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https://www.readbyqxmd.com/read/28422829/impact-of-pain-on-cognitive-functions-in-primary-sj%C3%A3-gren-syndrome-with-small-fiber-neuropathy-10-cases-and-a-literature-review
#1
Sandrine Indart, Jacques Hugon, Pierre Jean Guillausseau, Alice Gilbert, Julien Dumurgier, Claire Paquet, Damien Sène
Primary Sjögren syndrome (pSS) is a chronic systemic autoimmune disease characterized by xerophthalmia, xerostomia, and potential peripheral or central neurological involvement. In pSS, the prevalence of cognitive disorders is generally sparse across literature and the impact of pain on cognitive profile is unclear. The aim of this study was to determine the relation between pain, cognitive complaint, and impairment in a very homogenous population of 10 pSS patients with painful small fiber neuropathy (PSFN) and spontaneous cognitive complaint...
April 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28416698/selective-in-vivo-removal-of-pathogenic-anti-mag-autoantibodies-an-antigen-specific-treatment-option-for-anti-mag-neuropathy
#2
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/28412705/interferon-%C3%AE-for-induction-and-maintenance-of-remission-in-eosinophilic-granulomatosis-with-polyangiitis-a-single-center-retrospective-observational-cohort-study
#3
Benjamin Seeliger, Martin Förster, Janett Happe, Thomas Forberg, Anne Moeser, Thomas Neumann, Claus Kroegel
OBJECTIVE: Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by frequent relapses following induction therapy. Interferon-α (IFN-α) can reverse the underlying Th2-driven immune response and has successfully induced remission in previous reports. We undertook this study to investigate its efficacy and safety in patients with EGPA. METHODS: We conducted a retrospective monocentric cohort study including 30 patients (16 women) with active EGPA under IFN-α treatment...
April 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28411165/vitamin-d-and-sj%C3%A3-gren-syndrome
#4
REVIEW
Mario Garcia-Carrasco, Erick Alejandro Jiménez-Herrera, Jose Luis Gálvez-Romero, Luis Vázquez de Lara, Claudia Mendoza-Pinto, Ivet Etchegaray-Morales, Pamela Munguía-Realpozo, Alejandro Ruíz-Argüelles, Rosas Jose, Mauricio Vera-Recabarren, Ricard Cervera
The immunomodulatory effects of vitamin D have been extensively studied in the context of autoimmunity. Multiple studies have demonstrated a high prevalence of vitamin D deficiency in autoimmune diseases. Recently, a possible protective role of vitamin D in autoimmunity has been described; however, this function remains controversial. Few studies have investigated the role of vitamin D in patients with Sjögren syndrome (SS). In this review, we compiled the main features of SS pathogenesis, the vitamin D immunomodulatory effects and the possible interaction between both...
April 12, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28405484/thyroid-associated-ophthalmopathy
#5
REVIEW
Esra Şahlı, Kaan Gündüz
Thyroid-associated ophthalmopathy is the most frequent extrathyroidal involvement of Graves' disease but it sometimes occurs in euthyroid or hypothyroid patients. Thyroid-associated ophthalmopathy is an autoimmune disorder, but its pathogenesis is not completely understood. Autoimmunity against putative antigens shared by the thyroid and the orbit plays a role in the pathogenesis of disease. There is an increased volume of extraocular muscles, orbital connective and adipose tissues. Clinical findings of thyroid-associated ophthalmopathy are soft tissue involvement, eyelid retraction, proptosis, compressive optic neuropathy, and restrictive myopathy...
April 2017: Turkish Journal of Ophthalmology
https://www.readbyqxmd.com/read/28394224/neuro-ophthalmologic-manifestations-in-systemic-lupus-erythematosus
#6
F A de Andrade, G Guimarães Moreira Balbi, L G Bortoloti de Azevedo, G Provenzano Sá, H Vieira de Moraes Junior, E Mendes Klumb, R Abramino Levy
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can affect different ocular structures, such as cornea, conjunctiva, episclera, sclera, uveal tract, retina, optic nerve and vessels. Neuro-ophthalmologic manifestations in SLE include different degrees of involvement of retina, choroid and optic nerve. Retinal changes are one of the most common ocular involvements and are frequently used as clinical criteria for activity, even if isolated. Studies show that up to 29% of patients with active SLE manifest retinal disease...
April 2017: Lupus
https://www.readbyqxmd.com/read/28386159/severe-reactive-ischemic-posterior-segment-inflammation-in-acanthamoeba-keratitis-case-report-of-a%C3%A2-patient-with-sj%C3%A3-gren-s-syndrome
#7
Christoph Palme, Bernhard Steger, Gertrud Haas, Barbara Teuchner, Nikolaos E Bechrakis
PURPOSE: We report on a case of Acanthamoeba keratitis (AK)-related reactive ischemic posterior segment inflammation following intraocular surgery in a patient with primary Sjögren's syndrome (PSS). CASE REPORT: A 48-year-old female patient with severe protracted AK underwent uneventful cataract surgery upon development of a corneal scar. Four weeks postoperatively, she experienced a rapid loss of vision to no light perception. Central retinal artery occlusion and ischemic optic neuropathy could be excluded, and a diagnosis of PSS was made...
2017: Spektrum der Augenheilkunde: Zeitschrift der Österreichischen Ophthalmologischen Gesellschaft, ÖOG
https://www.readbyqxmd.com/read/28379880/immunoresponsive-autonomic-neuropathy-in-sj%C3%A3-gren-syndrome-case-series-and-literature-review
#8
Brent P Goodman
BACKGROUND: Sjögren syndrome (SS) is one of the most common autoimmune disorders that classically affects exocrine glands, resulting in keratoconjunctivitis sicca and xerostomia, and frequently is associated with other systemic symptoms. SS appears to have a particular predilection for involving the autonomic nervous system. STUDY QUESTION: Does immunotherapy improve signs and symptoms of autonomic nervous system impairment in SS? STUDY DESIGN: This is a retrospective review of patients seen in the autonomic clinic at our institution who underwent an evaluation for a suspected autonomic disorder that ultimately was attributed to SS...
March 31, 2017: American Journal of Therapeutics
https://www.readbyqxmd.com/read/28363822/lewis-sumner-syndrome-in-a-patient-with-rheumatoid-arthritis-link-between-rheumatoid-arthritis-and-demyelinating-polyradiculoneuropathies
#9
Joanna Kedra, Violaine Foltz, Karine Viala, Sivy Tan, Bruno Fautrel
Chronic Inflammatory Demyelinating Polyradiculoneuropathies are a group of autoimmune neuropathies with a chronic course. Lewis-Sumner syndrome is a variant of this disease, characterized by an asymmetrical distal and mostly motor involvement, predominating at upper limb. We report the case of a patient who developed almost currently rheumatoid arthritis and Lewis-Sumner syndrome, which raised the problem of therapeutic intensification for his rheumatism when methotrexate proved to be ineffective. Finally, rituximab had been introduced by common consent with neurologists, and the patient noticed an improvement fifteen days after the first infusion...
March 28, 2017: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/28348631/optic-neuropathy-as-the-initial-presenting-sign-of-n-methyl-d-aspartate-nmda-encephalitis
#10
Mark Mugavin, Brett H Mueller, Masoom Desai, Karl C Golnik
A 52-year-old woman presented with painless vision loss for 3 months. She was in custody for allegedly robbing a bank and had recently been diagnosed with paranoid schizophrenia. She had 20/100 VA OD, a 2+RAPD, and optic atrophy. Extensive diagnostic workup including MRI, Fluorescein Angiography, Infectious Disease Panel, lumbar puncture, and leptomeningeal biopsy were unrevealing. Vision in her right eye declined to NLP and her left eye declined to 20/200 VA. Anti N-methyl-D-aspartate (NMDA) Autoimmune Encephalitis was diagnosed based on CSF serology and clinical suspicion...
April 2017: Neuro-ophthalmology
https://www.readbyqxmd.com/read/28329893/graves-ophthalmopathy-in-a-paediatric-population
#11
Y Sadeghi, A Obéric, G Theintz, M Hamédani
Background Graves' disease is an autoimmune disorder leading to hyperthyroidism. It is often associated with ophthalmic manifestations. Graves' disease is reported to be rare in the paediatric population. Patients and Methods We performed a retrospective analysis on all patients diagnosed with ophthalmopathy related to Graves' disease at a paediatric age (less than 18 years old) in our institution between 2004 and 2015. Results Eight patients were identified: 6 females and 2 males. The median age at diagnosis was 11...
March 22, 2017: Klinische Monatsblätter Für Augenheilkunde
https://www.readbyqxmd.com/read/28319561/pediatric-enteric-neuropathies-diagnosis-and-current-management
#12
Maggie L Westfal, Allan M Goldstein
PURPOSE OF REVIEW: Neurointestinal diseases are increasingly recognized as causes of significant gastrointestinal morbidity in children. This review highlights the most common pediatric enteric neuropathies and their diagnosis and management, emphasizing insights and discoveries from the most recent literature available. RECENT FINDINGS: The embryologic and histopathologic causes of enteric neuropathies are varied. They range from congenital aganglionosis in Hirschsprung disease, to autoimmune-mediated loss of neuronal subtypes in esophageal achalasia and Chagas disease, to degenerative neuropathies in some cases of chronic intestinal pseudo-obstruction and gastroparesis...
March 17, 2017: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/28295417/subcutaneous-versus-intravenous-immunoglobulin-for-chronic-autoimmune-neuropathies
#13
EDITORIAL
Elie Naddaf, M Hassan Murad, P James B Dyck
No abstract text is available yet for this article.
March 10, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28284349/4-aminopyridine-ameliorates-experimental-autoimmune-neuritis-in-lewis-rats
#14
Kota Moriguchi, Katsuichi Miyamoto, Susumu Kusunoki
We investigated the effect of 4-aminopyridine (4-AP) on experimental autoimmune neuritis (EAN) using a 4-AP-treated group in which 4-AP was administered in the diet, and a control group (n=10 per group). Electrophysiological and pathological assessment was performed in the sciatic nerve. The EAN clinical scores were significantly lower in the 4-AP-treated group than in the control group (p<0.05). The motor conductance velocity two weeks post-immunization was significantly higher in the 4-AP-treated group (p<0...
April 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28216074/autoimmunity-in-guillain-barr%C3%A3-syndrome-associated-with-zika-virus-infection-and-beyond
#15
REVIEW
Carlos A Pinto-Díaz, Yhojan Rodríguez, Diana M Monsalve, Yeny Acosta-Ampudia, Nicolás Molano-González, Juan-Manuel Anaya, Carolina Ramírez-Santana
Autoimmune diseases share common immunopathogenic mechanisms (i.e., the autoimmune tautology), which explain the clinical similarities among them as well as their familial clustering. Guillain-Barré syndrome (GBS), an autoimmune peripheral neuropathy, has been recently associated with Zika virus (ZIKV) infection. Based on a series of cases, this review article provides a comparative analysis of GBS associated with ZIKV infection, contrasted with the general characteristics of GBS in light of the autoimmune tautology, including gender differences in prevalence, subphenotypes, polyautoimmunity, familial autoimmunity, age at onset, pathophysiology, ecology, genetics, ancestry, and treatment...
February 13, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28216073/autoimmune-neuropathies-associated-to-rheumatic-diseases
#16
REVIEW
Alberto R M Martinez, Ingrid Faber, Anamarli Nucci, Simone Appenzeller, Marcondes C França
Systemic manifestations are frequent in autoimmune rheumatic diseases and include peripheral nervous system damage. Neuron cell body, axons and myelin sheath may all be affected in this context. This involvement results in severe and sometimes disabling symptoms. Sensory, motor and autonomic features may be present in different patterns that emerge as peculiar clinical pictures. Prompt recognition of these neuropathies is pivotal to guide treatment and reduce the risks of long term disability. In this review, we aim to describe the main immune-mediated neuropathies associated to rheumatic diseases: sensory neuronopathies, multiple mononeuropathies and chronic inflammatory demyelinating polyradiculoneuropathy, with an emphasis on clinical features and therapeutic options...
February 12, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28194064/therapeutic-plasma-exchange-in-patients-with-neurologic-disorders-review-of-63-cases
#17
Anil Tombak, Mehmet Ali Uçar, Aydan Akdeniz, Arda Yilmaz, Hakan Kaleagası, Mehmet Ali Sungur, Eyup Naci Tiftik
Therapeutic plasma exchange (TPE) is a procedure that reduces circulating autoantibodies of the patients. TPE is commonly used in neurological disorders where autoimmunity plays a major role. We report our experience with regard to the indications, adverse events and outcomes of plasma exchange in neurological disorders. Sixty-three patients were included to this retrospective study. Median age was 48 years (range 1-85), there was a predominance of males. Neurological indications included Guillain-Barrè syndrome (n = 22), myasthenia gravis (n = 21), chronic inflammatory demyelinating polyneuropathy (n = 7), polymyositis (n = 3), multifocal motor neuropathy (n = 2), acute disseminated encephalomyelitis (n = 2), neuromyelitis optica (n = 2), multiple sclerosis (n = 2), limbic encephalitis (n = 1) and transverse myelitis (n = 1)...
March 2017: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/28181561/peripheral-sensory-neuron-injury-contributes-to-neuropathic-pain-in-experimental-autoimmune-encephalomyelitis
#18
I-Ching Wang, Chen-Yen Chung, Fang Liao, Chih-Cheng Chen, Cheng-Han Lee
Multiple sclerosis (MS)-induced neuropathic pain deteriorates quality of life in patients but is often refractory to treatment. In experimental autoimmune encephalomyelitis (EAE), a rodent model of MS, animals develop neuropathy and inflammation-induced tissue acidosis, which suggests the involvement of acid-sensing ion channels (ASICs). Also, peripheral neuropathy is reported in MS patients. However, the involvement of the peripheral nervous system (PNS) in MS neuropathic pain remains elusive. This study investigated the contribution of ASICs and peripheral neuropathy in MS-induced neuropathic pain...
February 9, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28069788/proteinase-3-antineutrophil-cytoplasmic-antibody-positive-ulcerative-colitis-presenting-with-abducens-neuropathy
#19
Yuki Kirito, Daisuke Yamamoto, Tsuyoshi Uchiyama
A 72-year-old man with ulcerative colitis (UC) presented with complete left abducens nerve palsy. Although MRI showed no significant changes, cerebrospinal fluid analysis revealed pleocytosis and elevated protein and interleukin (IL)-6 levels. His serum proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) level was also elevated to 31.1 U/mL, but granulomatosis with polyangiitis was not observed. On the basis of the diagnosis of autoimmune cranial neuropathy, he was treated with steroid therapy. While tapering steroid therapy, his serum PR3-ANCA levels; cerebrospinal fluid findings, including IL-6 levels; and symptoms improved...
January 9, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28067631/schwann-cells-are-activated-by-atp-released-from-neurons-in-an-in-vitro-cellular-model-of-miller-fisher-syndrome
#20
Umberto Rodella, Samuele Negro, Michele Scorzeto, Elisanna Bergamin, Kees Jalink, Cesare Montecucco, Nobuhiro Yuki, Michela Rigoni
The neuromuscular junction is exposed to different types of insults including mechanical traumas, toxins or autoimmune antibodies and, accordingly, has retained through evolution a remarkable ability to regenerate. Regeneration is driven by multiple signals that are exchanged among the cellular components of the junction. These signals are largely unknown.Miller Fisher syndrome is a variant of Guillain-Barré syndrome caused by autoimmune antibodies specific for epitopes of peripheral axon terminals. Using an animal model of Miller Fisher syndrome, we recently reported that a monoclonal anti-polysialoganglioside GQ1b antibody plus complement damages nerve terminals with production of mitochondrial hydrogen peroxide, that activates Schwann cells...
January 6, 2017: Disease Models & Mechanisms
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