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demyelinating disorder

Panagiotis Zis, Marios Hadjivassiliou, Ptolemaios G Sarrigiannis, Thomas M Jenkins, Dimos-Dimitrios Mitsikostas
INTRODUCTION: Nocebo is very prevalent among neurological disorders, resulting in low adherence and treatment outcome. We sought to examine the adverse events (AE) following placebo administration in placebo-controlled randomized clinical trials (RCTs) for chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: After a systematic literature search for RCTs for CIDP pharmacotherapy treatments, we assessed the number of AE in the placebo groups and the number discontinuations because of placebo intolerance...
May 15, 2018: Journal of the Neurological Sciences
Sheng Zhang, Abeer Rasai, Yan Wang, Jie Xu, Peter Bannerman, Daffcar Erol, Danayit Tsegaye, Aijun Wang, Athena Soulika, Xiangjiang Zhan, Fuzheng Guo
Myelination in the central nervous system takes place predominantly during the postnatal development of humans and rodents by myelinating oligodendrocytes (OLs), which are differentiated from oligodendrocyte progenitor cells (OPCs). We recently reported that Sox2 is essential for developmental myelination in the murine brain and spinal cord. It is still controversial regarding the role of Sox2 in oligodendroglial lineage progression in the postnatal murine spinal cord. Analyses of a series of cell- and stage-specific Sox2 mutants reveal that Sox2 plays a biphasic role in regulating oligodendroglial lineage progression in the postnatal murine spinal cord...
April 5, 2018: Molecular Neurobiology
Martin S Weber, Tobias Derfuss, Imke Metz, Wolfgang Brück
Extensive research over the last decades basically failed to identify a common cause of noninfectious inflammatory central nervous system (CNS) demyelinating disease. To a great extent, this may reflect that the group of inflammatory CNS demyelinating disorders likely contains multiple pathogenetically distinct disease entities. Indeed, the greatest success so far in deciphering the pathogenesis of a CNS demyelinating disorder resulted from the discovery of anti-aquaporin (AQP)-4 antibodies (ab), which allowed progressive delineation of neuromyelitis optica (NMO), formerly considered a variant of the most common CNS demyelinating disorder, multiple sclerosis (MS), as a distinct disease...
2018: Therapeutic Advances in Neurological Disorders
Tingting Lu, Yaqing Shu, Yongqiang Dai, Xu Liu, Yanyu Chang, Qiao Huang, Allan G Kermode, Wei Qiu
Multiple sclerosis and neuromyelitis optica spectrum disorder are currently thought to be independent entities. Some patients display intermediate manifestations that fit the criteria for both diseases without positive relevant serobiomarkers. An overall standard and consensus for the diagnosis and treatment of these overlapping patients have not been reached. We describe a patient with frequently relapsing demyelinating episodes and repeatedly adjusted treatment regimens due to diagnostic difficulties. This case did not respond adequately to glucocorticoid plus azathioprine or to interferon...
March 26, 2018: Multiple Sclerosis and related Disorders
Karla Cervantes-Gracia, Holger Husi
Multiple sclerosis (MS) is a chronic autoimmune disorder characterized by inflammatory-demyelinating events in the central nervous system. Despite more than 40 years of MS research its aetiology remains unknown. This study aims to identify the most frequently reported and consistently regulated molecules in MS in order to generate molecular interaction networks and thereby leading to the identification of deregulated processes and pathways which could give an insight of the underlying molecular mechanisms of MS...
April 4, 2018: Scientific Reports
Petra Huehnchen, Wolfgang Boehmerle, Matthias Endres
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune disorder, which causes progressive sensory and motor deficits and often results in severe disability. Knockout of the co-stimulatory protein CD86 in mice of the non-obese diabetic background (NoD.129S4-Cd86tm1Shr /JbsJ) results in the development of a spontaneous autoimmune peripheral polyneuropathy (SAPP). We used this previously described transgenic model to study the effects of the sphingosine-1-phosphate receptor agonist fingolimod on SAPP symptoms, functional and electrophysiological characteristics...
April 4, 2018: Scientific Reports
Ioan-Cristian Lupescu, Adriana Octaviana Dulamea
Chronic inflammatory demyelinating polyneuropathy is an acquired, presumably immune-mediated peripheral neuropathy, characterized by symmetric sensory-motor involvement. Although most often idiopathic, it has been described in association with several disorders, sometimes improving under treatment. We present the case of a 57-year-old male who was admitted to hospital for paresthesias and muscle weakness affecting both upper limbs, initially only the hands, but with worsening and ascending progression during the last three years...
December 2017: Mædica
Fengna Chu, Mingchao Shi, Chao Zheng, Donghui Shen, Jie Zhu, Xiangyu Zheng, Li Cui
Multiple sclerosis (MS) is an autoimmune and neurodegenerative disorder characterized by chronic inflammation, demyelination, as well as axonal and neuronal loss in the central nervous system (CNS). Macrophages and microglia are important components of the innate immune system. They participate in the primary response to microorganisms and play a role in inflammatory responses, homeostasis, and tissue regeneration. In the initial phase of MS and experimental autoimmune encephalomyelitis (EAE), an animal model of MS, macrophages from peripheral tissues infiltrate into the CNS and, together with residential microglia, contribute to the pathogenesis of MS...
May 15, 2018: Journal of Neuroimmunology
Susan Morgello
Primary human immunodeficiency virus (HIV) neuropathologies can affect all levels of the neuraxis and occur in all stages of natural history disease. Some, like HIV encephalitis, HIV myelitis, and diffuse infiltrative lymphocytosis of peripheral nerve, reflect productive infection of the nervous system; others, like vacuolar myelopathy, distal symmetric polyneuropathy, and central and peripheral nervous system demyelination, are not clearly related to regional viral replication, and reflect more complex cascades of dysregulated host immunity and metabolic dysfunction...
2018: Handbook of Clinical Neurology
Ping-Chang Kuo, Dennis A Brown, Barbara A Scofield, Hallel C Paraiso, Pei-Yu Wang, I-Chen Yu, Jui-Hung Yen
Multiple sclerosis (MS) is an autoimmune disorder characterized by the central nervous system (CNS) infiltration of myelin-specific pathogenic T cells followed by brain inflammation in association with demyelination. Similarly, experimental autoimmune encephalomyelitis (EAE), the animal model of MS, also exhibits increased CNS infiltration of pathogenic T cells, including Th1 and Th17, leading to detrimental effects of neuroinflammation and demyelination. We previously reported that 3H-1,2-dithiole-3-thione (D3T), the structurally-simplest of the sulfur-containing dithiolethiones, exerted a promising therapeutic effect in EAE...
March 28, 2018: Brain, Behavior, and Immunity
Lucas Alessandro, José M Pastor Rueda, Miguel Wilken, Luis A Querol Gutiérrez, Mariano Marrodán, Julián N Acosta, Alberto Rivero, Fabio Barroso, Mauricio F Farez
Acute Inflammatory Demyelinating Polyneuropathy (AIDP) and Acute-onset Chronic Inflammatory Demyelinating Polyneuropathy (A-CIDP) are conditions presenting overlapping clinical features during early stages (first 4 weeks), although the latter may progress after 8 weeks. The aim of this study was to identify predictive factors contributing to their differential diagnosis. Clinical records of adult patients with AIDP or A-CIDP diagnosed at our institution between January-2006 and July-2017 were retrospectively reviewed...
March 30, 2018: Journal of the Peripheral Nervous System: JPNS
Kimberley D Bruce, Sachi Gorkhali, Katherine Given, Alison M Coates, Kristen E Boyle, Wendy B Macklin, Robert H Eckel
Severe demyelinating disorders of the central nervous system (CNS) such as multiple sclerosis (MS), can be devastating for many young lives. To date, the factors resulting in poor remyelination and repair are not well understood, and reparative therapies that benefit MS patients have yet to be developed. We have previously shown that the activity and abundance of Lipoprotein Lipase (LPL)-the rate-limiting enzyme in the hydrolysis of triglyceride-rich lipoproteins-is increased in Schwann cells and macrophages following nerve crush injury in the peripheral nervous system (PNS), suggesting that LPL may help scavenge myelin-derived lipids...
2018: Frontiers in Molecular Neuroscience
Edith Segura-Anaya, Rommel Flores-Miranda, Alejandro Martínez-Gómez, Myrna A R Dent
BACKGROUND: The Golgi silver method has been widely used in neuroscience for the study of normal and pathological morphology of neurons. The method has been steadily improved and Bielschowsky's silver staining method (BSSM) is widely used in various pathological conditions, like Alzheimer's disease. NEW METHOD: In this work, teased sciatic nerves were silver impregnated using BSSM. We also developed simultaneous staining by silver impregnation and single- or double-immunofluorescence of the same section in teased nerve preparations...
March 26, 2018: Journal of Neuroscience Methods
Olivier Simon, Blandine Acket, Carole Forfait, Dominique Girault, Ann-Claire Gourinat, Pauline Millon, Maguy Daures, Jessica Vanhomwegen, Segolene Billot, Antoine Biron, Damien Hoinard, Elodie Descloux, David Guyon, Jean Claude Manuguerra, Sylvie Laumond, Nicolas Molko, Myrielle Dupont-Rouzeyrol
Zika virus (ZIKV) infection has been associated with neurologic disorders including Guillain-Barré syndrome (GBS). In New Caledonia during the ZIKV outbreak (2014-2015), case-control and retrospective studies have been performed to assess the link between ZIKV and GBS. Among the 15 cases included, 33% had evidence of a recent ZIKV infection compared to only 3.3% in the 30 controls involved. All patients were Melanesian, had facial diplegia and similar neurophysiological pattern consistent with acute inflammatory demyelinating polyneuropathy, and recovered well...
March 28, 2018: Journal of Neurovirology
Ming Lu, Bruce V Taylor, Heinrich Körner
Vitamin D has a plethora of functions that are important for the maintenance of general health and in particular, the functional integrity of the immune system, such as promoting an anti-inflammatory cytokine profile and reducing the Treg/Th17 ratio. Multiple sclerosis (MS) is a chronic, inflammatory, and neurodegenerative central nervous system (CNS) disorder of probable autoimmune origin. MS is characterized by recurring or progressive demyelination and degeneration of the CNS due in part to a misguided immune response to as yet undefined (CNS) antigens, potentially including myelin basic protein and proteolipid protein...
2018: Frontiers in Immunology
Lena Sagi-Dain, Lilach Shemer, Nathanel Zelnik, Yusri Zoabi, Orit Sadeh, Vardit Adir, Aharon Schif, Amir Peleg
BACKGROUND: Charcot-Marie-Tooth (CMT) is a heterogeneous group of progressive disorders, characterized by chronic motor and sensory polyneuropathy. This hereditary disorder is related to numerous genes and varying inheritance patterns. Thus, many patients do not reach a final genetic diagnosis. PATIENT: We describe a 13-years old girl presenting with progressive bilateral leg weakness and gait instability. Extensive laboratory studies and spinal Magnetic resonance imaging scan were normal...
March 26, 2018: Journal of the Peripheral Nervous System: JPNS
Xiao-Lu Yuan, Yi-Peng Zhao, Jie Huang, Jun-Chen Liu, Wen-Qian Mao, Jun Yin, Bi-Wen Peng, Wan-Hong Liu, Song Han, Xiao-Hua He
AIM: Multiple sclerosis (MS) is a neurological autoimmune disorder characterized by mistaken attacks of inflammatory cells against the central nervous system (CNS), resulting in demyelination and axonal damage. Kv1.3 channel blockers can inhibit T-cell activation and have been designed for MS therapy. However, little is known about the effects of Kv1.3 blockers on protecting myelin sheaths/axons in MS. This study aimed at investigating the neuroprotection efficacy of a selective Kv1.3 channel blocker ImKTx88 (ImK) in MS animal model...
March 25, 2018: CNS Neuroscience & Therapeutics
Maren Lindner, Luisa Klotz, Heinz Wiendl
It is widely accepted that development of autoimmunity in the central nervous system (CNS) is triggered by autoreactive T cells, that are activated in the periphery and gain the capacity to migrate through endothelial cells at the blood-brain-barrier (BBB) into the CNS. Upon local reactivation, an inflammatory cascade is initiated, that subsequently leads to a recruitment of additional immune cells ultimately causing demyelination and axonal damage. Even though the interaction of immune cells with the BBB has been in the focus of research for many years, the exact mechanisms of how immune cells enter and exit the CNS remains poorly understood...
March 25, 2018: Journal of Neurochemistry
Farhat Husain, Gabriel Pardo, Meheroz Rabadi
PURPOSE OF REVIEW: The purpose of this review was to discuss the prevalence, impact, pathophysiology, and treatment of headaches (H/As) in patients with multiple sclerosis (MS). RECENT FINDINGS: Headaches and multiple sclerosis are more common in women than in men with the ratio of female to male being 3:1. It is not entirely clear if there is a correlation or an incidental comorbidity of two neurological conditions. A review of the literature shows a variable prevalence of H/As in MS patients...
March 24, 2018: Current Treatment Options in Neurology
Maxime Vande Vyver, Roel Beelen, Jacques De Keyser, Guy Nagels, Anne-Marie Van Binst, Christiaan Verborgh, Miguel D'haeseleer
BACKGROUND: Multiple sclerosis is an inflammatory demyelinating disorder of the central nervous system (CNS). Myelin basic protein (MBP), which is one of the main compounds of CNS myelin, appears to be hypercitrullinated in the brain of patients with MS. We hypothesized that MS is associated with an increased release of citrulline from the brain. METHODS: Twenty-five patients with MS, 25 controls without neurological disease (CwND) and 25 subjects with non-MS cerebral white matter lesions were included in this study...
April 15, 2018: Journal of the Neurological Sciences
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