chem Neuroanat 2019 Mar 14;96:41-48. 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

View Article

Download full-text PDF

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

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

Mov Disord 2019 Feb 17;34(2):246-254. 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

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/mds.27542DOI Listing
February 2019

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

View Article

Download full-text PDF

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

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

View Article

Download full-text PDF

Source
http://link.springer.com/10.1007/s00330-018-5874-4
Publisher Site
http://dx.doi.org/10.1007/s00330-018-5874-4DOI Listing
December 2018
12 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

View Article

Download full-text PDF

Source
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

View Article

Download full-text PDF

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