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https://www.readbyqxmd.com/read/29761122/bortezomib-treatment-for-severe-refractory-anti-nmda-receptor-encephalitis
#1
Yong-Won Shin, Soon-Tae Lee, Tae-Joon Kim, Jin-Sun Jun, Kon Chu
Objective: To evaluate the therapeutic potential of bortezomib, a proteasome inhibitor that target plasma cells, in order to revive stalled recovery in patients with anti- N -methyl-d-aspartate (NMDA) receptor encephalitis who remain bedridden even after aggressive immunotherapy. Methods: We consecutively enrolled patients with anti-NMDA receptor encephalitis who remained bedridden after first-line immunotherapy (steroids and intravenous immunoglobulin), second-line immunotherapy (rituximab), and tocilizumab treatment, and treated them with subcutaneous bortezomib...
May 2018: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29749407/peripheral-n-methyl-d-aspartate-receptor-localization-and-role-in-gastric-acid-secretion-regulation-immunofluorescence-and-pharmacological-studies
#2
Iuliia Golovynska, Tatiana V Beregova, Tatiana M Falalyeyeva, Ludmila I Stepanova, Sergii Golovynskyi, Junle Qu, Tymish Y Ohulchanskyy
The enteric nervous system (ENS) and a glutamate receptor (GluR), N-methyl-D-aspartate receptor (NMDAR), participate in gastric acid secretion (GAS) regulation. NMDARs are localized in different stomach cells; however, knowledge of NMDAR expression and function in the ENS is limited. In the present study, we clarified the types of stomach cells that express the NMDARs that are involved in GAS regulation. The pharmacological method of isolated stomach perfusion by Ghosh and Shild combined with direct mapping of NMDARs by fluorescence microscopy in the rat stomach was employed...
May 10, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29724875/unusual-case-of-anti-n-methyl-d-aspartic-acid-receptor-nmda-r-encephalitis-and-autoimmune-polyglandular-syndrome-aps
#3
Simona Frunza-Stefan, Hilary B Whitlatch, Gautam G Rao, Rana Malek
Anti-N-methyl-D-aspartic acid-receptor (NMDA-R) encephalitis is a novel disease discovered within the past 10 years. It is an autoimmune disease (AD) that has been associated with other ADs, such as Graves' disease. However, association with autoimmune polyglandular syndromes (APS) has not been previously described. A 58-year-old woman presented with altered mental status and an 8-month history of weight loss, apathy and somnolence. Laboratory evaluation confirmed Graves' disease with thyrotoxicosis and type 1 diabetes mellitus...
May 2, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29629396/anti-nmda-receptor-encephalitis-and-nonencephalitic-hsv-1-infection
#4
Amy Salovin, Jason Glanzman, Kylie Roslin, Thais Armangue, David R Lynch, Jessica A Panzer
Objective: To determine whether there is an association between nonencephalitic herpes simplex virus 1 (HSV-1) infection and anti-NMDA receptor encephalitis (anti-NMDARE). Methods: Antibody testing was performed using samples from 2 cohorts in a case-control observational study. The cohort "Philadelphia" included 16 serum samples of pediatric anti-NMDARE cases and 42 age-matched controls with other neuroinflammatory disorders studied at the Children's Hospital of Philadelphia and University of Pennsylvania...
July 2018: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/29628174/early-identification-of-anti-nmda-receptor-encephalitis-presenting-cerebral-lesions-in-unconventional-locations-on-magnetic-resonance-imaging
#5
Weihe Zhang, Lei Cui, Wei Wang, Yujuan Jiao, Yeqiong Zhang, Jinsong Jiao
To facilitate the diagnosis of anti-NMDAR encephalitis presenting with brain lesions in unconventional locations (BLUL) on MRI, we retrospectively analyzed forty-five Chinese patients. Eighteen (40.0%) of their MRI initially exhibited one or more BLUL. These locations predominantly included cerebral gray matter (cortex, basal ganglia and thalamus), as well as white matter and brainstem. Due to these BLUL, thirteen (72.2%) patients were originally misdiagnosed with other diseases and developed poor clinical and imaging outcomes...
March 29, 2018: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/29607915/-a-case-of-limbic-encephalitis-associated-with-sj%C3%A3-gren-s-syndrome-mimicking-anti-n-methyl-d-aspartate-receptor-encephalitis
#6
Kenji Yoshimura, Rie Kanki, Satoshi Nakano
A 25-year-old woman in her 37 weeks and 5 days pregnant presented with abnormal behavior and memory impairment following a high fever. Her manifestations were diagnosed as limbic encephalitis, and she delivered a baby by Cesarean section. In the operation, bilateral ovarian tumors were found and resected, though they were revealed as non-teratoma afterward. After operation, she became agitated, and started to present oral dyskinesia, intractable systemic myoclonus, central hypoventilation, and autonomic manifestations such as labile blood pressure, but her symptoms responded well to immunotherapy...
March 31, 2018: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/29580710/extreme-delta-with-or-without-brushes-a-potential-surrogate-marker-of-disease-activity-in-anti-nmda-receptor-encephalitis
#7
Claude Steriade, Stephen Hantus, Ahsan N V Moosa, Alexander D Rae-Grant
OBJECTIVE: Anti-NMDA receptor encephalitis (NMDARE) may not respond to first line immunotherapy. Biomarkers to track disease course and guide escalation of immunotherapy are needed. We describe the evolution of EEG in four patients with NMDARE requiring prolonged intensive care. METHODS: Within a database of 121 patients with immune-mediated neurological disorders, ten with NMDARE were retrospectively identified. Four patients did not respond to first line immunotherapy...
March 10, 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/29549218/clinical-and-pathogenic-significance-of-igg-iga-and-igm-antibodies-against-the-nmda-receptor
#8
Makoto Hara, Eugenia Martinez-Hernandez, Helena Ariño, Thais Armangué, Marianna Spatola, Mar Petit-Pedrol, Albert Saiz, Myrna R Rosenfeld, Francesc Graus, Josep Dalmau
OBJECTIVE: To determine the frequency and clinical relevance of immunoglobulin (Ig)G, IgA, and IgM N -methyl-d-aspartate receptor (NMDAR) antibodies in several diseases, and whether the IgG antibodies occur in disorders other than anti-NMDAR encephalitis. METHODS: Evaluation of IgG, IgA, and IgM NMDAR antibodies in serum of 300 patients with anti-NMDAR encephalitis, stroke, dementia, schizophrenia, or seronegative autoimmune encephalitis. Antibodies and their effect on cultured neurons were examined with cell-based assays and brain and live neuronal immunostaining...
April 17, 2018: Neurology
https://www.readbyqxmd.com/read/29535092/ovarian-teratoma-associated-with-anti-nmda-n-methyl-d-aspartate-receptor-encephalitis
#9
Nellihela Leel, Hemanshoo S Thakkar, David Drake, Nordeen Bouhadiba
Anti-N-methyl D-aspartate receptor (NMDAR) encephalitis is a paraneoplastic, immune-mediated encephalopathy with a known association with ovarian teratomas. We present the first case in the UK of a 7-year-old patient presenting with this condition. Having been previously fit and healthy, the patient presented with an acute onset of cognitive disturbances. The initial suspicion was that of an infective encephalopathy; however, the lumbar puncture results were negative. Electroencephalography reported diffuse background slowing, supporting a diagnosis of encephalopathy...
March 13, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29515425/dystonic-seizures-and-intense-hyperperfusion-of-the-basal-ganglia-in-a-patient-with-anti-n-methyl-d-aspartate-receptor-encephalitis
#10
Hideyuki Matsumoto, Hideji Hashida, Yukitoshi Takahashi
This report describes a rare case presenting with dystonic seizures due to anti-N-methyl-D-aspartate (NMDA) receptor encephalitis. The patient was an 18-year-old woman with repeated right-dominant dystonic seizures even under sedation. Single-photon emission computed tomography (SPECT) showed intense hyperperfusion of the caudate nuclei, putamen, globus pallidus, thalamus, and insula on the left side, suggesting encephalitis. Antibodies against NMDA receptors were detected in the sera and cerebrospinal fluids...
September 2017: Case Reports in Neurology
https://www.readbyqxmd.com/read/29476014/positive-allosteric-modulation-as-a-potential-therapeutic-strategy-in-anti-nmda-receptor-encephalitis
#11
Natasha Warikoo, Samuel J Brunwasser, Ann Benz, Hong-Jin Shu, Steven M Paul, Michael Lewis, James Doherty, Michael Quirk, Laura Piccio, Charles F Zorumski, Gregory S Day, Steven Mennerick
N -methyl-d-aspartate receptors (NMDARs) are ionotropic glutamate receptors important for synaptic plasticity, memory, and neuropsychiatric health. NMDAR hypofunction contributes to multiple disorders, including anti-NMDAR encephalitis (NMDARE), an autoimmune disease of the CNS associated with GluN1 antibody-mediated NMDAR internalization. Here we characterize the functional/pharmacological consequences of exposure to CSF from female human NMDARE patients on NMDAR function, and we characterize the effects of intervention with recently described positive allosteric modulators (PAMs) of NMDARs...
March 28, 2018: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/29474315/antibodies-and-the-brain-anti-n-methyl-d-aspartate-receptor-antibody-and-the-clinical-effects-in-patients-with-systemic-lupus-erythematosus
#12
Yoshiyuki Arinuma
PURPOSE OF REVIEW: Neuropsychiatric manifestations are one of the fatal complications in patients with systemic lupus erythematosus (SLE). However, the diagnosis and monitoring of that aspect of SLE is still challenging, as there are no reliable biomarkers linked to central nervous system (CNS) damage. This review emphasizes potential candidate autoantibodies that appear to be associated with development of behavioral and psychiatric manifestations in SLE patients. RECENT FINDINGS: Developments in the pathogenesis in SLE, not surprising for this immune disorder, point to specific, autoantibody toxicity...
June 2018: Current Opinion in Neurology
https://www.readbyqxmd.com/read/29443773/anti-n-methyl-d-aspartate-receptor-encephalitis-associated-with-acute-toxoplasma-gondii-infection-a-case-report
#13
Xiaotang Cai, Hui Zhou, Yongmei Xie, Dan Yu, Zhiling Wang, Haitao Ren
RATIONALE: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis has been recognized as the most frequent autoimmune encephalitis in children. Several infectious agents have been implicated in anti-NMDA encephalitis. PATIENT CONCERNS: A previously healthy immunocompetent 9-year-old girl first presented with seizures, headaches and vomiting. Cerebrospinal fluid and brain magnetic resonance imaging were normal. After one week onset, the patient gradually developed unexplained personality and behavior changes, accompanied by fever and seizures again...
February 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29414288/comparison-of-myelin-oligodendrocyte-glycoprotein-mog-antibody-disease-and-aqp4-igg-positive-neuromyelitis-optica-spectrum-disorder-nmosd-when-they-co-exist-with-anti-nmda-n-methyl-d-aspartate-receptor-encephalitis
#14
Siyuan Fan, Yan Xu, Haitao Ren, Hongzhi Guan, Feng Feng, Xuehui Gao, Ding Ding, Fang Fang, Guangliang Shan, Tianjia Guan, Yao Zhang, Yi Dai, Ming Yao, Bin Peng, Yicheng Zhu, Liying Cui
BACKGROUND: Myelin oligodendrocyte glycoprotein (MOG)-antibody (ab) disease and AQP4-IgG-positive neuromyelitis optica spectrum disorder (NMOSD) can co-exist with anti-NMDA (N-methyl-D-aspartate) receptor encephalitis (NMDARe). OBJECTIVES: To characterize MOG-ab disease and AQP4-IgG-positive NMOSD during NMDARe. METHODS: We analyzed all the patients with overlapping MOG-ab disease and NMDARe (MNOS) and patients with AQP4-IgG-positive NMOSD and NMDARe (ANOS) in our hospital and compared those data with data from systematically review of previously published reports...
February 2018: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/29413523/anti-nmda-receptor-encephalitis-clinical-features-and-basic-mechanisms
#15
David R Lynch, Amy Rattelle, Yi Na Dong, Kylie Roslin, Amy J Gleichman, Jessica A Panzer
In slightly more than 10 years, anti-NMDA receptor (NMDAR) encephalitis has changed from a rare paraneoplastic syndrome to the most common cause of nonviral encephalitis. It presents fulminantly with progressive psychosis, seizures, and autonomic dysfunction, leading to death if untreated. However, rapid recognition and treatment can lead to survival and a return to baseline levels of functioning in many patients. While initially associated with ovarian teratomas, it is now associated with other tumors and can reflect a postviral event...
2018: Advances in Pharmacology
https://www.readbyqxmd.com/read/29406902/autoimmune-and-paraneoplastic-movement-disorders-an-update
#16
REVIEW
José Fidel Baizabal-Carvallo, Joseph Jankovic
Movement disorders (MDs) are common in patients with autoimmune disorders affecting the central and peripheral nervous system. They may be observed in autoimmune disorders triggered by an infectious agent, such as streptococcus in Sydenham's chorea, or in basal ganglia encephalitis with antibodies against the dopamine-D2 receptors. In these patients chorea or dystonia are usually the most prominent hyperkinetic MDs. MDs are also observed in patients with diffuse or limbic encephalitis with antibodies directed against neuronal cell-surface antigens...
February 15, 2018: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29370977/severe-dysautonomia-as-a-main-feature-of-anti-gad-encephalitis-report-of-a-paediatric-case-and-literature-review
#17
Nedia Ben Achour, Thouraya Ben Younes, Ibtihel Rebai, Melika Ben Ahmed, Ichraf Kraoua, Ilhem Ben Youssef-Turki
INTRODUCTION: Anti-glutamic acid decarboxylase (anti-GAD65) antibodies are a rare cause of autoimmune encephalitis. This entity is mainly recognized in adults and very few cases were reported in children. We report on a paediatric case of anti-GAD encephalitis with severe presentation and uncontrollable dysautonomia. CASE STUDY: A 9-year-old girl was referred to our department for refractory seizures and behavioral disturbances. Brain magnetic resonance imaging (MRI) was normal...
May 2018: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29356973/pitfalls-in-clinical-diagnosis-of-anti-nmda-receptor-encephalitis
#18
Atsushi Kaneko, Juntaro Kaneko, Naomi Tominaga, Naomi Kanazawa, Kasumi Hattori, Yoshikazu Ugawa, Arata Moriya, Daisuke Kuzume, Daisuke Ishima, Eiji Kitamura, Kazutoshi Nishiyama, Takahiro Iizuka
OBJECTIVES: To report pitfalls in the clinical diagnosis of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. METHODS: We retrospectively reviewed the clinical information of 221 patients with clinically suspected autoimmune neurological disorders who underwent testing for autoantibodies against neuronal cell-surface antigens between January 1, 2007 and September 10, 2017. Forty-one patients met the diagnostic criteria for probable anti-NMDAR encephalitis (probable criteria), but one was excluded because neither serum nor CSF was examined at the active stage...
March 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29277332/cytokine-chemokine-elevation-during-the-transition-phase-from-hsv-encephalitis-to-autoimmune-anti-nmda-receptor-encephalitis
#19
Takanori Omae, Yoshiaki Saito, Hirokazu Tsuchie, Koyo Ohno, Yoshihiro Maegaki, Hiroshi Sakuma
A 3-year-old girl suffered from anti-N-methyl-d-aspartate (anti-NMDA) receptor encephalitis after resolution of herpes simplex virus encephalitis (HSE). Methylprednisolone pulse and immunoglobulin therapies showed little effect, but the patient completely recovered after six courses of monthly cyclophosphamide pulse therapy and successive maintenance on mycophenolate mofetil for one year. Anti-NMDA receptor antibody in the cerebrospinal fluid (CSF) was minimally detected during the prodromal febrile period and then was seen to be markedly elevated at the onset of second encephalopathy phase...
April 2018: Brain & Development
https://www.readbyqxmd.com/read/29258969/a-case-of-anti-nmda-receptor-encephalitis-revealed-by-insular-epilepsy
#20
Charlotte De Maeseneire, Riem El Tahry, Susana Ferrao Santos
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder of the central nervous system that typically manifests predominantly as a psychiatric disorder. However, other manifestations such as epileptic seizures, abnormal movements, and memory or language complications are not unusual. Here, we report the case of a young man who presented with a new-onset epilepsy, with ictal semiology suggestive of insular involvement; this hypothesis was supported by a PET-CT study. Anti-NMDAR antibodies were found in the CSF, confirming the diagnosis of anti-NMDAR encephalitis...
December 1, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
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