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Demyelination diseases

Tünde Csépány
The revolutionary progress of research in neuroimmu-nology has led to the introduction of disease modifying therapies in multiple sclerosis at the end of the last century. The International Panel on Diagnosis of Multiple Sclerosis originally proposed the 2001 McDonald criteria to facilitate the diagnosis of MS in patients with the first objective neurological symptom(s) suggesting demyelinating event, when magnetic resonance imaging is integrated with clinical and other paraclinical diagnostic methods. New terms have been introduced to substitute clinical information by MRI: dissemination in space - indicating a multifocal central demyelinating process and dissemination in time - indicating the development of new CNS lesions over time...
September 30, 2018: Ideggyógyászati Szemle
Shogo Tanabe, Yuki Fujita, Kaori Ikuma, Toshihide Yamashita
Multiple sclerosis (MS) is an autoimmune disease of the central nervous system that is characterized by motor deficits, fatigue, pain, cognitive impairment, and sensory and visual dysfunction. Secondary progressive multiple sclerosis (SPMS) is a progressive form of MS that develops from relapsing-remitting MS. Repulsive guidance molecule-a (RGMa) has diverse functions, including axon growth inhibition and immune regulation. Here, we show inhibiting RGMa had therapeutic effects in mouse models of SPMS. We induced experimental autoimmune encephalomyelitis in nonobese diabetic mice (NOD-EAE mice) and treated them with humanized anti-RGMa monoclonal antibody...
October 17, 2018: Cell Death & Disease
Carole Rosier, Neïla Graveline, Arnaud Lacour, Jean-Christophe Antoine, Jean-Philippe Camdessanché
INTRODUCTION: Chronic Inflammatory demyelinating polyneuropathies (CIDP) and multifocal motor neuropathies (MMN) are rare autoimmune diseases. Guidelines have been published in 2010 for their diagnosis and treatment. In France, intravenous immunoglobulins (IGIVs) are largely used for their first-line treatment but the burden of health-care cost is often underlined. The aim of this survey is to compare to the published guidelines, the French daily practice of neurologists with IGIVs for CIDP and MMN treatment...
October 16, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
Jun Li, Junyu Shi, Yan Sun, Fang Zheng
Autoimmune encephalomyelitis is a chronic autoimmune disease caused by immune-mediated sterile inflammatory response and demyelination in the central nervous system (CNS). High-mobility group box protein 1 (HMGB1) is a ubiquitous nuclear protein, which can be released from damaged cells and induce proinflammatory responses in autoimmune encephalomyelitis. Glycyrrhizin (GL), a major constituent of licorice root, can inhibit the proinflammatory bioactivities of HMGB1. In this article, we bring some insight into the effects of GL on CNS inflammatory diseases and discuss the therapeutic potential of GL in autoimmune encephalomyelitis in the future...
October 16, 2018: DNA and Cell Biology
Ali Seidkhani-Nahal, Ali Noori-Zadeh, Salar Bakhtiyari, Afra Khosravi
Multiple sclerosis (MS) is a neuro-immunological demyelinating disease. From the immunological aspects, it is well accepted that T cells play a pivotal role in the etiology of the disease. T helper (Th) 1 and Th17 cells are thought to be the main pathogenic T cells in the pathogenesis of MS and are known as effector T cells. As the self-reactive T lymphocytes can escape clonal deletion in the thymus and subsequently are released into the periphery, there is an urgent need for peripheral tolerance, which is executed by the specialized regulatory T (Treg) cells...
October 15, 2018: Acta Neurologica Belgica
Muniba Fayyaz, Syeda S Jaffery
Progressive multifocal leukoencephalopathy is a fatal demyelinating disease caused by the John Cunningham virus. It causes white matter inflammation in multiple areas of the brain. Although rare, it has been known to occur in patients with immunodeficiency states and those taking chronic immunosuppressive medications for diseases such as multiple sclerosis and psoriasis. In this article, we will discuss about the drug-induced progressive multifocal encephalopathy and its diagnosis and management in patients taking natalizumab and other disease-modifying therapies among patients with multiple sclerosis...
August 3, 2018: Curēus
Ling Xu, Cuili Zhang, Nan Jiang, Dan He, Ying Bai, Yi Xin
Multiple sclerosis (MS) is a highly disabling demyelinating disease, which mainly affects young adults and is difficult to cure. Activated microglia may be involved in the process of neuronal cell damage and release inflammatory cytokines to injure neurons. Rapamycin (RAPA), an immunosuppressant, can induce autophagy in microglia to delay the process of the disease. As an inhibitor of NLRP3, MCC950 (CP-456773) can regulate the activation of inflammasome. An experimental autoimmune encephalomyelitis model, a disease model of MS, was established to detect the role of activated microglia in the dynamic evolution of MS...
October 15, 2018: Journal of Cellular Biochemistry
Yanjun Liu, Xiangqi Tang
Autoimmune diseases of the nervous system (ADNS) consist of a group of severely disabling disorders characterized by abnormal immune attack against protein components of the nervous system. This type of attack behavior may occur in the central or peripheral nervous system, and in the neuromuscular junction, resulting in neuronal damage, axonal injury, demyelination or destruction of the neuromuscular junction. While the neurological deficits of patients with ADNS have received significant research attention, the manifestation of depression tends to be ignored...
2018: Frontiers in Psychiatry
David John Burrows, Alexander McGown, Saurabh A Jain, Milena De Felice, Tennore M Ramesh, Basil Sharrack, Arshad Majid
Multiple sclerosis (MS) is a chronic, immune-mediated demyelinating disease of the central nervous system. Animal models of MS have been critical for elucidating MS pathological mechanisms and how they may be targeted for therapeutic intervention. Here we review the most commonly used animal models of MS. Although these animal models cannot fully replicate the MS disease course, a number of models have been developed to recapitulate certain stages. Experimental autoimmune encephalomyelitis (EAE) has been used to explore neuroinflammatory mechanisms and toxin-induced demyelinating models to further our understanding of oligodendrocyte biology, demyelination and remyelination...
October 15, 2018: Multiple Sclerosis: Clinical and Laboratory Research
Elisabetta Zucchi, Francesco Cavallieri, Giada Giovannini, Francesca Antonelli, Maria Teresa Mascia, Roberta Bedin, Jessica Mandrioli
A 64 years-old woman presented subacute onset distal paraesthesia concurrently with cold-induced urticaria, a rare form of physical urticaria. Both the disturbances developed a fortnight after an upper respiratory tract infection. EMG confirmed an exclusively sensory polyneuropathy, with prolongation of distal latencies and reduction of amplitudes. Anti-GQ1b and anti-GT1a antigangliosides antibodies were found in serum. The clinical workout included CSF analysis, cryoglobulin and paraprotein search, neurotropic infective agents, neoplastic markers and extensive autoimmune disease antibodies analysis, all of which resulted negative...
October 2018: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Assadullah Metlo, Aiman Rehan, Manahil Akmal, Unzela Iqbal, Momal Jamali
We present the case of a 3-year old girl with clinical manifestations typical of XP-CS, an extremely rare combination of Xeroderma Pigmentosum and Cockayne Syndrome. She had a swelling above the upper lip and multiple brown spots on her face, neck, arms and back. She was globally delayed, deaf, dumb and photophobic. MRI brain showed mild cerebral atrophy and bilateral demyelination. De Sanctis Cacchione variant (dSCS) and Rothmund Thomson syndrome (RTS), which were among the differential diagnosis were ruled out upon careful evaluation...
October 2018: JPMA. the Journal of the Pakistan Medical Association
Gwendoline Montes Diaz, Raymond Hupperts, Judith Fraussen, Veerle Somers
Multiple sclerosis (MS) is a chronic inflammatory autoimmune disease of the central nervous system (CNS) in which demyelination and neurodegeneration occurs. The immune system of MS patients is characterized by a dysregulation in the balance between pro- and anti-inflammatory immune cells, whereby both the innate and adaptive immune system are involved. Dimethyl fumarate (DMF) was licensed in 2013 as an oral first-line therapy for relapsing-remitting (RR)MS patients. It has a strong efficacy with neuroprotective and immunomodulatory effects and a favourable benefit-risk profile...
October 11, 2018: Autoimmunity Reviews
T Mochizuki, J Teranishi, D Takamoto, M Ohtaka, T Kawahara, K Makiyama, H Uemura
Post-kidney transplantation progressive multifocal leukoencephalopathy (PML) is a rare disease on which there are very few published reports on record. PML is a demyelinating disease caused by a destructive infection of the oligodendrocytes by the JC polyomavirus. No effective therapeutic protocol has been established other than measures to revive the immune function by reducing or discontinuing the administration of immunosuppressive agents. Most cases are progressive and show a poor prognosis. We herein report a case in which renal function has been maintained for 2 years following the onset of PML, which was initially diagnosed 3 years after kidney transplantation...
October 2018: Transplantation Proceedings
Xi Guo, Jiajia Zhu, Ningnannan Zhang, Linjie Zhang, Yuan Qi, Huanhuan Cai, Xue Zhang, Jie Sun, Qiuhui Wang, Li Yang, Fu-Dong Shi, Chunshui Yu
Neurovascular coupling reflects the close relationship between neuronal activity and cerebral blood flow (CBF), providing a new mechanistic insight into health and disease. Neuromyelitis optica (NMO) is an autoimmune inflammatory demyelinating disease of the central nervous system and shows cognitive decline-related brain gray matter abnormalities besides the damage of optic nerve and spinal cord. We aimed to investigate neurovascular coupling alteration and its clinical significance in NMO by using regional homogeneity (ReHo) to measure neuronal activity and CBF to measure vascular response...
October 13, 2018: Human Brain Mapping
Stefanie Kuerten, Leila J Jackson, Joel Kaye, Timothy L Vollmer
Growing evidence indicates that B cells play a key role in the pathogenesis of multiple sclerosis (MS). B cells occupy distinct central nervous system (CNS) compartments in MS, including the cerebrospinal fluid and white matter lesions. Also, it is now known that, in addition to entering the CNS, B cells can circulate into the periphery via a functional lymphatic system. Data suggest that the role of B cells in MS mainly involves their in situ activation in demyelinating lesions, leading to altered pro- and anti-inflammatory cytokine secretion, and a highly effective antigen-presenting cell function, resulting in activation of memory or naïve T cells...
October 12, 2018: CNS Drugs
Katherine Chuere Nunes Duarte, Thaís Torres Soares, Angela Maria Paiva Magri, Lívia Assis Garcia, Luciana Le Sueur-Maluf, Ana Cláudia Muniz Renno, Gláucia Monteiro de Castro
There are no effective therapies for remyelination. Low-level laser therapy (LLLT) has been found advantageous in neurogenesis promotion, cell death prevention, and modulation of inflammation in central and peripheral nervous system models. The purpose of this study was to analyse LLLT effects on cuprizone-induced demyelination. Mice were randomly distributed into three groups: Control Laser (CTL), Cuprizone (CPZ), and Cuprizone Laser (CPZL). Mice from CPZ and CPZL groups were exposed to a 0.2% cuprizone oral diet for four complete weeks...
October 3, 2018: Journal of Photochemistry and Photobiology. B, Biology
S Jarius, A B Wuthenow, B Wildemann
The term 'neuromyelitis optica' (NMO, Devic syndrome) is used to refer to a syndrome characterized by optic neuritis and myelitis. For many decades NMO was classified as a clinical variant of multiple sclerosis (MS). Recent research has shown, however, that NMO differs from MS in terms of immunopathogenesis, clinical presentation, and optimum treatment. In most cases, NMO is caused by autoantibodies to aquaporin-4 or myelin oligodendrocyte glycoprotein. While the history of classic MS has been studied extensively, only relatively little is known about the early history of NMO...
September 6, 2018: Journal of the Neurological Sciences
Gary M Owens
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a symmetric demyelinating peripheral neuropathy with either a progressive or relapsing remitting course. CIDP is both debilitating and degenerative, leaving patients with functional impairment due to nerve damage in their extremities. Along with its medical burden, CIDP has a significant economic impact, with disease-related expenses and the often fairly high costs related to the immune therapies used to treat the disease. Costs for patients with this disorder often exceed those of other immune-mediated neuromuscular diseases...
September 2018: American Journal of Managed Care
João Durães, Inês Coutinho, Angelina Mariano, Argemiro Geraldo, Maria Carmo Macário
Immune checkpoint inhibitors are used in metastatic melanoma with good efficacy and safety profile. We report the first case of an inflammatory demyelinating disease of the central nervous system during treatment with Pembrolizumab and discuss the evidence in the literature supporting its causative role. The patient had a good clinical recovery after intravenous steroids, plasma exchange and discontinuation of Pembrolizumab. Due to the expected increase in the importance of immune checkpoint inhibitors in cancer treatment, it is important to be aware of neurological adverse events, as early treatment usually leads to good clinical responses...
October 12, 2018: Multiple Sclerosis: Clinical and Laboratory Research
Shukoofeh Torabi, Mona Tamaddon, Mojtaba Asadolahi, Gelareh Shokri, Rezvan Tavakoli, Nooshin Tasharrofi, Reza Rezaei, Vahid Tavakolpour, Hossein Sazegar, Fatemeh Kouhkan
MicroRNA-455-5p (miR-455-5p) seems to have an anti-inflammatory role in the immune system since its expression is induced by IL-10 cytokine. Multiple sclerosis (MS) is a chronic demyelinating neurodegenerative disease of the central nervous system that is caused by an autoimmune inflammatory attack against the myelin insulation of neurons. The expression level of miR-455-5p and its role in MS pathogenesis has yet to be elucidated. We found that miR-455-5p expression was highly correlated with disease severity in MS patients...
October 11, 2018: Immunogenetics
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