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demyelinating disorder

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https://www.readbyqxmd.com/read/28725551/cervical-cord-myelin-water-imaging-shows-degenerative-changes-over-one-year-in-multiple-sclerosis-but-not-neuromyelitis-optica-spectrum-disorder
#1
Anna J E Combes, Lucy Matthews, Jimmy S Lee, David K B Li, Robert Carruthers, Anthony L Traboulsee, Gareth J Barker, Jacqueline Palace, Shannon Kolind
Spinal cord pathology is a feature of both neuromyelitis optica spectrum disorder (NMOSD) and relapsing-remitting multiple sclerosis (MS). While subclinical disease activity has been described in MS using quantitative magnetic resonance imaging measures, current evidence suggests that neurodegeneration is absent between relapses in NMOSD, although most evidence comes from brain studies. We aimed to assess cross-sectional differences and longitudinal changes in myelin integrity in relapse-free MS and NMOSD subjects over one year...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28724595/hypertensive-encephalopathy-mimicking-cerebral-vasculitis-with-pontine-oedema-cerebellar-white-matter-lesions-and-multiple-cerebral-infarctions
#2
Daniela Ceccarelli, David Hargroves, Ibrahim Balogun, Thomas Webb
A 47-year-old man with poorly controlled hypertension presented with headaches, right-sided weakness and dysarthria. CT and MRI scans of the brain showed widespread abnormalities including significant pontine oedema, basal ganglia and corona radiata infarctions and cerebellar white matter high signal. Imaging of the intracerebral vasculature also demonstrated wall irregularities. Initially a central nervous system inflammatory disorder was thought to be the most likely diagnosis, possibly acute demyelinating encephalomyelitis or cerebral vasculitis, and the patient was treated with high-dose intravenous steroids...
July 19, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28714395/five-decades-of-cuprizone-an-updated-model-to-replicate-demyelinating-diseases
#3
Jose M Vega-Riquer, Gerardo Mendez-Victoriano, Raul A Morales-Luckie, Oscar Gonzalez-Perez
Multiple sclerosis, Guillain-Barré syndrome, peripheral nerve polyneuropathy and others comprise a group of demyelinating diseases characterized by progressive (and eventually irreversible) loss of oligodendrocytes and myelin sheaths in the white matter of the brain. To date, the etiology of these disorders is not well known and no effective treatments are currently available. Therefore, further research is needed to gain a better understand and treat these patients. To accomplish this goal, it is necessary to have appropriate animal models that closely resemble the pathophysiology of these diseases...
July 17, 2017: Current Neuropharmacology
https://www.readbyqxmd.com/read/28712329/organotypic-cultures-of-cerebellar-slices-as-a-model-to-investigate-demyelinating-disorders
#4
Frédéric Doussau, Jean-Luc Dupont, Dorine Neel, Aline Schneider, Bernard Poulain, Jean Louis Bossu
Demyelinating disorders, characterized by a chronic or episodic destruction of the myelin sheath, are a leading cause of neurological disability in young adults in western countries. Studying the complex mechanisms involved in axon myelination, demyelination and remyelination requires an experimental model preserving the neuronal networks and neuro-glial interactions. Organotypic cerebellar slice cultures appear to be the best alternative to in vivo experiments and the most commonly used model for investigating etiology or novel therapeutic strategies in multiple sclerosis...
July 17, 2017: Expert Opinion on Drug Discovery
https://www.readbyqxmd.com/read/28708133/autoantibodies-in-chronic-inflammatory-neuropathies-diagnostic-and-therapeutic-implications
#5
REVIEW
Luis Querol, Jérôme Devaux, Ricard Rojas-Garcia, Isabel Illa
The chronic inflammatory neuropathies (CINs) are rare, very disabling autoimmune disorders that generally respond well to immune therapies such as intravenous immunoglobulin (IVIg). The most common forms of CIN are chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy, and polyneuropathy associated with monoclonal gammopathy of unknown significance. The field of CIN has undergone a major advance with the identification of IgG4 autoantibodies directed against paranodal proteins in patients with CIDP...
July 14, 2017: Nature Reviews. Neurology
https://www.readbyqxmd.com/read/28707358/suppressed-oligodendrocyte-steroidogenesis-in-multiple-sclerosis-implications-for-regulation-of-neuroinflammation
#6
Roobina Boghozian, Brienne A McKenzie, Leina B Saito, Ninad Mehta, William G Branton, JianQiang Lu, Glen B Baker, Farshid Noorbakhsh, Christopher Power
Multiple sclerosis (MS) is an inflammatory demyelinating disease of the central nervous system (CNS). Neurosteroids are reported to exert anti-inflammatory effects in several neurological disorders. We investigated the expression and actions of the neurosteroid, dehydroepiandrosterone (DHEA), and its more stable 3β-sulphated ester, DHEA-S, in MS and associated experimental models. CNS tissues from patients with MS and animals with experimental autoimmune encephalomyelitis (EAE) displayed reduced DHEA concentrations, accompanied by diminished expression of the DHEA-synthesizing enzyme CYP17A1 in oligodendrocytes (ODCs), in association with increased expression of inflammatory genes including interferon (IFN)-γ and interleukin (IL)-1β...
July 14, 2017: Glia
https://www.readbyqxmd.com/read/28704229/treatment-of-hyponatremic-encephalopathy-in-the-critically-ill
#7
Steven G Achinger, Juan Carlos Ayus
OBJECTIVES: Hyponatremic encephalopathy, symptomatic cerebral edema due to a low osmolar state, is a medical emergency and often encountered in the ICU setting. This article provides a critical appraisal and review of the literature on identification of high-risk patients and the treatment of this life-threatening disorder. DATA SOURCES, STUDY SELECTION, AND DATA EXTRACTION: Online search of the PubMed database and manual review of articles involving risk factors for hyponatremic encephalopathy and treatment of hyponatremic encephalopathy in critical illness...
July 11, 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/28699668/poems-syndrome-2017-update-on-diagnosis-risk-stratification-and-management
#8
Angela Dispenzieri
DISEASE OVERVIEW: POEMS syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. Diagnoses are often delayed because the syndrome is rare and can be mistaken for other neurologic disorders, most commonly chronic inflammatory demyelinating polyradiculoneuropathy...
August 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28699327/acute-onset-severe-occipital-neuralgia-associated-with-high-cervical-lesion-in-patients-with-neuromyelitis-optica-spectrum-disorder
#9
Yuichi Hayashi, Akihiro Koumura, Megumi Yamada, Akio Kimura, Toshirou Shibata, Takashi Inuzuka
OBJECTIVE: To address occipital neuralgia in patients with neuromyelitis optica spectrum disorder (NMOSD). BACKGROUND: NMOSD is an inflammatory demyelinating disease that commonly presents with pain; however, headache symptoms have received little attention. METHODS: We presented three cases of NMOSD in which the patients experienced acute-onset, severe, and steroid-responsive occipital neuralgia. All patients provided consent to use their demographic and imaging data retrospectively...
July 2017: Headache
https://www.readbyqxmd.com/read/28698711/anti-n-methyl-d-aspartate-receptor-encephalitis-a-severe-potentially-reversible-autoimmune-encephalitis
#10
REVIEW
Cai-Yun Liu, Jie Zhu, Xiang-Yu Zheng, Chi Ma, Xu Wang
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is potentially lethal, but it is also a treatable autoimmune disorder characterized by prominent psychiatric and neurologic symptoms. It is often accompanied with teratoma or other neoplasm, especially in female patients. Anti-NMDAR antibodies in cerebrospinal fluid (CSF) and serum are characteristic features of the disease, thereby suggesting a pathogenic role in the disease. Here, we summarize recent studies that have clearly documented that both clinical manifestations and the antibodies may contribute to early diagnosis and multidisciplinary care...
2017: Mediators of Inflammation
https://www.readbyqxmd.com/read/28694334/loss-of-tuberous-sclerosis-complex1-in-adult-oligodendrocyte-progenitor-cells-enhances-axon-remyelination-and-increases-myelin-thickness-after-a-focal-demyelination
#11
Lauren E McLane, Jennifer N Bourne, Angelina V Evangelou, Luipa Khandker, Wendy B Macklin, Teresa L Wood
While the mammalian target of rapamycin (mTOR) is an essential regulator of developmental oligodendrocyte differentiation and myelination, oligodendrocyte-specific deletion of tuberous sclerosis complex (TSC), a major upstream inhibitor of mTOR, surprisingly also leads to hypomyelination during CNS development. However, the function of TSC has not been studied in the context of remyelination. Here, we utilized the inducible Cre-lox system to study the function of TSC in the remyelination of a focal, lysolecithin demyelinated lesion in adult male mice...
July 10, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28694071/first-clinical-and-genetic-description-of-a-family-diagnosed-with-late-onset-pompe-disease-from-costa-rica
#12
Gabriel Torrealba-Acosta, María Consuelo Rodríguez-Roblero, Sixto Bogantes-Ledezma, Kenneth Carazo-Céspedes, Claude Desnuelle
Glycogen storage disease type II, also known as Pompe disease, is an autosomal recessive disorder caused by deficiency of enzymatic activity of acid alpha-glucosidase. The wide phenotypical variation of this disease relates to the amount of residual enzymatic activity depending on the combination of mutations on each allele. We confirmed Pompe disease in a patient that presented with progressive weakness, recurrent episodes of respiratory failure associated with pneumonia, a predominantly demyelinating mixed sensorimotor polyneuropathy and paraspinal complex repetitive discharges...
June 20, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28684532/longitudinal-analysis-of-myelin-oligodendrocyte-glycoprotein-antibodies-in-cns-inflammatory-diseases
#13
Jae-Won Hyun, Mark R Woodhall, Su-Hyun Kim, In Hye Jeong, Byeong-Su Kong, Gayoung Kim, Yeseul Kim, Min Su Park, Sarosh R Irani, Patrick Waters, Ho Jin Kim
BACKGROUND: We evaluated the seroprevalence of myelin oligodendrocyte glycoprotein immunoglobulin G1 (MOG-IgG) and associated clinical features of patients from a large adult-dominant unselected cohort with mainly relapsing central nervous system (CNS) inflammatory diseases. We also investigate the clinical relevance of MOG-IgG through a longitudinal analysis of serological status over a 2-year follow-up period. METHODS: Serum samples from 505 patients with CNS inflammatory diseases at the National Cancer Center were analysed using cell-based assays for MOG-IgG and aquaporin-4 immunoglobulin G (AQP4-IgG)...
July 6, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28684154/coexistence-of-multiple-sclerosis-and-ankylosing-spondylitis-report-of-four-cases-from-russia-and-review-of-the-literature
#14
Vera Fominykh, Tatyana Shevtsova, Narine Arzumanian, Lev Brylev
Multiple sclerosis is a chronic demyelinating disorder of the central nervous system. There are many cases of multiple sclerosis - like syndrome and demyelinating disorders in systemic lupus erythematosus, Sjogren disease, Behcet disease and other autoimmune conditions. Coexistence of ankylosing spondylitis and multiple sclerosis usually is rare but in this article we report 4 Russian patients with concomitant multiple sclerosis and ankylosing spondylitis diseases. None of these patients received anti-tumor necrosis factor alpha therapy prior to diagnosis of multiple sclerosis...
July 3, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28682959/paraneoplastic-neuropathies
#15
Jean-Christophe Antoine, Jean-Philippe Camdessanché
PURPOSE OF REVIEW: To review recent advances in paraneoplastic neuropathies with emphasis on their definition, different forms and therapeutic development. RECENT FINDINGS: A strict definition of definite paraneoplastic neuropathies is necessary to avoid confusion. With carcinoma, seronegative sensory neuronopathies and neuronopathies and anti-Hu and anti-CV2/Contactin Response Mediator Protein 5 antibodies are the most frequent. With lymphomas, most neuropathies occur with monoclonal gammopathy including AL amyloidosis, Polyneuropathy-Organomegaly-Endocrinopathy-M component-Skin changes (POEMS) syndrome, type I cryoglobulinemia and antimyelin-associated glycoprotein (MAG) neuropathies and Waldenström's disease...
July 5, 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28679367/relationship-between-azathioprine-metabolites-and-therapeutic-efficacy-in-chinese-patients-with-neuromyelitis-optica-spectrum-disorders
#16
Xindi Li, Shenghui Mei, Xiaoqing Gong, Heng Zhou, Li Yang, Anna Zhou, Yonghong Liu, Xingang Li, Zhigang Zhao, Xinghu Zhang
BACKGROUND: Neuromyelitis optica spectrum disorders (NMOSD) are demyelinating autoimmune diseases in the central nervous system (CNS) that are characterized by a high relapse rate and the presence of anti-aquaporin 4 antibodies (AQP4-IgG) in the serum. Azathioprine (AZA) is a first-line immunomodulatory drug that is widely used for the treatment of patients with NMOSD. However, the efficacy and safety of AZA vary in different individuals. METHOD: Thirty-two patients with NMOSD who regularly took AZA were enrolled in the study at Beijing Tiantan Hospital, Capital Medical University...
July 5, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28675955/psychiatric-disorders-in-children-with-demyelinating-diseases-of-the-central-nervous-system
#17
Julia Pakpoor, Raph Goldacre, Klaus Schmierer, Gavin Giovannoni, Emmanuelle Waubant, Michael J Goldacre
INTRODUCTION: The profile of psychiatric disorders associated with multiple sclerosis (MS) may differ in children. We aimed to assess the risk of psychiatric disorders in children with MS and other demyelinating diseases, and vice versa. PATIENTS AND METHODS: We analyzed linked English Hospital Episode Statistics, and mortality data, 1999-2011. Cohorts were constructed of children admitted with MS and other central nervous system (CNS) demyelinating diseases. We searched for any subsequent episode of care with psychiatric disorders in these cohorts and compared to a reference cohort...
July 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28657431/csf-and-clinical-data-are-useful-in-differentiating-cns-inflammatory-demyelinating-disease-from-cns-lymphoma
#18
Ryotaro Ikeguchi, Yuko Shimizu, Satoru Shimizu, Kazuo Kitagawa
BACKGROUND: It is often difficult to diagnose central nervous system (CNS) inflammatory demyelinating diseases (IDDs) because they are similar to CNS lymphoma and glioma. OBJECTIVE: To evaluate whether cerebrospinal fluid (CSF) analysis can differentiate CNS IDDs from CNS lymphoma and glioma. METHODS: We measured CSF cell counts; concentrations of proteins, glucose, interleukin (IL)-6, IL-10, soluble IL-2 receptor (sIL-2R), and myelin basic protein; and IgG index in patients with multiple sclerosis (MS, n = 64), neuromyelitis optica spectrum disorder (NMOSD, n = 35), tumefactive demyelinating lesion (TDL, n = 17), CNS lymphoma ( n = 12), or glioma ( n = 10)...
June 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28647707/hepatitis-e-virus-and-neurological-disorders
#19
REVIEW
Brendan N Mclean, James Gulliver, Harry R Dalton
Hepatitis E is the most common cause of hepatitis worldwide. While originally considered a disease of developing countries, it is increasingly recognised in developed countries, probably related to contaminated pork meat, and where infection is often asymptomatic. However, several non-liver manifestations have become apparent, the most important of which are neurological, including Guillain-Barré syndrome (acute inflammatory demyelinating polyradiculoneuropathy (AIDP)), neuralgic amyotrophy and meningoencephalitis...
June 24, 2017: Practical Neurology
https://www.readbyqxmd.com/read/28641774/membranous-nephropathy-with-neuromyelitis-optica-spectrum-disorder
#20
Xiangling Li, YanQiang Wang
Membranous nephropathy (MN) accompanying Neuromyelitis optica spectrum disorders (NMOSD) has rarely been described previously. We recently presented a 45-year-old Chinese male presenting with recurrent lower extremity pitting edema, or eyelid edema, proteinuria and hyperlipidemia. especially intractable hiccup and vomiting, painful tonic spasm (PTS) as the revealing symptom of a demyelinating disorder of central nervous system. The kindey biopsy specimen showed MN stage 2. Serological testing revealed antibodies AQP4, MRI head and spine revealed medulla oblongata and C1-C2 cervical vertebra lesions...
July 2017: Multiple Sclerosis and related Disorders
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