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atrophy multiple systems

Tomoyuki Miyamoto, Masayuki Miyamoto
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...
September 2018: Movement Disorders Clinical Practice
Reiji Muto, Yasuo Sugita, Seiya Momosaki, Yuriko Ito, Yoshiyuki Wakugawa, Koichi Ohshima
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. A 63-year-old woman who had undergone chemotherapy for non-Hodgkin lymphoma developed progressive dysarthria and cerebellar ataxia...
December 3, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
Dorian Sargent, Dominique Bétemps, Matthieu Drouyer, Jérémy Verchere, Damien Gaillard, Jean-Noël Arsac, Latifa Lakhdar, Anna Salvetti, Thierry Baron
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. M83 neonate or adult mice were injected with adeno-associated virus vectors carrying the human β-syn gene (AAVβ-syn) or green fluorescent protein gene (AAVGFP) using different injection sites...
December 3, 2018: Scientific Reports
Claudia Testa, Giovanna Calandra-Buonaura, Stefania Evangelisti, Giulia Giannini, Federica Provini, Stefano Ratti, Annagrazia Cecere, Lia Talozzi, David Neil Manners, Raffaele Lodi, Caterina Tonon, Pietro Cortelli
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. Differences in GM density between MSA patients with and without stridor and a sample of 22 matched healthy controls were evaluated with Voxel Based Morphometry protocol supplemented by a specific tool (SUIT) for analysing infratentorial structures...
November 17, 2018: Parkinsonism & related Disorders
Takuma Ohmichi, Masato Mitsuhashi, Harutsugu Tatebe, Takashi Kasai, Omar M Ali El-Agnaf, Takahiko Tokuda
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)...
November 20, 2018: Parkinsonism & related Disorders
Roongroj Bhidayasiri, Watchara Rattanachaisit, Onanong Phokaewvarangkul, Thien Thien Lim, Hubert H Fernandez
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...
November 5, 2018: Parkinsonism & related Disorders
Haiyan Yu, Xiaoling Yuan, Lifeng Liu, Tian Wang, Dianrong Gong
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. Human umbilical cord blood is the residue of blood in the placenta and umbilical cord after fetal delivery. It is the most abundant cell bank and its usage is not limited to treat hematological diseases. The researches about hUCB-MNC treatment on MSA are increasing gradually...
2018: American Journal of Clinical and Experimental Immunology
Tritia R Yamasaki, Brandon B Holmes, Jennifer L Furman, Dhruva D Dhavale, Bryant W Su, Eun-Suk Song, Nigel J Cairns, Paul T Kotzbauer, Marc I Diamond
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. Prion strains are aggregated conformers that stably propagate in vivo and cause disease with defined incubation times and patterns of neuropathology...
November 26, 2018: Journal of Biological Chemistry
Mario Mascalchi, Alessandra Vella
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...
2018: International Review of Neurobiology
Miaad Bader, Yazhou Li, Daniela Lecca, Vardit Rubovitch, David Tweedie, Elliot Glotfelty, Lital Rachmany, Hee Kyung Kim, Ho-Il Choi, Barry J Hoffer, Chaim G Pick, Nigel H Greig, Dong Seok Kim
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. In this study, the pharmacokinetics and efficacy of PT302, a clinically available sustained-release Exenatide formulation (SR-Exenatide) were evaluated in a concussive mild (m)TBI mouse model...
November 21, 2018: Neurobiology of Disease
Viorica Chelban, Martina Bocchetta, Sara Hassanein, Nourelhoda A Haridy, Henry Houlden, Jonathan D Rohrer
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...
November 20, 2018: Journal of Neurology
Antonio Heras-Garvin, Daniel Weckbecker, Sergey Ryazanov, Andrei Leonov, Christian Griesinger, Armin Giese, Gregor K Wenning, Nadia Stefanova
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. At present, there is no effective disease-modifying therapy. Previous experiments have shown that the aggregation inhibitor, anle138b, reduces neurodegeneration and behavioral deficits in mouse models of other proteinopathies...
November 19, 2018: Movement Disorders: Official Journal of the Movement Disorder Society
Marek Sąsiadek, Katarzyna Katulska, Agata Majos, Małgorzata Siger, Marcin Hartel, Alicja Kalinowska, Jerzy Walecki
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...
October 4, 2018: Neurologia i Neurochirurgia Polska
A B González Escobar, A Alberte González, A Ibañez García, M Lorenzo Soto, S Gismero Moreno
Sclerochoroidal calcification (SCC) is uncommon and benign. It is usually detected in a routine examination, finding multiple yellow-white lesions in the upper temporal region of the retina in middle-aged and elderly men. A case report is presented of a 79 year-old male patient, who during a routine examination with a pseudoexfoliative glaucoma in the right eye, as well as raised white-yellow subretinal lesions in the upper temporal region in both eyes. After establishing hypotensive treatment and performing autofluorescence, optical coherence tomography (OCT), ultrasound, ocular computed tomography (CT) and complete laboratory analysis, idiopathic SCC was diagnosed...
November 13, 2018: Archivos de la Sociedad Española de Oftalmología
Ruo-Xuan Sui, Qiang Miao, Jing Wang, Qing Wang, Li-Juan Song, Jing-Wen Yu, Liang Cao, Wei Xiao, Bao-Guo Xiao, Cun-Gen Ma
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. The results demonstrate that Bilobalide improved behavioral abnormality and promoted remyelination in the corpus callosum by using Luxol Fast Blue, Black Gold II and myelin basic protein (MBP) staining...
November 13, 2018: International Immunopharmacology
Alana Hoffmann, Benjamin Ettle, Kristina Battis, Simone Reiprich, Johannes C M Schlachetzki, Eliezer Masliah, Michael Wegner, Tanja Kuhlmann, Markus J Riemenschneider, Jürgen Winkler
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. Here, we demonstrate MSA-associated neuroinflammation being restricted to myeloid cells and tightly linked to oligodendroglial α-syncleinopathy. In human putaminal post-mortem tissue of MSA patients, neuroinflammation was observed in white matter regions only...
November 16, 2018: Brain Pathology
Nicole Ziliotto, Robert Zivadinov, Dejan Jakimovski, Marcello Baroni, Veronica Tisato, Paola Secchiero, Niels Bergsland, Deepa P Ramasamy, Bianca Weinstock-Guttman, Francesco Bernardi, Murali Ramanathan, Giovanna Marchetti
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...
October 28, 2018: Journal of the Neurological Sciences
Roongroj Bhidayasiri, Jirada Sringean, Stephen G Reich, Carlo Colosimo
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)...
October 6, 2018: Parkinsonism & related Disorders
Zheyu Xu, Javier Arbizu, Nicola Pavese
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...
2018: International Review of Neurobiology
Federica Agosta, Elisabetta Sarasso, Massimo Filippi
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...
2018: International Review of Neurobiology
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