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https://www.readbyqxmd.com/read/28329893/graves-ophthalmopathy-in-a-paediatric-population
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
https://www.readbyqxmd.com/read/28030545/longitudinal-analysis-of-serum-autoantibody-reactivities-in-patients-with-primary-open-angle-glaucoma-and-optic-disc-hemorrhage
#11
Katrin Lorenz, Sabine Beck, Munir M Keilani, Joanna Wasielica-Poslednik, Norbert Pfeiffer, Franz H Grus
BACKGROUND: The aim of our current investigation was to analyze the autoantibody-reactivities of primary open angle glaucoma patients with optic disc hemorrhage as possibly correlated to disease progression by means of a protein microarray approach. METHODS: Sera of patients with primary open angle glaucoma and optic disc hemorrhage (n = 16) were collected directly after study inclusion (0 weeks) and after 2 weeks, 4 weeks and 12 weeks. As a control group patients with primary open angle glaucoma (n = 18) were used (0 weeks and 12 weeks)...
2016: PloS One
https://www.readbyqxmd.com/read/28002820/zika-virus-infection-in-the-elderly-possible-relationship-with-guillain-barr%C3%A3-syndrome
#12
Wilson Savino, Carolina V Messias, Daniella A Mendes-da-Cruz, Pamela Passos, Ana Carolina A F Ferreira, Osvaldo J Nascimento
The Zika virus (ZIKV) outbreak in French Polynesia, in 2013, and in Brazil, in 2015, was correlated with neurological complications, which comprised, among others, congenital microcephaly and Guillain-Barré syndrome (GBS), which includes a group of acute autoimmune neuropathies generally reported after respiratory or gastrointestinal infectious diseases. Despite being relatively rare, the incidence rate of GBS rises with age, which makes GBS more frequent in the elderly, in whom it is also a more severe disease with slower recovery than in younger patients...
December 22, 2016: Gerontology
https://www.readbyqxmd.com/read/27997917/cerebrospinal-fluid-findings-in-neurological-diseases-associated-with-sj%C3%A3-gren-s-syndrome
#13
Kaweh Pars, Refik Pul, Philipp Schwenkenbecher, Kurt-Wolfram Sühs, Ulrich Wurster, Torsten Witte, Paul Bronzlik, Martin Stangel, Thomas Skripuletz
BACKGROUND: Sjögren's syndrome is a chronic autoimmune-mediated disease that can cause a variety of neurological manifestations. METHODS: This study investigated characteristics of clinical and cerebrospinal fluid (CSF) features in patients with neurological diseases associated with Sjögren's syndrome. Eighty-two patients were examined separately according to the presence of Sjögren's syndrome alone or in combination with other autoimmune diseases. RESULTS: In the 47 patients with primary Sjögren's syndrome, peripheral neuropathy (57%) was found most frequently, followed by the involvement of the central nervous system (CNS; 17%), cranial neuropathy (15%), and myalgia (11%)...
2017: European Neurology
https://www.readbyqxmd.com/read/27976606/the-progression-of-coeliac-disease-its-neurological-and-psychiatric-implications
#14
Giovanna Campagna, Mirko Pesce, Raffaella Tatangelo, Alessia Rizzuto, Irene La Fratta, Alfredo Grilli
The aim of the paper is to show the various neurological and psychiatric symptoms in coeliac disease (CD). CD is a T cell-mediated, tissue-specific autoimmune disease which affects genetically susceptible individuals after dietary exposure to proline- and glutamine-rich proteins contained in certain cereal grains. Genetics, environmental factors and different immune systems, together with the presence of auto-antigens, are taken into account when identifying the pathogenesis of CD. CD pathogenesis is related to immune dysregulation, which involves the gastrointestinal system, and the extra-intestinal systems such as the nervous system, whose neurological symptoms are evidenced in CD patients...
December 15, 2016: Nutrition Research Reviews
https://www.readbyqxmd.com/read/27974981/an-overlapping-case-of-miller-fisher-syndrome-bickerstaff-s-encephalitis-and-the-asman-variant-of-guillain-barre-syndrome
#15
E J Pegg, S K Chhetri, U G Lekwuwa, T Majeed
A 56-year-old man presented with a 3-day history of progressive tingling of the hands, unsteadiness, and diplopia. He was initially diagnosed clinically with Miller Fisher Syndrome (MFS) but later developed limb weakness consistent with Guillain-Barre Syndrome (GBS) and subsequently reduced consciousness consistent with Bickerstaff's brainstem encephalitis (BBE). Neurophysiology revealed an axonal motor and sensory neuropathy, in keeping with the Acute Motor and Sensory Axonal Neuropathy (AMSAN) variant of GBS...
2016: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/27957617/neuromyotonia-as-an-unusual-neurological-complication-of-primary-sj%C3%A3-gren-s-syndrome-case-report-and-literature-review
#16
REVIEW
Fei Xiao
Primary Sjögren's syndrome (PSS) is a systemic autoimmune disorder characterized by chronic inflammation of exocrine glands such as the lachrymal and salivary glands, leading to xerophthalmia and xerostomia. Neurological manifestations are sometimes found in patients with PSS. A variety of neurological complications has been reported in patients with PSS, and both the central nervous system (CNS) and peripheral nervous system (PNS) can be involved in PSS. Several forms of neuropathy, including polyneuropathy, cranial neuropathy, and multiple mononeuropathy, are often seen in PSS patients...
December 12, 2016: Clinical Rheumatology
https://www.readbyqxmd.com/read/27951607/-chronic-inflammatory-demyelinating-polyneuropathy
#17
M Balke, G Wunderlich, A Brunn, G R Fink, H C Lehmann
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic progressive or relapsing autoimmune neuropathy with heterogeneous clinical presentation. Symptoms typically include symmetrical, proximal and/or distal paresis and sensory loss. Atypical CIDP variants are increasingly recognized, including subtypes with rapid onset as well as variants with pure sensory, focal or marked asymmetrical deficits. Diagnosis is established by compatible symptoms, characteristic electrophysiological features and cerebrospinal fluid analysis...
December 2016: Fortschritte der Neurologie-Psychiatrie
https://www.readbyqxmd.com/read/27940347/proteasome-inhibitors-in-cancer-therapy-treatment-regimen-and-peripheral-neuropathy-as-a-side-effect
#18
REVIEW
Gulce Sari Kaplan, Ceyda Corek Torcun, Tilman Grune, Nesrin Kartal Ozer, Betul Karademir
Proteasomal system plays an important role in protein turnover, which is essential for homeostasis of cells. Besides degradation of oxidized proteins, it is involved in the regulation of many different signaling pathways. These pathways include mainly cell differentiation, proliferation, apoptosis, transcriptional activation and angiogenesis. Thus, proteasomal system is a crucial target for treatment of several diseases including neurodegenerative diseases, cystic fibrosis, atherosclerosis, autoimmune diseases, diabetes and cancer...
December 8, 2016: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/27939129/guillain-barr%C3%A3-syndrome-is-induced-in-non-obese-diabetic-nod-mice-following-campylobacter-jejuni-infection-and-is-exacerbated-by-antibiotics
#19
J L St Charles, J A Bell, B J Gadsden, A Malik, H Cooke, L K Van de Grift, H Y Kim, E J Smith, L S Mansfield
Campylobacter jejuni is a leading cause of bacterial gastroenteritis linked to several serious autoimmune sequelae such as the peripheral neuropathies Guillain Barré syndrome (GBS) and Miller Fisher syndrome (MFS). We hypothesized that GBS and MFS can result in NOD wild type (WT) mice or their congenic interleukin (IL)-10 or B7-2 knockouts secondary to C. jejuni infection. Mice were gavaged orally with C. jejuni strains HB93-13 and 260.94 from patients with GBS or CF93-6 from a patient with MFS and assessed for clinical neurological signs and phenotypes, anti-ganglioside antibodies, and cellular infiltrates and lesions in gut and peripheral nerve tissues...
February 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/27913968/post-ganglionic-autonomic-neuropathy-associated-with-anti-glutamic-acid-decarboxylase-antibodies
#20
E Fileccia, R Rinaldi, R Liguori, A Incensi, R D'Angelo, M P Giannoccaro, V Donadio
PURPOSE: Antibodies to glutamic acid decarboxylase (GAD-Abs) have been associated with several conditions, rarely involving the autonomic nervous system. Here, we describe two patients complaining of autonomic symptoms in whom a post-ganglionic autonomic neuropathy has been demonstrated in association with significantly elevated serum and CSF GAD-Abs levels. METHODS: Patients underwent nerve conduction studies, sympathetic skin response testing, evaluation of autonomic control of the cardiovascular system and skin biopsy...
December 2, 2016: Clinical Autonomic Research: Official Journal of the Clinical Autonomic Research Society
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