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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/29718831/generalized-and-lateralized-rhythmic-patterns
#4
Sarah E Schmitt
The relationship between generalized and lateralized rhythmic delta activity (RDA) and seizures is more ambiguous than the relationship between periodic discharges and seizures. Although frontally predominant generalized RDA is not associated with seizures, occipitally predominant RDA may be associated with the absence of seizures. Lateralized RDA seems to be more strongly associated with the presence of seizure activity. Appropriate recognition of generalized RDA and lateralized rhythmic delta activity may be confounded by benign etiologies of RDA, such as phi rhythm, slow alpha variant, subclinical rhythmic electrographic discharges of adults, or hyperventilation-induced high-amplitude rhythmic slowing...
May 2018: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
https://www.readbyqxmd.com/read/29718139/ion-channels-in-eeg-isolating-channel-dysfunction-in-nmda-receptor-antibody-encephalitis
#5
Mkael Symmonds, Catherine H Moran, M Isabel Leite, Camilla Buckley, Sarosh R Irani, Klaas Enno Stephan, Karl J Friston, Rosalyn J Moran
Neurological and psychiatric practice frequently lack diagnostic probes that can assess mechanisms of neuronal communication non-invasively in humans. In N-methyl-d-aspartate (NMDA) receptor antibody encephalitis, functional molecular assays are particularly important given the presence of NMDA antibodies in healthy populations, the multifarious symptomology and the lack of radiological signs. Recent advances in biophysical modelling techniques suggest that inferring cellular-level properties of neural circuits from macroscopic measures of brain activity is possible...
April 30, 2018: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29629396/anti-nmda-receptor-encephalitis-and-nonencephalitic-hsv-1-infection
#6
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
#7
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/29620091/nmda-receptor-antibody-encephalitis-presenting-with-enhancing-lesion-and-seizures
#8
Michael H Rosenbloom, Melissa K Samuelsson, Michael E Brogan, Teresa A Tran-Lim
No abstract text is available yet for this article.
October 2017: Neurology. Clinical Practice
https://www.readbyqxmd.com/read/29616344/novel-pharmacological-nonopioid-therapies-in-chronic-pain
#9
REVIEW
Alan David Kaye, Elyse M Cornett, Brendon Hart, Shilpadevi Patil, Andrew Pham, Matthew Spalitta, Kenneth F Mancuso
PURPOSE OF REVIEW: Opioid use and abuse has led to a worldwide opioid epidemic. And while opioids are clinically useful when appropriately indicated, they are associated with a wide range of dangerous side effects and whether they are effective at treating or eliminating chronic pain is controversial. There has long been a need for the development of nonopioid alternative treatments for patients that live in pain, and until recently, only a few effective treatments were available. Today, there are a wide range of nonopioid treatments available including NSAIDs, acetaminophen, corticosteroids, nerve blocks, SSRIs, neurostimulators, and anticonvulsants...
April 3, 2018: Current Pain and Headache Reports
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
#10
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/29588384/autoimmune-encephalitis-nmdar-antibody-in-a-patient-receiving-chronic-post-transplant-immunosuppression
#11
Anna Randall, Saif Huda, Anu Jacob, Andrew J Larner
Autoimmune encephalitis associated with antibodies (Abs) directed against the synaptic ligand-gated ion channel NMDA receptor (NMDAR) was first described as a paraneoplastic disorder in association with ovarian teratoma. Other forms of neoplasia have subsequently been reported although many patients do not have a tumour. Tumour removal, where applicable, and immunotherapy form the mainstays of treatment. We present a patient who developed NMDAR-Ab encephalitis despite being chronically immunosuppressed following organ transplantation, and who was eventually found to have an occult malignancy in the form of non-Hodgkin's lymphoma...
March 27, 2018: Practical 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
#12
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/29560942/-the-neurotoxicity-biomarkers-as-potential-indicators-of-the-spinal-cord-ischemia
#13
G V Ponomarev, T V Lalayan, S A Dambinova, A A Skoromets
The study aimed at assessing the level of glutamate receptors antibodies (Abs) in blood serum and cerebrospinal fluid (CSF) of patients with spinal cord ischemia along with traditional diagnostic approaches. MATERIAL AND METHODS: Forty patients with spinal cord ischemia (10 with spinal stroke and 30 with subacute and chronic course of the disease) were enrolled. After exclusion of some participants, 27 patients continued the study. Comparison groups included 30 patients with ischemic stroke and 30 patients with radiculopathy...
2018: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/29549218/clinical-and-pathogenic-significance-of-igg-iga-and-igm-antibodies-against-the-nmda-receptor
#14
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/29536122/-principles-of-autoimmune-and-paraneoplastic-encephalitis
#15
REVIEW
C G Bien
The paraneoplastic and autoimmune encephalitides are now well-established entities. Detection of neural autoantibodies enables specific diagnoses, provides information on the underlying disease pathophysiology, immunological treatability and the likelihood of a tumor being the underlying cause. This is true for the "high ranking" neural antibodies that have been established in the context of circumscribed clinical images and in consideration of large control groups, have been found in the same way by other laboratories and they respond to immunotherapy...
March 13, 2018: Der Nervenarzt
https://www.readbyqxmd.com/read/29535092/ovarian-teratoma-associated-with-anti-nmda-n-methyl-d-aspartate-receptor-encephalitis
#16
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
#17
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
#18
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
#19
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/29468181/seizure-semiology-an-important-clinical-clue-to-the-diagnosis-of-autoimmune-epilepsy
#20
Rui-Juan Lv, Hai-Tao Ren, Hong-Zhi Guan, Tao Cui, Xiao-Qiu Shao
Objective: The purpose of this study is to analyze the seizure semiologic characteristics of patients with autoimmune epilepsy (AE) and describe the investigation characteristics of AE using a larger sample size. Methods: This observational retrospective case series study was conducted from a tertiary epilepsy center between May 2014 and March 2017. Cases of new-onset seizures were selected based on laboratory evidence of autoimmunity. At the same time, typical mesial temporal lobe epilepsy (MTLE) patients with hippocampal sclerosis (HS) were recruited as the control group from the subjects who underwent presurgical evaluation during the same period...
February 2018: Annals of Clinical and Translational Neurology
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