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Autoimmune encephalitis

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https://www.readbyqxmd.com/read/29928585/autoimmune-encephalitis-associated-with-two-antibodies
#1
Nikolai Gil D Reyes, Mario B Prado, Christian Wilson R Turalde, Marc Laurence L Fernandez
•In patients presenting with clinical manifestations of encephalitis without clinical or laboratory signs of infection, an autoimmune etiology should be suspected.•Antibodies for various neural antigens may coexist, thus a complete and clinically-guided autoimmune panel must be done in suspected cases of autoimmune encephalitis.•Tumor resection, if applicable, combined with high dose steroids and immunotherapy are effective treatment strategies for autoimmune encephalitis with coexisting antibodies.
2018: Epilepsy & Behavior Case Reports
https://www.readbyqxmd.com/read/29923617/cd93-regulates-cns-inflammation-in-two-mouse-models-of-autoimmune-encephalomyelitis
#2
Mark R Griffiths, Marina Botto, B Paul Morgan, J W Neal, P Gasque
Microglia and non-professional immune cells (endothelial cells, neurons) participate in the recognition and removal of pathogens and tissue-debris in the injured CNS through major pro-inflammatory processes. However, the mechanisms involved in regulating these responses remain ill-characterised. We herein show that CD93 also known as complement C1qRp/AA4 stem cell marker has an important role in the regulation of inflammatory processes. The role of CD93 was evaluated in two models of neuroinflammation. We used the MOG-experimental autoimmune encephalomyelitis (EAE) model and the antibody-dependent EAE (ADEAE) which were induced in wild type and CD93 knockout mice...
June 20, 2018: Immunology
https://www.readbyqxmd.com/read/29915659/a-case-report-anti-nmda-receptor-encephalitis
#3
Pavan Bhat, Ameer Ahmed, Preetam Jolepalem, Charmian Sittambalam
N -methyl-d-aspartate receptor (NMDAR) antibody encephalitis is a potentially fatal autoimmune syndrome in which there is antibody production against the NMDAR causing profound dysregulation of neurotransmission. The syndrome is frequently associated with ovarian teratomas and women are disproportionately affected. Patients most often present with a constellation of neuropsychiatric signs and symptoms, including memory loss, hallucinations, and decreased level of consciousness. This condition is lethal if left untreated...
2018: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/29915593/detection-of-glycan-shedding-in-the-blood-new-class-of-multiple-sclerosis-biomarkers
#4
Brian DellaValle, Alba Manresa-Arraut, Henrik Hasseldam, Allan Stensballe, Jørgen Rungby, Agnete Larsen, Casper Hempel
Introduction: Multiple sclerosis (MS) is a devastating autoimmune disease, afflicting people in the prime of their lives. Presently, after initial clinical presentation, there are no reliable markers for whether a patient will develop MS, or whether their prognosis will be aggressive or relapsing-remitting. Furthermore, many MS patients do not respond to treatment. Thus, markers for diagnosis, prognosis, and treatment-responsiveness are lacking for a disease, where a precision medicine approach would be valuable...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29905150/gad65-antibody-associated-autoimmune-epilepsy-with-unique-independent-bitemporal-onset-ictal-asystole
#5
Caitlin E D'Souza, Anteneh M Feyissa
Antibodies against the 65-kDa isoform of the intracellular enzyme, glutamate decarboxylase (GAD65), have been found in patients with limbic encephalitis and drug-resistant autoimmune epilepsy. We report a 22-year-old female who presented with new-onset seizures and neuropsychiatric symptoms. Video-EEG captured unique, independent bitemporal-onset focal seizures with impaired awareness and ictal asystole. An autoimmune epilepsy panel revealed elevated GAD65 antibodies in the serum (225 nmol/l) and CSF (2.78 nmol/l), while [18 F]-fluoro-deoxy-glucose positron emission tomography showed bitemporal hypometabolism (left > right)...
June 15, 2018: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29901009/a-case-of-primary-sj%C3%A3-gren-s-syndrome-presenting-as-mass-like-encephalitis-with-progression-to-neuromyelitis-optica-spectrum-disorder
#6
Hyun-Jung Lee, Sung Hae Chang, Eun Ha Kang, Yun Jong Lee, Yeong Wook Song, You-Jung Ha
Neuromyelitis optica is an idiopathic inflammatory demyelinating disease of the central nervous system (CNS) that predominantly affects the optic nerves and spinal cord. With the discovery of the pathogenic anti-aquaporin-4 (AQP4) antibody, the disease was recognized as part of a spectrum of autoimmune diseases that target AQP4, collectively referred to as neuromyelitis optica spectrum disorder (NMOSD). NMOSD consists of conditions that affect various parts of the CNS with the AQP4 antibody. In this article, we report a 43-year-old female patient who was initially diagnosed with primary Sjögren's syndrome (pSS) with CNS involvement, but was later diagnosed with overlapping pSS and NMOSD, which required more intensive treatment...
December 2017: Archives of Rheumatology
https://www.readbyqxmd.com/read/29899790/pediatric-n-methyl-d-aspartate-receptor-autoimmune-encephalitis
#7
N A Uvais
No abstract text is available yet for this article.
January 2018: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/29886429/beyond-the-limbic-system-disruption-and-functional-compensation-of-large-scale-brain-networks-in-patients-with-anti-lgi1-encephalitis
#8
Josephine Heine, Harald Prüss, Ute A Kopp, Florian Wegner, Florian Then Bergh, Thomas Münte, Klaus-Peter Wandinger, Friedemann Paul, Thorsten Bartsch, Carsten Finke
OBJECTIVE: Hippocampal inflammation in anti-LGI1 encephalitis causes memory deficits, seizures and behavioural abnormalities. Recent findings suggest that extralimbic brain areas are additionally affected and that patients also suffer from non-limbic cognitive symptoms. Moreover, up to 60% of patients show no structural MRI abnormalities in the acute disease stage. We therefore investigated whether functional connectivity analyses can identify brain network changes underlying disease-related symptoms...
June 9, 2018: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/29884664/neuropsychological-assessment-as-an-objective-tool-to-monitor-treatment-response-in-anti-n-methyl-d-aspartate-receptor-encephalitis
#9
Erica Sieg, Michael Brook, Jenny Linnoila, Stephen VanHaerents
We report a 1-year follow-up of a young woman with anti-N-methyl-D-aspartate receptor encephalitis. Management of autoimmune encephalitis remains challenging as objective and clinically relevant biomarkers are sought, which allow for the monitoring of treatment response. While further investigation is required, we believe that this case highlights the importance of following a comprehensive neuropsychological profile as a clinically relevant biomarker to guide therapeutic decision-making. By relying on the neuropsychological assessment of the patient, treatment with more toxic medications was avoided and her antiepileptic drug regimen was simplified...
June 8, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29883325/multiple-sclerosis-pathogenesis-missing-pieces-of-an-old-puzzle
#10
Reza Rahmanzadeh, Wolfgang Brück, Alireza Minagar, Mohammad Ali Sahraian
Traditionally, multiple sclerosis (MS) was considered to be a CD4 T cell-mediated CNS autoimmunity, compatible with experimental autoimmune encephalitis model, which can be characterized by focal lesions in the white matter. However, studies of recent decades revealed several missing pieces of MS puzzle and showed that MS pathogenesis is more complex than the traditional view and may include the following: a primary degenerative process (e.g. oligodendroglial pathology), generalized abnormality of normal-appearing brain tissue, pronounced gray matter pathology, involvement of innate immunity, and CD8 T cells and B cells...
June 8, 2018: Reviews in the Neurosciences
https://www.readbyqxmd.com/read/29882008/overview-of-therapeutic-plasma-exchange-in-pediatric-neurology-a-single-center-experience
#11
Murat Özkale, Ilknur Erol, Yasemin Özkale, İlknur Kozanoğlu
Therapeutic plasma exchange (TPE) is used in the treatment of neurological, hematological, renal and autoimmune diseases with known or suspected immune pathogenesis. In comparison with neurological diseases of adults, knowledge about the use of TPE in children is incomplete. We report our experience on TPE in children with neurological diseases in a single institution and describe the underlying etiology, clinical course, treatment and outcome. We retrospectively evaluated 22 consecutive children (12 girls, 10 boys, aged 2-16 years) who underwent TPE in the pediatric intensive care unit between January 2010 and January 2017...
June 7, 2018: Acta Neurologica Belgica
https://www.readbyqxmd.com/read/29876905/-autoimmune-encephalitis
#12
M Guasp, H Arino, J Dalmau
Autoimmune encephalitis are a new category of inflammatory diseases of the central nervous system mediated by antibodies that attack neurotransmitter or protein receptors on the surface of neurons. The clinical syndromes are complex and are associated with manifestations that vary according to the type of antibody that is associated. The autoimmune response can start due to the presence of a tumour or viral infection, but in many case the cause remains unknown. In paediatrics, the most frequent autoimmune encephalitis is that associated with NMDA glutamate receptor antibodies (or anti-NMDA encephalitis)...
June 5, 2018: Revista de Neurologia
https://www.readbyqxmd.com/read/29801900/practical-issues-in-measuring-autoantibodies-to-neuronal-cell-surface-antigens-in-autoimmune-neurological-disorders-190-cases
#13
Juntaro Kaneko, Naomi Kanazawa, Naomi Tominaga, Atsushi Kaneko, Hiroki Suga, Ryo Usui, Daisuke Ishima, Eiji Kitamura, Tsugio Akutsu, Koji Yoshida, Kazutoshi Nishiyama, Takahiro Iizuka
OBJECTIVES: To address practical issues in measuring autoantibodies to neuronal cell-surface antigens (NSAs) in various autoimmune neurological disorders (ANDs). METHODS: We retrospectively reviewed the clinical information of 221 patients with clinically suspected ANDs who underwent antibody testing for NSAs between January 2007 and September 2017. 31 were excluded. In 190 patients, antibody-detection rate (ADR) and antibody-phenotype association were assessed...
July 15, 2018: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/29792178/anti-ganglioside-complex-antibody-profiles-in-a-recurrent-complicated-case-of-gq1b-seronegative-miller-fisher-syndrome-and-bickerstaff-brainstem-encephalitis-a-case-report
#14
Hiroto Ito, Yuki Hatanaka, Yuki Fukami, Yumiko Harada, Rei Kobayashi, Hisashi Okada, Ayumi Uchibori, Atsuro Chiba, Satoshi Okuda
BACKGROUND: Guillain-Barré syndrome (GBS), Miller Fisher syndrome (MFS) and Bickerstaff brainstem encephalitis (BBE) are a group of autoimmune neurological disorders (GBS spectrum disorder) that rarely recur. Recently, anti-ganglioside complex antibodies (GSC-Abs) were identified in patients with GBS spectrum disorder. However, there has been no case report describing GSC-Abs profiles in a recurrent case showing different phenotypes. CASE PRESENTATION: We report the case of a 33-year-old male patient with GQ1b-seronegative BBE-GBS after two prior episodes of MFS-GBS...
May 23, 2018: BMC Neurology
https://www.readbyqxmd.com/read/29791844/regulation-of-pathogenic-t-helper-17-cell-differentiation-by-steroid-receptor-coactivator-3
#15
Kentaro Tanaka, Gustavo J Martinez, Xiaowei Yan, Weiwen Long, Kenji Ichiyama, Xinxin Chi, Byung-Seok Kim, Joseph M Reynolds, Yeonseok Chung, Shinya Tanaka, Lan Liao, Yoichi Nakanishi, Akihiko Yoshimura, Pan Zheng, Xiaohu Wang, Qiang Tian, Jianming Xu, Bert W O'Malley, Chen Dong
T helper 17 (Th17) cell development is programmed by the orphan nuclear receptor RORγt, but the underlying mechanism is not well understood. Nuclear receptor-mediated transcriptional activation depends on coactivators. Here, we show that steroid receptor coactivator-3 (SRC-3) critically regulates Th17 cell differentiation. Reduced incidence of experimental autoimmune encephalitis (EAE) associated with decreased Th17 cell generation in vivo was observed in mice with SRC-3 deletion specifically in T cells...
May 22, 2018: Cell Reports
https://www.readbyqxmd.com/read/29788790/eeg-differences-in-two-clinically-similar-rapid-dementias-voltage-gated-potassium-channel-complex-associated-autoimmune-encephalitis-and-creutzfeldt-jakob-disease
#16
Brin Freund, John C Probasco, Mackenzie C Cervenka, Raoul Sutter, Peter W Kaplan
Distinguishing treatable causes for rapidly progressive dementia from those that are incurable is vital. Creutzfeldt-Jakob disease (CJD) and voltage-gated potassium channel complex-associated autoimmune encephalitis (VGKC AE) are 2 such conditions with disparate outcomes and response to treatment. To determine the differences in electroencephalography between CJD and VGKC AE, we performed a retrospective review of medical records and examined clinical data, neuroimaging, and electroencephalographs performed in patients admitted for evaluation for rapidly progressive dementia diagnosed with CJD and VGKC AE at the Johns Hopkins Hospital and Bayview Medical Center between January 1, 2007 and December 31, 2015...
May 1, 2018: Clinical EEG and Neuroscience: Official Journal of the EEG and Clinical Neuroscience Society (ENCS)
https://www.readbyqxmd.com/read/29781194/paraneoplastic-autoimmune-encephalitis-associated-with-pleomorphic-lung-carcinoma-an-autopsy-case-report
#17
Takashi Ando, Yoji Goto, Kazuo Mano, Fumio Nomura, Masako Kurashige, Masafumi Ito, Maya Mimuro, Yasushi Iwasaki, Masahisa Katsuno, Mari Yoshida
A 64-year-old man was admitted with acute onset disturbed consciousness. Cerebrospinal fluid analysis revealed pleocytosis and elevated protein, with negative cultures and PCR. Serum antibodies for autoimmune encephalitis were also negative. Brain magnetic resonance imaging (MRI) was unremarkable, but whole-body CT scan showed a tumor in the left lower lung lobe. Bronchial brush cytology demonstrated clusters of malignant cells, and 18 F-fluorodeoxyglucose positron emission tomography showed multiple lesions and increased uptake in the lung tumor...
May 20, 2018: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29780690/an-update-on-the-treatment-of-pediatric-autoimmune-encephalitis
#18
Cory Stingl, Kathleen Cardinale, Heather Van Mater
Purpose of review: Autoimmune encephalitis (AE) is an increasingly recognized etiology for neuropsychiatric deficits that are highly responsive to immunotherapy. As a result, rheumatologists are often called upon to help with the diagnosis and treatment of these conditions. The purpose of this review is to provide an update on the pharmacologic treatment of AE. Recent findings: To date, there are no prospective randomized placebo-controlled trials to guide treatment recommendations for AE...
March 2018: Current Treatment Options in Rheumatology
https://www.readbyqxmd.com/read/29774846/occupation-associated-fatal-limbic-encephalitis-caused-by-variegated-squirrel-bornavirus-1-germany-2013
#19
Dennis Tappe, Kore Schlottau, Daniel Cadar, Bernd Hoffmann, Lorenz Balke, Burkhard Bewig, Donata Hoffmann, Philip Eisermann, Helmut Fickenscher, Andi Krumbholz, Helmut Laufs, Monika Huhndorf, Maria Rosenthal, Walter Schulz-Schaeffer, Gabriele Ismer, Sven-Kevin Hotop, Mark Brönstrup, Anthonina Ott, Jonas Schmidt-Chanasit, Martin Beer
Limbic encephalitis is commonly regarded as an autoimmune-mediated disease. However, after the recent detection of zoonotic variegated squirrel bornavirus 1 in a Prevost's squirrel (Callosciurus prevostii) in a zoo in northern Germany, we retrospectively investigated a fatal case in an autoantibody-seronegative animal caretaker who had worked at that zoo. The virus had been discovered in 2015 as the cause of a cluster of cases of fatal encephalitis among breeders of variegated squirrels (Sciurus variegatoides) in eastern Germany...
June 2018: Emerging Infectious Diseases
https://www.readbyqxmd.com/read/29774053/postherpes-simplex-encephalitis-a-case-series-of-viral-triggered-autoimmunity-synaptic-autoantibodies-and-response-to-therapy
#20
Harry Alexopoulos, Sofia Akrivou, Sotiria Mastroyanni, Maria Antonopoulou, Argirios Dinopoulos, Melpo Giorgi, Kostas Konstantinou, Evangelos Kouremenos, Maria Lariou, Dimitrios Naoumis, Efterpi Pavlidou, Evaggelos Pavlou, Konstantinos Voudris, Panayotis Vlachoyiannopoulos, Marinos C Dalakas
Background: Recent evidence suggests that patients with herpes simplex virus (HSV) encephalitis may relapse because of autoimmunity against the N-methyl-D-aspartate receptor (NMDAR). We present a case series of post-HSV relapsing encephalopathy associated with antibodies to central nervous system (CNS) synaptic antigens. Patient/Methods: Sera and cerebrospinal fluid (CSF) from five patients with HSV encephalitis who relapsed after antiviral therapy were tested for anti-NMDAR, gamma-aminobutyric acid b receptor (GABAbR), α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), Leucine-rich, glioma inactivated 1 (LGI1), anti -contactin-associated protein-like 2 (CASPR2) and dipeptidyl-peptidase-like protein-6 (DDPX) antibodies using cell-based assays...
2018: Therapeutic Advances in Neurological Disorders
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