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Aquaporin-4 antibodies

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https://www.readbyqxmd.com/read/28929473/-comparison-of-neuromyelitis-optica-spectra-according-to-aqp4-antibody-serostatus-in-a-mexican-referral-centre
#1
E Antonio-Luna, G I Acosta-Castillo, J F Ortiz-Maldonado, A Estrada-Galindo, T Corona, J Flores
INTRODUCTION: Recent studies report that patients with neuromyelitis optica (NMO) or seropositive NMO spectra disorders display clinical characteristics that are different from those of patients who are seropositive for the aquaporin-4 (AQP4) antibody. AIM: To analyse the clinical and paraclinical characteristics of patients with NMO according to their serum AQP4 status. PATIENTS AND METHODS: We conducted a retrospective study of 100 patients with NMO who fulfilled the Wingerchuk criteria: 70 were positive for the AQP4 antibody and 30 were seronegative...
October 1, 2017: Revista de Neurologia
https://www.readbyqxmd.com/read/28926419/peduncular-hallucinosis-and-autonomic-dysfunction-in-anti-aquaporin-4-antibody-syndrome
#2
Renee Berry, Peter K Panegyres
Neuromyelitis optica is a rare, severe inflammatory demyelinating disease of the central nervous system, previously described as affecting only the optic nerve and spinal cord. Since the discovery of a highly specific autoantibody, anti-aquaporin-4, lesions are now recognized outside these regions. We report a man with severe, debilitating symptoms resulting from a symptomatic lesion within the diencephalon, manifesting with abnormal circadian rhythms, autonomic dysfunction, behavioral disturbance, and complex visual hallucinations...
September 2017: Cognitive and Behavioral Neurology: Official Journal of the Society for Behavioral and Cognitive Neurology
https://www.readbyqxmd.com/read/28920766/clinical-profile-of-patients-with-paraneoplastic-neuromyelitis-optica-spectrum-disorder-and-aquaporin-4-antibodies
#3
Maria Sepúlveda, Nuria Sola-Valls, Domingo Escudero, Bojan Rojc, Manuel Barón, Luis Hernández-Echebarría, Begoña Gómez, Josep Dalmau, Albert Saiz, Francesc Graus
BACKGROUND: In a minority of patients with neuromyelitis optica spectrum disorder (NMOSD) and aquaporin-4 antibodies (AQP4-IgG), the disease has a paraneoplastic origin. It is unknown whether these patients have distinctive clinical features. OBJECTIVE: To report the clinical features of a series of patients with paraneoplastic NMOSD and AQP4-IgG and to review previously reported cases. METHODS: Retrospective analysis of clinical records of 156 patients with NMOSD and AQP4-IgG and review of previously reported patients with paraneoplastic NMOSD and AQP4-IgG...
September 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28910968/efficiency-of-antibody-therapy-in-demyelinating-diseases
#4
Tetsuya Akaishi, Ichiro Nakashima
Monoclonal antibody therapy is a new treatment strategy for many types of diseases including cancers and autoimmune diseases, realizing a high efficacy and tolerability. In multiple sclerosis (MS) and neuromyelitis optica (NMO) spectrum disorders, several monoclonal antibodies have been suggested to decrease the incidence of clinical relapse and the disease activity. In MS, anti-α4 integrin (natalizumab), anti-CD52 (alemtuzumab), anti-CD25 (daclizumab) and anti-CD20 (ocrelizumab) have been shown to effectively reduce the relapses in randomized controlled trials and have been approved by the Food and Drug Administration...
July 1, 2017: International Immunology
https://www.readbyqxmd.com/read/28883692/fluctuation-of-zonulin-levels-in-blood-vs-stability-of-antibodies
#5
Aristo Vojdani, Elroy Vojdani, Datis Kharrazian
AIM: To evaluate the measurement of zonulin level and antibodies of zonulin and other tight junction proteins in the blood of controls and celiac disease patients. METHODS: This study was conducted to assess the variability or stability of zonulin levels vs IgA and IgG antibodies against zonulin in blood samples from 18 controls at 0, 6, 24 and 30 h after blood draw. We also measured zonulin level as well as zonulin, occludin, vinculin, aquaporin 4 and glial fibrillary acidic protein antibodies in the sera of 30 patients with celiac disease and 30 controls using enzyme-linked immunosorbent assay methodology...
August 21, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28879451/prognostic-factors-and-disease-course-in-aquaporin-4-antibody-positive-chinese-patients-with-acute-optic-neuritis
#6
Hao Kang, Tingjun Chen, Hongyang Li, Quangang Xu, Shanshan Cao, Shihui Wei
The objective is to assess the frequency of AQP4-Ab in Chinese patients with optic neuritis (ON) and to investigate the prognostic implications of AQP4-Ab seropositivity in such patients. The presence of aquaporin-4 (AQP4) antibodies in human AQP4-transfected cells was determined by indirect immunofluorescence, and the diagnostic and prognostic relevance of AQP4 antibodies in 215 Chinese patients with optic neuritis was evaluated. The patients were enrolled from the Chinese People's Liberation Army General Hospital (PLAGH) and followed up to 1-228 months...
September 6, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28872108/induction-of-paralysis-and-visual-system-injury-in-mice-by-t-cells-specific-for-neuromyelitis-optica-autoantigen-aquaporin-4
#7
Sharon A Sagan, Andrés Cruz-Herranz, Collin M Spencer, Peggy P Ho, Lawrence Steinman, Ari J Green, Raymond A Sobel, Scott S Zamvil
While it is recognized that aquaporin-4 (AQP4)-specific T cells and antibodies participate in the pathogenesis of neuromyelitis optica (NMO), a human central nervous system (CNS) autoimmune demyelinating disease, creation of an AQP4-targeted model with both clinical and histologic manifestations of CNS autoimmunity has proven challenging. Immunization of wild-type (WT) mice with AQP4 peptides elicited T cell proliferation, although those T cells could not transfer disease to naïve recipient mice. Recently, two novel AQP4 T cell epitopes, peptide (p) 135-153 and p201-220, were identified when studying immune responses to AQP4 in AQP4-deficient (AQP4(-/-)) mice, suggesting T cell reactivity to these epitopes is normally controlled by thymic negative selection...
August 21, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28863861/neuromyelitis-optica-atypical-hemophagocytic-lymphohistiocytosis-and-heterozygous-perforin-a91v-mutation
#8
Boaz Palterer, Francesca Brugnolo, Elena Sieni, Alessandro Barilaro, Paola Parronchi
Neuromyelitis optica is an autoimmune demyelinating inflammatory disease characterized by optic neuritis and myelitis with anti-aquaporin 4 antibodies. Hemophagocytic lymphohistiocytosis is a severe systemic inflammatory syndrome that can present in a genetic primary form or secondarily to infective, neoplastic or autoimmune diseases. Our case discusses the first reported case of atypical late-onset hemophagocytic lymphohistiocytosis in a patient with neuromyelitis optica, with multiple triggering factors and carrying the common A91V hypomorphic perforin mutation, that blurs the distinction between primary and secondary forms...
October 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28840314/what-proportion-of-aqp4-igg-negative-nmo-spectrum-disorder-patients-are-mog-igg-positive-a-cross-sectional-study-of-132-patients
#9
Shahd H M Hamid, Daniel Whittam, Kerry Mutch, Samantha Linaker, Tom Solomon, Kumar Das, Maneesh Bhojak, Anu Jacob
Antibodies to myelin oligodendrocyte glycoprotein (MOG-IgG) have been described in patients with neuromyelitis optica spectrum disorders (NMOSD) without aquaporin-4 antibodies (AQP4-IgG). We aimed to identify the proportion of AQP4-IgG-negative NMOSD patients who are seropositive for MOG-IgG. In a cross sectional study, we reviewed all patients seen in the National NMO clinic over the last 4 years (after the availability of MOG-IgG testing), including clinical information, MRI, and antibody tests. 261 unique patients were identified...
August 24, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28834867/cortical-blindness-and-not-optic-neuritis-as-a-cause-of-vision-loss-in-a-sj%C3%A3-gren-s-syndrome-ss-patient-with-the-neuromyelitis-optica-spectrum-disorder-nmosd-challenges-of-ascribing-demyelinating-syndromes-to-ss-a-case-report
#10
Aliya Lalji, Izlem Izbudak, Julius Birnbaum
RATIONALE: The conception that multiple sclerosis may be challenging to distinguish from demyelinating manifestations of Sjögren's syndrome (SS) was introduced more than 30 years ago. However, it is now recognized that the neuromyelitis optica spectrum disorder (NMOSD) may occur more frequently in SS as opposed to multiple sclerosis. Characteristic NMOSD features can include severe attacks of optic neuritis, myelitis which is frequently longitudinally-extensive (spanning at least three vertebral segments on magnetic resonance imaging [MRI]), and an association with anti-aquaporin-4 antibodies...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28820014/postinfectious-anti-myelin-oligodendrocyte-glycoprotein-antibody-positive-optic-neuritis-and-myelitis
#11
J P Vieira, J Sequeira, M J Brito
We report the case of a 9-year-old girl admitted with fever, headache, and a cerebrospinal fluid lymphocytic pleocytosis. Polymerase chain reaction was positive for human herpes virus 6. She subsequently developed ataxia and bilateral loss of vision. Magnetic resonance imaging (MRI) showed bilateral optic nerve lesions with extension to optic chiasm and a short-segment myelitis. Serologic studies were positive for Borrelia burgdorferi IgM. Anti-aquaporin 4 antibody was negative and anti-myelin oligodendrocyte glycoprotein antibody (MOG) positive...
January 1, 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/28803524/endocrinopathies-in-paediatric-onset-neuromyelitis-optica-spectrum-disorder-with-aquaporin-4-aqp4-antibody
#12
Yael Hacohen, Silvia Messina, Hoong-Wei Gan, Sukhvir Wright, Saleel Chandratre, Maria Isabel Leite, Penny Fallon, Angela Vincent, Olga Ciccarelli, Evangeline Wassmer, Ming Lim, Jacqueline Palace, Cheryl Hemingway
The involvement of the diencephalic regions in neuromyelitis optica spectrum disorder (NMOSD) may lead to endocrinopathies. In this study, we identified the following endocrinopathies in 60% (15/25) of young people with paediatric-onset aquaporin 4-Antibody (AQP4-Ab) NMOSD: morbid obesity ( n = 8), hyperinsulinaemia ( n = 5), hyperandrogenism ( n = 5), amenorrhoea ( n = 5), hyponatraemia ( n = 4), short stature ( n = 3) and central hypothyroidism ( n = 2) irrespective of hypothalamic lesions...
August 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28795614/shifting-borders-crossing-boundaries-the-case-of-combined-central-and-peripheral-demyelination
#13
Orhan Aktas
The case report by Puthenparampil et al. suggests that heterogeneity of demyelinating diseases may be more complex than expected. The authors describe a female patient starting with isolated but relapsing myelitis (in the absence of oligoclonal bands or anti-aquaporin 4 antibodies) and later experiencing the simultaneous occurrence of inflammatory demyelinating polyneuropathy and of a tumefactive hippocampal brain lesion responsive to immunotherapy. As testing for antibodies against established and novel auto-antigens including neurofascin-155 was negative, this case may be regarded as an example for the unfolding spectrum of combined central and peripheral demyelination (CCPD) syndromes with so far unknown immunological target(s)...
August 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28780848/-clinical-characteristics-of-neuromyelitis-optica-spectrum-disorders-associated-with-syringomyelia
#14
Y G Wang, Y Q Wang, W Qiu, X Q Hu, Z Z Lu
Objective: To analyze the clinical characteristics of neuromyelitis optica spectrum disorders (NMOSD) associated with syringomyelia (SML). Methods: The clinical manifestations, laboratory parameters and imaging findings of 7 patients with NMOSD associated with SML during June 2008 to August 2016 from The Third Affiliated Hospital of Sun Yat-sen University were retrospectively analyzed. Results: Six patients were female and 1 was male, with ages ranging from 27-67 years, the course of the disease was 3-12 years, recurrence was 2-7 times, all the patients were cerebrospinal fluid oligoclonal bands (CSF OCB) negative, 5 patients were positive for aquaporin-4-antibody/NMO-IgG...
August 1, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28748449/first-report-of-persistent-dengue-1-associated-autoimmune-neurological-disturbance-neuromyelitis-optica-spectrum-disorder
#15
Marzia Puccioni-Sohler, Alice M M Ornelas, Andrea S de Souza, Mauro Jorge Cabral-Castro, Jessyca T M A Ramos, Carolina Rosadas, Maria Cecilia F Salgado, Alexandre A Castiglione, Fernando Ferry, Jose Mauro Peralta, Carolina Moreira Voloch, Amilcar Tanuri, Fernanda Tovar-Moll, Renato Santana Aguiar
Dengue virus (DENV) causes immune-mediated diseases. Neurological involvement represents a severe condition that is rarely observed in DENV-1 infection. Neuromyelitis optica (NMO)/NMO spectrum disorders (NMOSD) are idiopathic immune-mediated demyelinating syndromes of the central nervous system. We report a 17-year-old female with oligosymptomatic DENV-1 viremia, diagnosed as NMOSD. Magnetic resonance imaging showed spinal cord and brainstem lesions. Antibody for aquaporin 4 was negative. DENV-1 RNA infection was detected by serial RT-PCR and confirmed by phylogenetic analysis in serum...
July 26, 2017: Journal of Neurovirology
https://www.readbyqxmd.com/read/28748216/t-cells-targeting-neuromyelitis-optica-autoantigen-aquaporin-4-cause-paralysis-and-visual-system-injury
#16
COMMENT
Andrés Cruz-Herranz, Sharon A Sagan, Raymond A Sobel, Ari J Green, Scott S Zamvil
Aquaporin-4 (AQP4)-specific antibodies are instrumental in promoting central nervous system (CNS) tissue injury in neuromyelitis optica (NMO), yet evidence indicates that AQP4-specific T cells also have a pivotal role in NMO pathogenesis. Although considerable effort has been devoted to creation of animal models to study how AQP4-specific T cells and antibodies may cooperate in development of both clinical and histologic opticospinal inflammatory disease, the initial attempts were unsuccessful. Recently, it was discovered that T cells from AQP4-deficient (AQP4(-/-)) mice recognize distinct AQP4 epitopes that were not identified previously in wild-type (WT) mice, and that donor Th17 cells from AQP4(-/-) mice that target those novel epitopes could cause paralysis and visual system injury associated with opticospinal inflammation in WT recipient mice...
May 2017: Journal of Nature and Science
https://www.readbyqxmd.com/read/28733756/glial-and-neuronal-antibodies-in-patients-with-idiopathic-intracranial-hypertension
#17
Güneş Altıokka-Uzun, Ece Erdağ, Betül Baykan, John Tzartos, Duygu Gezen-Ak, Bedia Samancı, Erdinç Dursun, Paraskevi Zisimopoulou, Katerina Karagiorgou, Christos Stergiou, Erdi Şahin, Esme Ekizoğlu, Murat Kürtüncü, Erdem Tüzün
Headache and visual disturbances are the main presenting symptoms of idiopathic intracranial hypertension (IIH) characterized by increased intracranial pressure (ICP) with an unknown cause. We aimed to investigate the antibodies against optic neuritis-associated glial antigens, aquaporin-4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) and uncharacterized neuronal membrane antigens in IIH patients. Consecutive patients diagnosed according to Friedman revised diagnostic criteria and control subjects were included after their consent...
July 21, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28716289/devic-s-disease-before-devic-on-the-contribution-of-friedrich-albin-schanz-1863-1923
#18
S Jarius, B Wildemann
Neuromyelitis optica (NMO, Devic's disease) is an autoimmune disorder of the CNS that mainly affects the optic nerves and spinal cord and, if not treated, can leave patients blind and wheelchair-bound. NMO was long considered a clinical variant of multiple sclerosis (MS). While the history of MS has been studied extensively, relatively little is known about the early history of NMO. A dissertation written by Fernand Gault (1873-1936), a doctoral student to Eugène Devic (1858-1930), a Lyon-based neurologist after whom the disease would later be named, is considered by many the first review of NMO...
August 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28684532/longitudinal-analysis-of-myelin-oligodendrocyte-glycoprotein-antibodies-in-cns-inflammatory-diseases
#19
Jae-Won Hyun, Mark R Woodhall, Su-Hyun Kim, In Hye Jeong, Byungsoo Kong, Gayoung Kim, Yeseul Kim, Min Su Park, Sarosh R Irani, Patrick Waters, Ho Jin Kim
BACKGROUND: We evaluated the seroprevalence of myelin oligodendrocyte glycoprotein immunoglobulin G1 (MOG-IgG) and associated clinical features of patients from a large adult-dominant unselected cohort with mainly relapsing central nervous system (CNS) inflammatory diseases. We also investigate the clinical relevance of MOG-IgG through a longitudinal analysis of serological status over a 2-year follow-up period. METHODS: Serum samples from 505 patients with CNS inflammatory diseases at the National Cancer Center were analysed using cell-based assays for MOG-IgG and aquaporin-4 immunoglobulin G (AQP4-IgG)...
July 6, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28679661/glucose-regulated-protein-78-autoantibody-associates-with-blood-brain-barrier-disruption-in-neuromyelitis-optica
#20
Fumitaka Shimizu, Kristin L Schaller, Gregory P Owens, Anne C Cotleur, Debra Kellner, Yukio Takeshita, Birgit Obermeier, Thomas J Kryzer, Yasuteru Sano, Takashi Kanda, Vanda A Lennon, Richard M Ransohoff, Jeffrey L Bennett
Neuromyelitis optica (NMO) is an inflammatory disorder mediated by antibodies to aquaporin-4 (AQP4) with prominent blood-brain barrier (BBB) breakdown in the acute phase of the disease. Anti-AQP4 antibodies are produced mainly in the periphery, yet they target the astrocyte perivascular end feet behind the BBB. We reasoned that an endothelial cell-targeted autoantibody might promote BBB transit of AQP4 antibodies and facilitate NMO attacks. Using monoclonal recombinant antibodies (rAbs) from patients with NMO, we identified two that strongly bound to the brain microvascular endothelial cells (BMECs)...
July 5, 2017: Science Translational Medicine
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