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Demyelinating disease

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https://www.readbyqxmd.com/read/28427710/clinical-outcomes-and-predictive-factors-related-to-good-outcomes-in-plasma-exchange-in-severe-attack-of-nmosd-and-long-extensive-transverse-myelitis-case-series-and-review-of-the-literature
#1
Saharat Aungsumart, Metha Apiwattanakul
OBJECTIVE: To investigate the predictive factors associated with good outcomes of plasma exchange in severe attacks through neuromyelitis optica spectrum disorder (NMOSD) and long extensive transverse myelitis (LETM). In addition, to review the literature of predictive factors associated with the good outcomes of plasma exchange in central nervous system inflammatory demyelinating diseases (CNS IDDs). METHODS: Retrospective study in 27 episodes of severe acute attacks myelitis and optic neuritis in 24 patients, including 20 patients with NMOSD seropositive, 1 patient with NMOSD seronegative and 3 patients with LETM...
April 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28427706/neuromyelitis-optica-spectrum-disorder-related-tonic-spasms-responsive-to-lacosamide
#2
A Baheerathan, W J Brownlee, F Rugg-Gunn, D T Chard, S A Trip
Paroxysmal tonic spasms [PTS] are common in patients with neuromyelitis optica spectrum disorder (NMOSD).1 2 In patients with demyelinating disease, PTS can significantly reduce the quality of life, limit activities of daily living and the rehabilitative process following an acute relapse 3. As in patients with multiple sclerosis (MS), paroxysmal tonic spasms in NMOSD usually respond well to treatment with carbamazepine.2 However, the optimal treatment in patients where carbamazepine is contraindicated or poorly tolerated is unclear...
April 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28427705/presence-of-central-veins-and-susceptibility-weighted-imaging-for-evaluating-lesions-in-multiple-sclerosis-and-leukoaraiosis
#3
Urška Lamot, Jernej Avsenik, Saša Šega, Katarina Šurlan Popovič
PURPOSE: The process of demyelination in multiple sclerosis (MS) is reflected in lesions of the central nervous system (CNS), which are found in an abundance of different diseases and are frequently radiographically indistinguishable. Our aim was to determine whether the perivenous distribution of MS lesions identified on susceptibility weighted images (SWI) could be used as a specific radiographic sign for MS, and also to determine whether the visibility of the central vein (CV) is affected by the activity of MS lesions...
April 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28427703/multiple-sclerosis-among-afghan-immigrants-in-isfahan-iran
#4
Masoud Etemadifar, Niyousha Sadeghpour, Kimia Nekouie, Mohammadmostafa Jahansouz, Mehri Salari, Mahboobeh Fereidan-Esfahani
BACKGROUND: Multiple sclerosis is a central nervous system demyelinating disease with unknown etiology. However, it is believed to be a multifactorial disease resulting from an interaction of genetic and environmental factors. Immigrant studies have been performed to provide a better view of the pattern of this interaction. We aimed to report the prevalence of MS Afghan immigrants of Isfahan, a population who share the same environment as Isfahan residents but with different genetic backgrounds...
April 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28427702/long-term-clinical-and-radiologic-follow-up-of-schilder-s-disease
#5
Hülya Maraş Genç, Bülent Kara, Emek Uyur Yalçın, Ayfer Sakarya Güneş, Adnan Deniz, Yonca Anık
BACKGROUND: Schilder's disease is a rare, subacute, or chronic demyelinating disorder that mainly affects children and generally shows a monophasic course. CASE: Here, we present three boys diagnosed with Schilder's disease, age at onset 10-14 years, and followed up for 4-8 years. All of them presented with headache, two with encephalopathy and vomiting, and one with diplopia and vertigo. Cranial magnetic resonance imaging (MRI) showed two large demyelinating lesions, asymmetric in two patients and symmetric in the other...
April 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28425868/pomegranate-as-a-possible-treatment-in-reducing-risk-of-developing-wound-healing-obesity-neurodegenerative-disorders-and-diabetes-mellitus
#6
Manaf AlMatar, Md Rashedul Islam, Othman Albari, Işıl Var, Fatih Köksal
Four distinguished although overlying stages make up the methodical procedure of wound healing, which are hemostasis, inflammation, proliferation and remodelling. Multiple sclerosis (MS) comprises a persistent inflammatory infection of the central nervous system, and is related to demyelination, neurodegeneration, as well as susceptibility to oxidative pressure. Obesity signifies a swiftly developing danger to the wellbeing of populations in a rising number of nations. Usually called diabetes mellitus (DM) by medical practitioners, diabetes details a collection of metabolic diseases within which the individual has raised blood glucose, either due to an insufficiency of insulin generation, or the lack of suitable response by the body to insulin, or both...
April 19, 2017: Mini Reviews in Medicinal Chemistry
https://www.readbyqxmd.com/read/28423529/pif-promotes-brain-re-myelination-locally-while-regulating-systemic-inflammation-clinically-relevant-multiple-sclerosis-m-smegmatis-model
#7
Giuseppe Migliara, Martin Mueller, Alessia Piermattei, Chaya Brodie, Michael J Paidas, Eytan R Barnea, Francesco Ria
Neurologic disease diagnosis and treatment is challenging. Multiple Sclerosis (MS) is a demyelinating autoimmune disease with few clinical forms and uncertain etiology. Current studies suggest that it is likely caused by infection(s) triggering a systemic immune response resulting in antigen/non-antigen-related autoimmune response in central nervous system (CNS). New therapeutic approaches are needed. Secreted by viable embryos, PreImplantation Factor (PIF) possesses a local and systemic immunity regulatory role...
March 28, 2017: Oncotarget
https://www.readbyqxmd.com/read/28422748/therapeutic-inhibition-of-soluble-brain-tnf-promotes-remyelination-by-increasing-myelin-phagocytosis-by-microglia
#8
Maria Karamita, Christopher Barnum, Wiebke Möbius, Malú G Tansey, David E Szymkowski, Hans Lassmann, Lesley Probert
Multiple sclerosis (MS) is an inflammatory CNS demyelinating disease in which remyelination largely fails. Transmembrane TNF (tmTNF) and TNF receptor 2 are important for remyelination in experimental MS models, but it is unknown whether soluble TNF (solTNF), a major proinflammatory factor, is involved in regeneration processes. Here, we investigated the specific contribution of solTNF to demyelination and remyelination in the cuprizone model. Treatment with XPro1595, a selective inhibitor of solTNF that crosses the intact blood-brain barrier (BBB), in cuprizone-fed mice did not prevent toxin-induced oligodendrocyte loss and demyelination, but it permitted profound early remyelination due to improved phagocytosis of myelin debris by CNS macrophages and prevented disease-associated decline in motor performance...
April 20, 2017: JCI Insight
https://www.readbyqxmd.com/read/28422408/prevention-of-the-osmotic-demyelination-syndrome-after-liver-transplantation-a-multidisciplinary-perspective
#9
J F Crismale, K A Meliambro, S DeMaria, D B Bronster, S Florman, T D Schiano
The osmotic demyelination syndrome (ODS) is a serious neurologic condition that occurs in the setting of rapid correction of hyponatremia. It presents with protean manifestations, from encephalopathy to the "locked-in" syndrome. ODS can complicate liver transplantation (LT), and its incidence may increase with the inclusion of serum sodium as a factor in the Model for End-Stage Liver Disease score. A comprehensive understanding of risk factors for the development of ODS in the setting of LT, along with recommendations to mitigate the risk of ODS, are necessary...
April 19, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28420330/anti-mog-antibody-positive-adem-following-infectious-mononucleosis-due-to-a-primary-ebv-infection-a-case-report
#10
Yoshitsugu Nakamura, Hideto Nakajima, Hiroki Tani, Takafumi Hosokawa, Shimon Ishida, Fumiharu Kimura, Kimihiko Kaneko, Toshiyuki Takahashi, Ichiro Nakashima
BACKGROUND: Anti-Myelin oligodendrocyte glycoprotein (MOG) antibodies are detected in various demyelinating diseases, such as pediatric acute disseminated encephalomyelitis (ADEM), recurrent optic neuritis, and aquaporin-4 antibody-seronegative neuromyelitis optica spectrum disorder. We present a patient who developed anti-MOG antibody-positive ADEM following infectious mononucleosis (IM) due to Epstein-Barr virus (EBV) infection. CASE PRESENTATION: A 36-year-old healthy man developed paresthesia of bilateral lower extremities and urinary retention 8 days after the onset of IM due to primary EBV infection...
April 19, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28418523/neurocognitive-trajectory-of-boys-who-received-a-hematopoietic-stem-cell-transplant-at-an-early-stage-of-childhood-cerebral-adrenoleukodystrophy
#11
Elizabeth I Pierpont, Julie B Eisengart, Ryan Shanley, David Nascene, Gerald V Raymond, Elsa G Shapiro, Rich S Ziegler, Paul J Orchard, Weston P Miller
Importance: Untreated childhood cerebral adrenoleukodystrophy (cALD) is a fatal disease associated with progressive cerebral demyelination and rapid, devastating neurologic decline. The standard of care to enhance long-term survival and stabilize cerebral disease is a hematopoietic stem cell transplant (HSCT). Neurologic outcomes are better when HSCT occurs at an earlier stage of cALD, yet there is limited understanding of the neurocognitive trajectory of patients who undergo HSCT. Objectives: To characterize neurocognitive outcomes of boys with cALD and early-stage cerebral disease who were treated with an allogeneic HSCT and to identify disease- and treatment-related factors associated with long-term functioning...
April 17, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/28416698/selective-in-vivo-removal-of-pathogenic-anti-mag-autoantibodies-an-antigen-specific-treatment-option-for-anti-mag-neuropathy
#12
Ruben Herrendorff, Pascal Hänggi, Hélène Pfister, Fan Yang, Delphine Demeestere, Fabienne Hunziker, Samuel Frey, Nicole Schaeren-Wiemers, Andreas J Steck, Beat Ernst
Anti-MAG (myelin-associated glycoprotein) neuropathy is a disabling autoimmune peripheral neuropathy caused by monoclonal IgM autoantibodies that recognize the carbohydrate epitope HNK-1 (human natural killer-1). This glycoepitope is highly expressed on adhesion molecules, such as MAG, present in myelinated nerve fibers. Because the pathogenicity and demyelinating properties of anti-MAG autoantibodies are well established, current treatments are aimed at reducing autoantibody levels. However, current therapies are primarily immunosuppressive and lack selectivity and efficacy...
April 17, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28415697/down-regulation-of-interleukin-7-receptor-il-7r-contributes-to-central-nervous-system-demyelination
#13
Xudan Lei, Shijiao Cai, Yang Chen, Jianlin Cui, Yajie Wang, Zongjin Li, Yuhao Li
Interleukin 7 receptor (IL-7R) has been associated with the pathogenesis of multiple sclerosis (MS), though the mechanisms are not clear. Because myelin expression is highly conserved between zebrafish and mammals, zebrafish have become an ideal model for studying demyelination. We used a transgenic (Tg; mbp:nfsB-egfp) zebrafish line in which oligodendrocytes expressed green fluorescent protein (GFP) from the larval stage to adulthood. Exposing adult transgenic zebrafish to metronidazole induced demyelination that resembled the morphological changes associated with the early stages of MS...
March 10, 2017: Oncotarget
https://www.readbyqxmd.com/read/28413712/variation-within-mbp-gene-predicts-disease-course-in-multiple-sclerosis
#14
Yuan Zhou, Steve Simpson, Jac C Charlesworth, Ingrid van der Mei, Robyn M Lucas, Anne-Louise Ponsonby, Bruce V Taylor
OBJECTIVE: Prognosis following a first demyelinating event is difficult to predict, with no genetic markers of MS progression currently identified. Myelin basic protein (MBP) is a major component of the myelin sheath of CNS neurons and may play a central role in demyelinating diseases such as MS. However, genetic variation in MBP has not been implicated in MS onset risk in large genome-wide association studies. We hypothesized that genetic variations in MBP may be a determinant of MS clinical course...
April 2017: Brain and Behavior
https://www.readbyqxmd.com/read/28410279/ocular-motor-manifestations-of-multiple-sclerosis
#15
Elodie Nerrant, Caroline Tilikete
BACKGROUND: Multiple sclerosis (MS) is a demyelinating disease of the central nervous system leading to disability, especially in young patients. Acute or chronic lesions of MS within the brainstem and the cerebellum frequently result in ocular motor disorders. EVIDENCE ACQUISITION: This review encompasses the spectrum of ocular motor disorders in patients with MS emphasizing prevalence, examination findings, diagnostic features, functional consequences, classification of MS course, and management of these disturbances of ocular motility...
April 13, 2017: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/28409314/regulation-of-asymmetric-cell-division-in-mammalian-neural-stem-and-cancer-precursor-cells
#16
Mathieu Daynac, Claudia K Petritsch
Stem and progenitor cells are characterized by their abilities to self-renew and produce differentiated progeny. The balance between self-renewal and differentiation is achieved through control of cell division mode, which can be either asymmetric or symmetric. Failure to properly control cell division mode may result in premature depletion of the stem/progenitor cell pool or abnormal growth and impaired differentiation. In many tissues, including the brain, stem cells and progenitor cells undergo asymmetric cell division through the establishment of cell polarity...
2017: Results and Problems in Cell Differentiation
https://www.readbyqxmd.com/read/28404595/nlr-members-nlrc4-and-nlrp3-mediate-sterile-inflammasome-activation-in-microglia-and-astrocytes
#17
Leslie Freeman, Haitao Guo, Clément N David, W June Brickey, Sushmita Jha, Jenny P-Y Ting
Inflammation in the brain accompanies several high-impact neurological diseases including multiple sclerosis (MS), stroke, and Alzheimer's disease. Neuroinflammation is sterile, as damage-associated molecular patterns rather than microbial pathogens elicit the response. The inflammasome, which leads to caspase-1 activation, is implicated in neuroinflammation. In this study, we reveal that lysophosphatidylcholine (LPC), a molecule associated with neurodegeneration and demyelination, elicits NLRP3 and NLRC4 inflammasome activation in microglia and astrocytes, which are central players in neuroinflammation...
April 12, 2017: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28401686/axonal-loss-in-the-multiple-sclerosis-spinal-cord-revisited
#18
N Petrova, D Carassiti, D R Altmann, D Baker, K Schmierer
Preventing chronic disease deterioration is an unmet need in people with multiple sclerosis, where axonal loss is considered a key substrate of disability. Clinically, chronic multiple sclerosis often presents as progressive myelopathy. Spinal cord cross-sectional area assessed using MRI predicts increasing disability and has, by inference, been proposed as an indirect index of axonal degeneration. However, the association between cross-sectional area and axonal loss, and their correlation with demyelination, have never been systematically investigated using human post mortem tissue...
April 12, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28399101/-clinical-and-epidemiological-characteristics-of-hereditary-motor-sensory-neuropathy-1x-caused-by-the-mutation-c-259c-g-p-p87a-in-the-gjb1-gene-of-patients-from-the-republic-of-bashkortostan
#19
E V Saifullina, R V Magzhanov, I M Khidiyatova, E K Khusnutdinova
BACKGROUND: Hereditary motor-sensory neuropathy 1X (НМСН 1X) is the second frequent form of hereditary motor-sensory neuropathies caused by mutations in the GJB1 gene (gap junction B1 type). The authors have established earlier that the с.259C>G (р.P87A) mutation is the most frequent cause of НМСН 1Х (92%) in patients from the Republic of Bashkortostan. AIM: To study in details the territorial ethnic distribution and clinical manifestations of the с...
2017: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/28397393/early-discrimination-of-sensorimotor-guillain-barr%C3%A3-syndrome-into-demyelinating-or-axonal-subtype-by-automated-nerve-excitability-testing
#20
So Young Pyun, Mi-Ri Kang, Joo Young Lee, Kim Jong Kuk, Seong-Il Oh, Jong Seok Bae
In the early stage of disease, differentiating acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor sensory axonal neuropathy (AMSAN) using only a conventional nerve conduction studies (NCS) may be difficult. We evaluated the differences in the motor axonal excitability properties of 16 cases of sensorimotor Guillain-Barré syndrome by nerve excitability testing (NET). The antiganglioside antibody assay and follow-up NCS resulted in 12 patients diagnosed as AIDP and 4 patients as AMSAN. Clinical and excitability parameters in each group were compared with those in 30 normal controls...
April 11, 2017: Journal of the Peripheral Nervous System: JPNS
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