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demyelinating diseases

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https://www.readbyqxmd.com/read/28086920/absence-of-system-xc-on-immune-cells-invading-the-central-nervous-system-alleviates-experimental-autoimmune-encephalitis
#1
Ellen Merckx, Giulia Albertini, Magdalena Paterka, Cathy Jensen, Philipp Albrecht, Michael Dietrich, Joeri Van Liefferinge, Eduard Bentea, Lise Verbruggen, Thomas Demuyser, Lauren Deneyer, Jan Lewerenz, Geert van Loo, Jacques De Keyser, Hideyo Sato, Pamela Maher, Axel Methner, Ann Massie
BACKGROUND: Multiple sclerosis (MS) is an autoimmune demyelinating disease that affects the central nervous system (CNS), leading to neurodegeneration and chronic disability. Accumulating evidence points to a key role for neuroinflammation, oxidative stress, and excitotoxicity in this degenerative process. System xc(-) or the cystine/glutamate antiporter could tie these pathological mechanisms together: its activity is enhanced by reactive oxygen species and inflammatory stimuli, and its enhancement might lead to the release of toxic amounts of glutamate, thereby triggering excitotoxicity and neurodegeneration...
January 13, 2017: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/28079948/induction-of-antibodies-directed-against-branched-core-o-mannosyl-glycopeptides-selectivity-complimentary-to-the-cona-lectin
#2
Jin Yu, Oliver C Grant, Christian Pett, Sabine Strahl, Robert J Woods, Ulrika Westerlind
Mammalian protein O-mannosylation comprise a group of post-translational modifications (PTMs) involved in muscle and brain development. Despite the enormous progress made, to date the biological impact of the mammalian O-mannosyl glycoproteome remains largely unknown. Tools are still needed to investigate the structure, role and abundance of O-mannosyl glycans. While O-mannosyl branching has been shown to be of relevance in integrin-dependent cell-migration, and also plays a role in demyelinating diseases, a broader understanding of the biological roles of branched O-mannosyl glycans is lacking in part due to the paucity of detection tools...
January 12, 2017: Chemistry: a European Journal
https://www.readbyqxmd.com/read/28077839/atypical-trigeminal-neuralgia-a-rare-neurological-manifestation-of-systemic-lupus-erythematosus
#3
Viki Kumar, Jaspinder Kaur, Pallavi Pothuri, Sahiba Bandagi
BACKGROUND Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder of unknown etiology which can present at any age with symptoms of mucocutaneous, musculoskeletal, renal, central nervous system, and nonspecific clinical pictures making the disease a "master of mimicry". CASE REPORT A 53-year-old female, who was recently diagnosed with SLE, presented with right-sided sharp and electric shock-like facial pain starting at the side of her right nostril and traveling down the naso-labial fold and then back to the angle of the jaw, mostly in the region of V2-V3 distribution with no radiation beyond trigeminal distribution...
January 12, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28070468/multiple-myeloma-associated-chronic-inflammatory-demyelinating-polyradiculoneuropathy-the-importance-of-continued-surveillance
#4
Adebayo A Fasanya, Michael F Loncharich, Viral Gandhi, Sandeep Rana, Marvin Balaan
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune disease presenting with weakness and numbness in a remitting or chronic progressive course. It is known to have several clinical presentations and several associated diseases. CIDP has been associated with multiple myeloma, monoclonal gammopathy of undetermined significance (MGUS), and other paraproteinemias. We present a case of refractory CIDP in which the initial workup for multiple myeloma was negative, and multiple myeloma was then diagnosed two and half years later...
November 28, 2016: Curēus
https://www.readbyqxmd.com/read/28069926/creatine-enhances-mitochondrial-mediated-oligodendrocyte-survival-following-demyelinating-injury
#5
Kelly A Chamberlain, Kristen S Chapey, Sonia E Nanescu, Jeffrey K Huang
: Chronic oligodendrocyte loss, which occurs in the demyelinating disorder multiple sclerosis (MS), contributes to axonal dysfunction and neurodegeneration. Current therapies are able to reduce MS severity, but do not prevent transition into the progressive phase of the disease, which is characterized by chronic neurodegeneration. Therefore, pharmacological compounds that promote oligodendrocyte survival could be beneficial for neuroprotection in MS. Here, we investigated the role of creatine, an organic acid involved in ATP buffering, in oligodendrocyte function...
January 9, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28069137/mitochondria-in-multiple-sclerosis-molecular-mechanisms-of-pathogenesis
#6
S Patergnani, V Fossati, M Bonora, C Giorgi, S Marchi, S Missiroli, T Rusielewicz, M R Wieckowski, P Pinton
Mitochondria, the organelles that function as the powerhouse of the cell, have been increasingly linked to the pathogenesis of many neurological disorders, including multiple sclerosis (MS). MS is a chronic inflammatory demyelinating disease of the central nervous system (CNS) and a leading cause of neurological disability in young adults in the western world. Its etiology remains unknown, and while the inflammatory component of MS has been heavily investigated and targeted for therapeutic intervention, the failure of remyelination and the process of axonal degeneration are still poorly understood...
2017: International Review of Cell and Molecular Biology
https://www.readbyqxmd.com/read/28068915/comparison-of-clinical-features-and-prognostic-factors-in-hiv-negative-adults-with-cryptococcal-meningitis-and-tuberculous-meningitis-a-retrospective-study
#7
Junyan Qu, Taoyou Zhou, Cejun Zhong, Rong Deng, Xiaoju Lü
BACKGROUND: The incidence of cryptococcal meningitis (CM) and tuberculous meningitis (TBM) have gradually increased in recent years. These two types of meningitis are easily misdiagnosed which leads to a poor prognosis. In this study we compared differences of clinical features and prognostic factors in non-HIV adults with CM and TBM. METHODS: We retrospectively reviewed the medical records of CM and TBM patients from January 2008 to December 2015 in our university hospital in China...
January 10, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28065579/roles-of-regulatory-t-cells-and-il-10-in-virus-induced-demyelination
#8
REVIEW
Stanley Perlman, Jingxian Zhao
Neurotropic viruses are important causes of morbidity and mortality in human populations. Some of these viruses preferentially infect oligodendrocytes in the white matter, causing either direct lysis of infected cells, or more commonly myelin damage as a consequence of the host immune response to the virus. Virus-induced demyelination has similarities to the human disease multiple sclerosis. To study this disease process in experimental animals, mice are infected, most commonly, with neurotropic strains of mouse hepatitis virus, a coronavirus or Theiler's murine encephalomyelitis, a picornavirus...
January 4, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28060988/-neuromyelitis-optica-presenting-concomitantly-with-systemic-lupus-erythematosus-report-of-one-case
#9
Felipe Suárez H, Daniela Urrutia E, Felipe Canales P, Camila Gutiérrez O
Neuromyelitis optica (NMO) is a severe demyelinating disease of the central nervous system, which preferentially attacks the optic nerve and spinal cord. It is associated with antibodies against aquaporin 4. Morbidity and mortality are higher than in multiple sclerosis and its treatment focuses on immunosuppressive drugs. Immunomodulators are contraindicated. We report a previously healthy 35-year-old man, presenting with NMO concomitantly with systemic lupus erythematosus. His evolution was torpid with three outbreaks in the 10 months after the diagnosis, requiring a first-line therapy with methylprednisolone and cyclophosphamide and then a second-line therapy with rituximab...
September 2016: Revista Médica de Chile
https://www.readbyqxmd.com/read/28059798/sporadic-cases-with-novel-mutations-and-pedigree-in-hereditary-leukoencephalopathy-with-axonal-spheroids
#10
Liyong Wu, Jia Liu, Longze Sha, Xianling Wang, Jieying Li, Jing Dong, Jianping Jia
Hereditary diffuse leukoencephalopathy with spheroids (HDLS) is an autosomal dominant hereditary disease, featured by cerebral white matter degeneration with demyelination and axonal spheroids. We collected three gene-confirmed HDLS cases in our neurodegenerative clinic. Two HDLS cases were sporadic with novel mutations, while another case had a family history with previously described mutations. All three cases suffered memory problems with white matter lesions and pyramid signs. No obvious clinical differences were observed between sporadic and familial HDLS cases...
December 3, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28056966/pseudorabies-virus-infection-aujeszky-s-disease-in-an-iberian-lynx-lynx-pardinus-in-spain-a-case-report
#11
A Javier Masot, María Gil, David Risco, Olga M Jiménez, José I Núñez, Eloy Redondo
BACKGROUND: The only natural hosts of Pseudorabies virus (PRV) are members of the family Suidae (Sus scrofa scrofa). In species other than suids infection is normally fatal. In these mammals, including carnivores, PRV typically causes serious neurologic disease. The endangered Iberian lynx (Lynx pardinus) is a wild feline endemic to south-western Europe (Iberian Peninsula). The Iberian lynx was found to be the world's most endangered felid species in 2002. In wild felines, PRV infection has only been previously reported once in a Florida panther in 1994...
January 5, 2017: BMC Veterinary Research
https://www.readbyqxmd.com/read/28055081/soluble-cd27-levels-in-cerebrospinal-fluid-as-a-prognostic-biomarker-in-clinically-isolated-syndrome
#12
Roos M van der Vuurst de Vries, Julia Y Mescheriakova, Tessel F Runia, Naghmeh Jafari, Theodora A M Siepman, Rogier Q Hintzen
Importance: There is a growing number of therapies that could be administered after the first symptom of central nervous system demyelination. These drugs can delay multiple sclerosis (MS) diagnosis and slow down future disability. However, treatment of patients with benign course may not be needed; therefore, there is a need for biomarkers to predict long-term prognosis in patients with clinically isolated syndrome (CIS). Objective: To investigate whether the T-cell activation marker soluble CD27 (sCD27) measured in cerebrospinal fluid of patients at time of a first attack is associated with a subsequent diagnosis of MS and a higher relapse rate...
January 3, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28052249/opposing-functions-of-microglial-and-macrophagic-tnfr2-in-the-pathogenesis-of-experimental-autoimmune-encephalomyelitis
#13
Han Gao, Matt C Danzi, Claire S Choi, Mehran Taherian, Camilla Dalby-Hansen, Ditte G Ellman, Pernille M Madsen, John L Bixby, Vance P Lemmon, Kate L Lambertsen, Roberta Brambilla
In multiple sclerosis (MS), soluble tumor necrosis factor (TNF) is detrimental via activation of TNF receptor 1 (TNFR1), whereas transmembrane TNF is beneficial primarily by activating TNF receptor 2 (TNFR2). Here, we investigate the role of TNFR2 in microglia and monocytes/macrophages in experimental autoimmune encephalomyelitis (EAE), a model of MS, by cell-specific gene targeting. We show that TNFR2 ablation in microglia leads to early onset of EAE with increased leukocyte infiltration, T cell activation, and demyelination in the central nervous system (CNS)...
January 3, 2017: Cell Reports
https://www.readbyqxmd.com/read/28049030/pregabalin-enhances-myelin-repair-and-attenuates-glial-activation-in-lysolecithin-induced-demyelination-model-of-rat-optic-chiasm
#14
Danyal Daneshdoust, Mohsen Khalili-Fomeshi, Maryam Ghasemi-Kasman, Davoud Ghorbanian, Mona Hashemian, Mohammad Gholami, Aliakbar Moghadamnia, Amir Shojaei
Multiple sclerosis (MS) is an autoimmune disease in which more than 70% of patients experience visual disturbance as the earliest symptoms. Lysolecithin (LPC)-induced focal demyelination model has been developed to evaluate the effects of different therapies on myelin repair improvement. In this study, the effects of pregabalin administration on myelin repair and glial activation were investigated. Local demyelination was induced by administration of LPC (1%, 2μL) into the rat optic chiasm. Rats underwent daily injection of pregabalin (30mg/kg, i...
January 1, 2017: Neuroscience
https://www.readbyqxmd.com/read/28042737/thyroid-hormone-level-is-associated-with-the-frequency-and-severity-of-guillain-barr%C3%A3-syndrome
#15
Yuanyuan Huang, Zhaojian Ying, Zhibo Chen, Weiwei Xiang, Zhongqian Su, Weiwei Quan, Yiyun Weng, Xu Zhang
OBJECTIVE: Guillain-Barré syndrome (GBS) is a neurodegenerative and inflammatory demyelination disorder, and oxidative stress is concerned with the pathogenesis of the disease. Also we found that thyroid hormone level is correlated to the oxidative and antioxidant status in previous studies. Our study was aimed to find the possible relationship between the frequency and severity of GBS and thyroid hormone levels. MATERIALS AND METHODS: We measured serum levels of thyroid stimulating hormone (TSH), free thyroxine (FT4) and free triiodothyronine (FT3) in 238 individuals, including 90 GBS, 44 multiple sclerosis (MS) and 104 healthy controls...
January 2, 2017: International Journal of Neuroscience
https://www.readbyqxmd.com/read/28040387/takotsubo-cardiomyopathy-associated-with-miller-fisher-syndrome
#16
Dalvir Gill, Kan Liu
51-year-old female who presented with progressive paresthesia, numbness of the lower extremities, double vision, and trouble walking. Physical exam was remarkable for areflexia, and ptosis. Her initial EKG showed nonspecific ST segment changes and her Troponin T was elevated to 0.41ng/mL which peaked at 0.66ng/mL. Echocardiogram showed a depressed left ventricular ejection fraction to 35% with severely hypokinetic anterior wall and left ventricular apex was severely hypokinetic. EMG nerve conduction study showed severely decreased conduction velocity and prolonged distal latency in all nerves consistent with demyelinating disease...
December 22, 2016: American Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28039494/demyelination-patterns-in-a-mathematical-model-of-multiple-sclerosis
#17
M C Lombardo, R Barresi, E Bilotta, F Gargano, P Pantano, M Sammartino
In this paper we derive a reaction-diffusion-chemotaxis model for the dynamics of multiple sclerosis. We focus on the early inflammatory phase of the disease characterized by activated local microglia, with the recruitment of a systemically activated immune response, and by oligodendrocyte apoptosis. The model consists of three equations describing the evolution of macrophages, cytokine and apoptotic oligodendrocytes. The main driving mechanism is the chemotactic motion of macrophages in response to a chemical gradient provided by the cytokines...
December 30, 2016: Journal of Mathematical Biology
https://www.readbyqxmd.com/read/28039317/subcutaneous-interferon-%C3%AE-1a-in-the-treatment-of-clinically-isolated-syndromes-3-year-and-5-year-results-of-the-phase-iii-dosing-frequency-blind-multicentre-reflexion-study
#18
Giancarlo Comi, Nicola De Stefano, Mark S Freedman, Frederik Barkhof, Bernard M J Uitdehaag, Marlieke de Vos, Kurt Marhardt, Liang Chen, Delphine Issard, Ludwig Kappos
OBJECTIVE: Early treatment following a first clinical demyelinating event (FCDE) delays further disease activity in patients with multiple sclerosis (MS). This study determined the effects of early versus delayed treatment (DT) with subcutaneous interferon (sc IFN) β-1a 44 μg in patients with an FCDE up to 60 months postrandomisation. METHODS: Patients who completed the 24-month double-blind REFLEX (REbif FLEXible dosing in early MS) study entered an extension (REFLEXION, REbif FLEXible dosing in early MS extensION): patients initially randomised to sc IFN β-1a and not reaching clinically definite MS (clinically definite MS, CDMS (second attack or sustained Expanded Disability Status Scale (EDSS) score increase)) continued original treatment (three times weekly (tiw) or once weekly (qw)); placebo patients switched to tiw (DT); patients with CDMS switched to tiw...
December 30, 2016: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28032003/a-rare-association-between-multiple-sclerosis-and-charcot-marie-tooth-type-1b
#19
Rosa Cortese, Stefano Zoccolella, Maria Muglia, Alessandra Patitucci, Antonio Scarafino, Damiano Paolicelli, Isabella Laura Simone
The association between multiple sclerosis (MS) and hereditary and sporadic demyelinating disorders of the peripheral nervous system is extremely rare. We herein report a case of Charcot-Marie-Tooth disease type 1B with p.Val102fs mutation in the MPZ gene that developed relapsing remitting MS.
December 2016: Brain and Behavior
https://www.readbyqxmd.com/read/28025778/role-of-mast-cells-in-the-pathogenesis-of-multiple-sclerosis-and-experimental-autoimmune-encephalomyelitis
#20
REVIEW
Daniel Elieh-Ali-Komi, Yonghao Cao
Multiple sclerosis (MS) is a neurological autoimmune disorder of the central nervous system (CNS), characterized by recurrent episodes of inflammatory demyelination and consequent axonal deterioration. The hallmark of the disease is the demyelinated plaque, a hypocellular area characterized by formation of astrocytic scars and infiltration of mononuclear cells. Recent studies have revealed that both innate and adaptive immune cells contribute to the pathogenesis of MS and its experimental autoimmune encephalomyelitis (EAE) model...
December 26, 2016: Clinical Reviews in Allergy & Immunology
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