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https://www.readbyqxmd.com/read/28819062/nodes-paranodes-and-neuropathies
#1
REVIEW
Janev Fehmi, Steven S Scherer, Hugh J Willison, Simon Rinaldi
This review summarises recent evidence supporting the involvement of the specialised nodal and perinodal domains (the paranode and juxtaparanode) of myelinated axons in the pathology of acquired, inflammatory, peripheral neuropathies.The identification of new target antigens in the inflammatory neuropathies heralds a revolution in diagnosis, and has already begun to inform increasingly targeted and individualised therapies. Rapid progress in our basic understanding of the highly specialised nodal regions of peripheral nerves serves to strengthen the links between their unique microstructural identities, functions and pathologies...
August 17, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28789710/transplanted-human-fecal-microbiota-enhanced-guillain-barr%C3%A3-syndrome-autoantibody-responses-after-campylobacter-jejuni-infection-in-c57bl-6-mice
#2
Phillip T Brooks, Kelsey A Brakel, Julia A Bell, Christopher E Bejcek, Trey Gilpin, Jean M Brudvig, Linda S Mansfield
BACKGROUND: Campylobacter jejuni is the leading antecedent infection to the autoimmune neuropathy Guillain-Barré syndrome (GBS), which is accompanied by an autoimmune anti-ganglioside antibody attack on peripheral nerves. Previously, we showed that contrasting immune responses mediate C. jejuni induced colitis and autoimmunity in interleukin-10 (IL-10)-deficient mice, dependent upon the infecting strain. Strains from colitis patients elicited T helper 1 (TH1)-dependent inflammatory responses while strains from GBS patients elicited TH2-dependent autoantibody production...
August 8, 2017: Microbiome
https://www.readbyqxmd.com/read/28741102/the-range-of-neurological-complications-in-chikungunya-fever
#3
T Cerny, M Schwarz, U Schwarz, J Lemant, P Gérardin, E Keller
BACKGROUND: Chikungunya fever is a globally spreading mosquito-borne disease that shows an unexpected neurovirulence. Even though the neurological complications have been a major cause of intensive care unit admission and death, to date, there is no systematic analysis of their spectrum available. OBJECTIVE: To review evidence of neurological manifestations in Chikungunya fever and map their epidemiology, clinical spectrum, pathomechanisms, diagnostics, therapies and outcomes...
May 25, 2017: Neurocritical Care
https://www.readbyqxmd.com/read/28736206/bpag1-in-muscles-structure-and-function-in-skeletal-cardiac-and-smooth-muscle
#4
REVIEW
Masao Horie, Nozomu Yoshioka, Hirohide Takebayashi
BPAG1, also known as Dystonin or BP230, belongs to the plakin family of proteins, which has multiple cytoskeleton-binding domains. Several BPAG1 isoforms are produced by a single BPAG1 genomic locus using different promoters and exons. For example, BPAG1a, BPAG1b, and BPAG1e are predominantly expressed in the nervous system, muscle, and skin, respectively. Among BPAG1 isoforms, BPAG1e is well studied because it was first identified as an autoantigen in patients with bullous pemphigoid, an autoimmune skin disease...
July 20, 2017: Seminars in Cell & Developmental Biology
https://www.readbyqxmd.com/read/28726788/neuroprotection-by-eif2%C3%AE-chop-inhibition-and-xbp-1-activation-in-eae-optic-neuritiss
#5
Haoliang Huang, Linqing Miao, Feisi Liang, Xiaodong Liu, Lin Xu, Xiuyin Teng, Qizhao Wang, William H Ridder, Kenneth S Shindler, Yang Sun, Yang Hu
No therapies exist to prevent neuronal deficits in multiple sclerosis (MS), because the molecular mechanism responsible for the progressive neurodegeneration is unknown. We previously showed that axon injury-induced neuronal endoplasmic reticulum (ER) stress plays an important role in retinal ganglion cell (RGC) death and optic nerve degeneration in traumatic and glaucomatous optic neuropathies. Optic neuritis, one of the most common clinical manifestations of MS, is readily modeled by experimental autoimmune encephalomyelitis (EAE) in mouse...
July 20, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28708133/autoantibodies-in-chronic-inflammatory-neuropathies-diagnostic-and-therapeutic-implications
#6
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/28681964/a-shared-comparison-of-diabetes-mellitus-and-neurodegenerative-disorders
#7
Mahmoud Morsi, Ahmed Maher, Ahmed Metwally, Omnia Abo-Elmagd, Dina Johar, Larry Bernstein
Diabetes Mellitus (DM) is one of the most common diseases in the world population, associated with obesity, pancreatic endocrine changes, cardiovascular disease, renal glomerular disease, cerebrovascular disease, peripheral neuropathy, neurodegenerative disease, retinal disease, sleep apnea, some of which are bundled into the metabolic syndrome. The main characteristic of this disease is hyperglycemia, and often with albuminuria. Nevertheless, the classic features, with ketoacidosis in the extreme, are only a first layer of description of this condition...
July 6, 2017: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/28680965/a-brain-derived-neurotrophic-factor-based-p75-ntr-peptide-mimetic-ameliorates-experimental-autoimmune-neuritis-induced-axonal-pathology-and-demyelination
#8
David G Gonsalvez, Giang Tran, Jessica L Fletcher, Richard A Hughes, Suzanne Hodgkinson, Rhiannon J Wood, Sang Won Yoo, Mithraka De Silva, Wong W Agnes, Catriona McLean, Paul Kennedy, Trevor J Kilpatrick, Simon S Murray, Junhua Xiao
Axonal damage and demyelination are major determinants of disability in patients with peripheral demyelinating neuropathies. The neurotrophin family of growth factors are essential for the normal development and myelination of the peripheral nervous system (PNS), and as such are potential therapeutic candidates for ameliorating axonal and myelin damage. In particular, BDNF promotes peripheral nerve myelination via p75 neurotrophin receptor (p75(NTR)) receptors. Here, we investigated the therapeutic efficacy of a small structural mimetic of the region of BDNF that binds to p75(NTR) (cyclo-dPAKKR) in experimental autoimmune neuritis (EAN), an established animal model of peripheral demyelinating neuropathy...
May 2017: ENeuro
https://www.readbyqxmd.com/read/28680531/mitochondrial-uncoupler-prodrug-of-2-4-dinitrophenol-mp201-prevents-neuronal-damage-and-preserves-vision-in-experimental-optic-neuritis
#9
Reas S Khan, Kimberly Dine, John G Geisler, Kenneth S Shindler
The ability of novel mitochondrial uncoupler prodrug of 2,4-dinitrophenol (DNP), MP201, to prevent neuronal damage and preserve visual function in an experimental autoimmune encephalomyelitis (EAE) model of optic neuritis was evaluated. Optic nerve inflammation, demyelination, and axonal loss are prominent features of optic neuritis, an inflammatory optic neuropathy often associated with the central nervous system demyelinating disease multiple sclerosis. Currently, optic neuritis is frequently treated with high-dose corticosteroids, but treatment fails to prevent permanent neuronal damage and associated vision changes that occur as optic neuritis resolves, thus suggesting that additional therapies are required...
2017: Oxidative Medicine and Cellular Longevity
https://www.readbyqxmd.com/read/28667708/acute-painful-autoimmune-neuropathy-a-variant-of-guillain-barr%C3%A3-syndrome
#10
Nobuhiro Yuki, Amanda C Chan, Anna Hiu Yi Wong, Takayuki Inoue, Masafumi Yokai, Takashi Kurihara, JérôMe J Devaux, Einar Wilder-Smith
INTRODUCTION: We present a painful small-fiber neuropathy variant of Guillain-Barré syndrome characterized by antecedent infectious symptoms, hyporeflexia, and albuminocytologic dissociation. METHODS: Two patients received intravenous immunoglobulin, one corticosteroids. RESULTS: The patients subsequently improved. Immunoglobulin G (IgG) antibodies in their acute phase sera strongly bound to murine small nerve fibers, and the binding disappeared during the convalescent phase...
July 1, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28644258/multifocal-motor-neuropathy-associated-with-infliximab-a-case-report-and-a-literature-review
#11
Ayse O Bayrak, Hasan Ulusoy, Necdet Bolat, Baki Doğan, Taner Ozbenli
Multifocal motor neuropathy with conduction block (MMN-CB) is purely a motor neuropathy with progressive weakness that is characteristically caused by conduction blocks. Association with antiganglioside antibodies and a good response to immunomodulating therapies suggest an autoimmune etiology. In rare cases, MMN-CB has been reported as an adverse effect of infliximab, a tumor necrosis factor-α blocker. We present a case of MMN-CB due to infliximab in a 45-year-old man with psoriatic arthritis who was exposed to the drug for 2 years because of a delayed diagnosis...
July 2017: Neurologist
https://www.readbyqxmd.com/read/28640813/identification-of-a-sj%C3%A3-gren-s-syndrome-susceptibility-locus-at-oas1-that-influences-isoform-switching-protein-expression-and-responsiveness-to-type-i-interferons
#12
He Li, Tove Ragna Reksten, John A Ice, Jennifer A Kelly, Indra Adrianto, Astrid Rasmussen, Shaofeng Wang, Bo He, Kiely M Grundahl, Stuart B Glenn, Corinne Miceli-Richard, Simon Bowman, Sue Lester, Per Eriksson, Maija-Leena Eloranta, Johan G Brun, Lasse G Gøransson, Erna Harboe, Joel M Guthridge, Kenneth M Kaufman, Marika Kvarnström, Deborah S Cunninghame Graham, Ketan Patel, Adam J Adler, A Darise Farris, Michael T Brennan, James Chodosh, Rajaram Gopalakrishnan, Michael H Weisman, Swamy Venuturupalli, Daniel J Wallace, Kimberly S Hefner, Glen D Houston, Andrew J W Huang, Pamela J Hughes, David M Lewis, Lida Radfar, Evan S Vista, Contessa E Edgar, Michael D Rohrer, Donald U Stone, Timothy J Vyse, John B Harley, Patrick M Gaffney, Judith A James, Sean Turner, Ilias Alevizos, Juan-Manuel Anaya, Nelson L Rhodus, Barbara M Segal, Courtney G Montgomery, R Hal Scofield, Susan Kovats, Xavier Mariette, Lars Rönnblom, Torsten Witte, Maureen Rischmueller, Marie Wahren-Herlenius, Roald Omdal, Roland Jonsson, Wan-Fai Ng, Gunnel Nordmark, Christopher J Lessard, Kathy L Sivils
Sjögren's syndrome (SS) is a common, autoimmune exocrinopathy distinguished by keratoconjunctivitis sicca and xerostomia. Patients frequently develop serious complications including lymphoma, pulmonary dysfunction, neuropathy, vasculitis, and debilitating fatigue. Dysregulation of type I interferon (IFN) pathway is a prominent feature of SS and is correlated with increased autoantibody titers and disease severity. To identify genetic determinants of IFN pathway dysregulation in SS, we performed cis-expression quantitative trait locus (eQTL) analyses focusing on differentially expressed type I IFN-inducible transcripts identified through a transcriptome profiling study...
June 2017: PLoS Genetics
https://www.readbyqxmd.com/read/28620980/case-of-autoimmune-autonomic-ganglionopathy-manifesting-anhidrosis
#13
Asuka Yoshifuku, Koichi Yoneda, Yusuke Sakiyama, Osamu Higuchi, Shunya Nakane, Takuro Kanekura
Autoimmune autonomic ganglionopathy (AAG), clinically characterized by gastrointestinal dysmotility, orthostatic hypotension and tonic pupils, is an idiopathic acquired disorder of the autonomic nervous system elicited by antibodies against ganglionic acetylcholine receptor (gAChR). We encountered a 60-year-old man who presented with severe anhidrosis, difficulty in thermoregulation, orthostatic hypotension, gastrointestinal dysmotility, tonic pupils and ptosis. Histologically, an anhidrotic skin sample was normal...
June 16, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28606272/-progression-of-the-pathogenesis-of-thyroid-associated-ophthalmopathy
#14
L Wang, J M Ma
Thyroid associated ophthalmopathy (TAO) is a common complication of Graves' disease (GD). The clinical manifestations are mainly swelling and retracted eyelid, protopsis and limited eye movements, and optic neuropathy can be seen in severe cases. The mechanism of TAO is not yet clear. A large number of studies have found that the disorders of autoimmunity play an important role in TAO, while genetic factors, oxidative stress, and environmental factors such as smoking participate in it. Therefore, we consult a large number of studies and expound the pathogenesis from the aspects of genetic susceptibility, immune mechanism, oxidative stress, and the effects of smoking to provide referrences for clinical associated studies...
June 11, 2017: [Zhonghua Yan Ke za Zhi] Chinese Journal of Ophthalmology
https://www.readbyqxmd.com/read/28601289/anti-inflammatory-and-immunomodulatory-potential-of-human-immunoglobulin-applied-intrathecally-in-lewis-rat-experimental-autoimmune-neuritis
#15
Kalliopi Pitarokoili, Felix Kohle, Jeremias Motte, Oluwaseun Fatoba, Xiomara Pedreiturria, Ralf Gold, Min-Suk Yoon
Intravenous human immunoglobulins dominate in the treatment of autoimmune neuropathies. We introduce intrathecal application as a new option for experimental autoimmune neuritis in Lewis rats. After immunisation with neuritogenic P2 peptide, we show a therapeutic and preventive effect of intrathecal human immunoglobulins (5-40mg/kg) on clinical and electrophysiological neuritis signs. Histology corroborated a lower degree of inflammation, demyelination, ICAM-1-dependent blood-nerve-barrier permeability and complement activation in the sciatic nerve...
August 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28597710/comparing-treatment-options-for-chronic-inflammatory-neuropathies-and-choosing-the-right-treatment-plan
#16
Eduardo Nobile-Orazio, Francesca Gallia, Fabrizia Terenghi, Mariangela Bianco
Chronic inflammatory neuropathies are disorders caused by an immune response to peripheral nerve. They include chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy (MMN) and neuropathy associated with anti-MAG IgM monoclonal gammopathy and other less frequent neuropathies. Several immune therapies have been proven to be effective in these neuropathies even if the best therapeutic option is still unsettled. Areas covered: The authors reviewed the literature to compare the efficacy and safety of currently used immune therapies in these neuropathies...
June 26, 2017: Expert Review of Neurotherapeutics
https://www.readbyqxmd.com/read/28588791/synchronous-adie-s-syndrome-and-type-1-antineuronal-nuclear-antibody-anti-hu-related-paraneoplastic-neurological-syndromes-as-predictors-of-complete-response-in-limited-stage-small-cell-lung-cancer-a-case-report
#17
Katherina Bernadette Sreter, Blazenka Barisic, Marija Barisic Kutija, Suzana Kukulj, Miroslav Samarzija
Adie's syndrome (AS) and paraneoplastic sensorimotor neuropathy with cerebellar ataxia (PSN CA) are extremely rare, rapidly progressive, autoimmune diseases associated with the development of antibodies against neuronal-specific Hu proteins that are abnormally expressed in small-cell lung cancer (SCLC). We herein present the unique case of a 55-year-old obese woman, previous heavy smoker, who, during treatment with standard cisplatin-etoposide chemotherapy for limited-stage SCLC, developed simultaneous AS and worsening symptoms consistent with PSN CA that led to significant neurological disability and severe axonal electrophysiological pattern on nerve conduction studies...
June 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28570327/gastroenterology-and-neurology
#18
Ronald F Pfeiffer
PURPOSE OF REVIEW: Just as gastrointestinal dysfunction may develop in the setting of neurologic disease, neurologic dysfunction may become evident in the setting of gastrointestinal disease. This article describes the range of neurologic features that have been described in three primary gastrointestinal diseases: celiac disease and gluten-related disorders, inflammatory bowel disease, and Whipple disease. Particular emphasis is placed on the controversial and evolving clinical picture of neurologic dysfunction in disorders of gluten sensitivity...
June 2017: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/28500433/the-effect-of-orbital-radiation-therapy-on-thyroid-associated-orbitopathy-complicated-with-dysthyroid-optic-neuropathy
#19
REVIEW
Yang Wang, Huifang Zhou, Xianqun Fan
Thyroid-associated orbitopathy (TAO) is an inflammatory autoimmune disorder. The most serious complication of TAO is dysthyroid optic neuropathy (DON), which can lead to permanent vision loss because of volume expansion in the orbital apex. Orbital radiation therapy (ORT) is an anti-inflammatory treatment used in the treatment of active TAO. Clinical studies support radiotherapy as having a modest effect on DON, and early radiotherapy may protect against disease progression to DON. Current studies suggest that radiotherapy is generally safe...
May 13, 2017: Frontiers of Medicine
https://www.readbyqxmd.com/read/28487747/a-predictive-model-for-guillain-barr%C3%A3-syndrome-based-on-single-learning-algorithms
#20
Juana Canul-Reich, Juan Frausto-Solís, José Hernández-Torruco
Background. Guillain-Barré Syndrome (GBS) is a potentially fatal autoimmune neurological disorder. The severity varies among the four main subtypes, named as Acute Inflammatory Demyelinating Polyneuropathy (AIDP), Acute Motor Axonal Neuropathy (AMAN), Acute Motor Sensory Axonal Neuropathy (AMSAN), and Miller-Fisher Syndrome (MF). A proper subtype identification may help to promptly carry out adequate treatment in patients. Method. We perform experiments with 15 single classifiers in two scenarios: four subtypes' classification and One versus All (OvA) classification...
2017: Computational and Mathematical Methods in Medicine
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