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Autoimmune encephalitis

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https://www.readbyqxmd.com/read/29215577/acute-encephalitis-in-an-adult-with-diffuse-large-b-cell-lymphoma-with-secondary-involvement-of-the-central-nervous-system-infectious-or-non-infectious-etiology
#1
Surinder S Moonga, Kenneth Liang, Burke A Cunha
Both infectious and non-infectious etiologies of acute encephalitis have been described, as well as their specific presentations, diagnostic tests, and therapies. Classic findings of acute encephalitis include altered mental status, fever, and new lesions on neuroimaging or electroencephalogram (EEG). We report an interesting case of a 61-year-old male with a history of diffuse large B-cell lymphoma with secondary involvement of the central nervous system (SCNS-DLBCL). He presented with acute encephalitis: altered mental status, fever, leukocytosis, neuropsychiatric symptoms, multiple unchanged brain lesions on computed tomography scan of the head, and EEG showed mild to moderate diffuse slowing with low-moderate polymorphic delta and theta activity...
December 7, 2017: Journal of Clinical Medicine
https://www.readbyqxmd.com/read/29203057/febrile-infection-related-epilepsy-syndrome-fires-with-super-refractory-status-epilepticus-revealing-autoimmune-encephalitis-due-to-gabaar-antibodies
#2
D Caputo, R Iorio, F Vigevano, L Fusco
BACKGROUND: Febrile infection-related epilepsy syndrome (FIRES) has been described as an epileptic encephalopathy of unknown etiology affecting previously healthy children following febrile illness. Despite large investigations on autoimmune pathogenesis no membrane antibodies has been associated since now. CASE STUDY: We report a 13 years-old girl with negative history for neurological or autoimmune disease that developed at the sixth day of high fever a super-refractory status epilepticus...
November 24, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29200116/paraneoplastic-disorders
#3
Eric Lancaster
PURPOSE OF REVIEW: Paraneoplastic neurologic syndromes target specific areas of the nervous system with pathogenic autoantibodies or T-cell responses. Each syndrome conveys a risk of particular tumors. Expanded paraneoplastic antibody testing has led to improved diagnosis but created challenges involving appropriate interpretation of test results. RECENT FINDINGS: Peripheral nervous system paraneoplastic disorders such as myasthenia gravis and Lambert-Eaton myasthenic syndrome involve pathogenic autoantibodies...
December 2017: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/29195105/lgi1-antibody-encephalitis-detailed-clinical-laboratory-and-radiological-description-of-13-cases-in-china
#4
Dahai Wang, Qinjian Hao, Lingshuang He, Lan He, Qiang Wang
BACKGROUND AND PURPOSE: LGI1 antibody encephalitis is a synaptic autoimmune disorder that was first reported in 2010. To date, LGI1 antibody encephalitis is a widely-recognized disease in neurology and psychiatry. In order to aid clinical recognition of the condition, we analyze the clinical characteristics of 13 Chinese LGI1 antibody encephalitis patients. METHODS: We analyzed clinical features of patients admitted to the West China Hospital who had been diagnosed with LGI1 antibody encephalitis from 2015 to 2017...
November 14, 2017: Comprehensive Psychiatry
https://www.readbyqxmd.com/read/29185868/three-case-studies-of-nonparaneoplastic-limbic-encephalitis-nple-in-young-adult-males-onset-course-and-recovery-following-rehabilitation-services
#5
Robert Perna, Lindsey Harik, Ana Arenivas
Limbic encephalitis (LE) is a rare neurological disorder characterized by inflammation of the brain caused by autoimmunity or infection. LE has been a difficult to define and diagnose disorder due to the insidious and nonspecific (e.g., irritability, low mood, short-term memory complaints) presentation of early symptoms, as well as inconsistent findings on neuroimaging, lumbar puncture serum analysis, and electroencephalogram. Seizures, memory problems, and psychiatric disturbance are among the earliest and most prominent clinical features...
November 29, 2017: Applied Neuropsychology. Child
https://www.readbyqxmd.com/read/29180979/clinical-characteristics-and-predictors-of-outcome-for-onconeural-antibody-associated-disorders-a-retrospective-analysis
#6
Shaohua Liao, Ying Qian, Huaiqiang Hu, Bing Niu, Hongwei Guo, Xiaoling Wang, Shuai Miao, Chuanfen Li, Bingzhen Cao
Objective: To describe and analyze the clinical characteristics, laboratory data, management, and outcome of patients with onconeural antibody-associated disorders (OAAD) and identify predictors for poor outcome. Methods: This was a retrospective review of all patients with potential OAAD, who were hospitalized in Jinan General Hospital between September 2009 and July 2017. We clarified the diagnosis, collected comprehensive information and categorized patients into three groups: paraneoplastic neurological disorders (PNDs), autoimmune encephalitis (AE), and possible OAAD...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/29166136/the-gamma-aminobutyric-acid-b-receptor-gabab-encephalitis-clinical-manifestations-and-response-to-immunotherapy
#7
Junzhao Cui, Hui Bu, Junying He, Zeyan Zhao, Weixin Han, Ruiping Gao, Xiaoqing Li, Qing Li, Xiaosu Guo, Yueli Zou
PURPOSE: We report 11 patients diagnosed with GABAB receptor (GABABR) antibodies encephalitis in China and aim to analyse the clinical characteristics, laboratory and imaging findings, therapeutic modalities and outcomes. METHODS: Clinical data from patients diagnosed with anti-GABAB receptor encephalitis in the Second Affiliated Hospital of Hebei Medical University from February 2016 to October 2016 January were retrospectively collected and evaluated. RESULTS: Seven of the eleven patients were males, and a mean age at presentation of 63 years (range: 47-79 years)...
November 22, 2017: International Journal of Neuroscience
https://www.readbyqxmd.com/read/29159205/decreased-occipital-lobe-metabolism-by-fdg-pet-ct-an-anti-nmda-receptor-encephalitis-biomarker
#8
John C Probasco, Lilja Solnes, Abhinav Nalluri, Jesse Cohen, Krystyna M Jones, Elcin Zan, Mehrbod S Javadi, Arun Venkatesan
Objective: To compare brain metabolism patterns on fluorodeoxyglucose (FDG)-PET/CT in anti-NMDA receptor and other definite autoimmune encephalitis (AE) and to assess how these patterns differ between anti-NMDA receptor neurologic disability groups. Methods: Retrospective review of clinical data and initial dedicated brain FDG-PET/CT studies for neurology inpatients with definite AE, per published consensus criteria, treated at a single academic medical center over a 10-year period...
January 2018: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/29159189/nmdar-encephalitis-passive-transfer-from-man-to-mouse-by-a-recombinant-antibody
#9
Manish Malviya, Sumanta Barman, Kristin S Golombeck, Jesús Planagumà, Francesco Mannara, Nathalie Strutz-Seebohm, Claudia Wrzos, Fatih Demir, Christine Baksmeier, Julia Steckel, Kim Kristin Falk, Catharina C Gross, Stjepana Kovac, Kathrin Bönte, Andreas Johnen, Klaus-Peter Wandinger, Elena Martín-García, Albert J Becker, Christian E Elger, Nikolaj Klöcker, Heinz Wiendl, Sven G Meuth, Hans-Peter Hartung, Guiscard Seebohm, Frank Leypoldt, Rafael Maldonado, Christine Stadelmann, Josep Dalmau, Nico Melzer, Norbert Goebels
Objective: Autoimmune encephalitis is most frequently associated with anti-NMDAR autoantibodies. Their pathogenic relevance has been suggested by passive transfer of patients' cerebrospinal fluid (CSF) in mice in vivo. We aimed to analyze the intrathecal plasma cell repertoire, identify autoantibody-producing clones, and characterize their antibody signatures in recombinant form. Methods: Patients with recent onset typical anti-NMDAR encephalitis were subjected to flow cytometry analysis of the peripheral and intrathecal immune response before, during, and after immunotherapy...
November 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29158368/anti-hu-associated-autoimmune-limbic-encephalitis-in-a-patient-with-pd-1-inhibitor-responsive-myxoid-chondrosarcoma
#10
Kyriakos P Papadopoulos, Rebecca S Romero, Gabriela Gonzalez, James E Dix, Israel Lowy, Matthew Fury
Autoimmune encephalitis is an uncommon complication of immune checkpoint inhibitor therapy. This article reports a case of fatal anti-Hu-associated autoimmune limbic encephalitis presenting within 8 weeks following anti-PD1 therapy in a patient with myxoid chondrosarcoma and pre-existing anti-Hu antibodies. Although tumor reduction occurred in response to PD-1 inhibitor therapy, the patient had a rapidly progressive decline in neurologic function despite initial stabilization with immunosuppression. Considering the increasing use of immune checkpoint inhibitors for the treatment of various malignancies, an increase in the occurrence of neurologic adverse events is likely, requiring prompt intervention and enhanced pharmacovigilance in malignancies associated with onconeuronal antibodies...
November 20, 2017: Oncologist
https://www.readbyqxmd.com/read/29157501/evaluation-and-management-of-autoimmune-encephalitis-a-clinical-overview-for-the-practicing-child-psychiatrist
#11
REVIEW
GenaLynne C Mooneyham, William Gallentine, Heather Van Mater
Medical conditions that present with psychiatric symptoms are becoming increasingly well-recognized in response to the emergence of the field of neuroimmunology. As the availability of testing for novel antineuronal antibodies has increased, so too has the clinical awareness of this diagnostic spectrum. Psychiatrists may have little exposure to this area of expertise, yet may be called on to assist in the diagnosis and treatment of patients with complex neuropsychiatric syndromes secondary to autoimmune encephalitis...
January 2018: Child and Adolescent Psychiatric Clinics of North America
https://www.readbyqxmd.com/read/29156319/large-scale-analysis-of-herpesviridae-in-epilepsy-patients-with-signs-of-autoimmune-encephalitis
#12
Freya Poulheim, Laura Esposito, Christian E Elger, Anna M Eis-Hübinger, Albert J Becker, Pitt Niehusmann
PURPOSE: Epilepsy is one of the most common primary brain disorders. Nonparaneoplastic autoimmune encephalitis is increasingly recognized as an important cause of adult onset epilepsy. However, only in rare cases an initiating factor of the syndrome can be identified. Autoantibody detection after central nervous herpesvirus infection indicates a postviral etiology in a subgroup of patients. In order to analyze a possible underrecognition of postinfectious autoimmunity we performed a large-scale analysis of herpesvirus DNA in cerebrospinal fluid samples from patients with clinical signs of autoimmune encephalitis...
November 17, 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/29154389/latent-autoimmune-diabetes-and-limbic-encephalitis-with-antibodies-against-glutamic-acid-decarboxylase
#13
Stoyan Popkirov, Seena Sebastian, Fatme Seval Ismail, Jörg Wellmer
Antibodies against glutamic acid decarboxylase (GAD) are a hallmark of type 1 diabetes and its late-onset variant, latent autoimmune diabetes of the adult (LADA).(1) While cerebrovascular disease and dementia are common causes of cognitive dysfunction and neurological deficits in diabetic patients,(2,3) rare autoimmune disorders of the central nervous system such as stiff-person syndrom or limbic encephalits can also occur on the basis of shared GAD autoimmunity.(4,5).
November 20, 2017: Journal of Diabetes
https://www.readbyqxmd.com/read/29147884/ocular-flutter-following-zika-virus-infection
#14
Emely Karam, Jose Giraldo, Flor Rodriguez, Carlos E Hernandez-Pereira, Alfonso J Rodriguez-Morales, Gabriela M Blohm, Alberto E Paniz-Mondolfi
Zika virus (ZIKV) is an emerging flavivirus which has been linked to a number of neurologic manifestations such as Guillain-Barré syndrome (GBS), transverse myelitis, and meningo-encephalitis. Ophthalmologic manifestations are increasingly being reported; however, ocular dyskinesias have not been described in this context to date. Herein, we report a case of a 22-year-old female who presented with ocular flutter and associated Guillain-Barré syndrome following acute ZIKV infection. We speculate that although such symptoms may have originated from a direct viral insult, a post-infectious autoimmune mechanism may not be excluded...
November 16, 2017: Journal of Neurovirology
https://www.readbyqxmd.com/read/29145168/frontal-infraslow-activity-marks-the-motor-spasms-of-anti-lgi1-encephalitis
#15
Richard Wennberg, Claude Steriade, Robert Chen, Danielle Andrade
OBJECTIVE: The clinical and electrographic features of seizures in anti-LGI1 encephalitis are distinct from those seen in other autoimmune encephalitides or non-encephalitic epilepsies. One electroclinical phenomenon specific to the condition consists of lateralized motor spasms, known as faciobrachial dystonic seizures (FBDS). An electrodecremental pattern overriding a "DC shift" has been described as the EEG correlate of these spasms. We sought to further characterize this pre-spasm infraslow activity (ISA)...
October 28, 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/29141829/cerebral-cortical-encephalitis-followed-by-recurrent-cns-demyelination-in-a-patient-with-concomitant-anti-mog-and-anti-nmda-receptor-antibodies
#16
Lei Zhou, Jingzi ZhangBao, Haiqing Li, Xiaoyang Li, Yongheng Huang, Min Wang, Chongbo Zhao, Jiahong Lu, Chuanzhen Lu, Yuxin Li, Chao Quan
We report the case of a patient who initially presented with fever, headache and seizure. MRI revealed a fluid attenuation inversion recovery (FLAIR) high-intensity lesion involving the right temporal, parietal and occipital cortex. Afterwards, the patient developed three recurrent episodes, manifested as brainstem encephalitis, optic neuritis and ADEM-like illness successively, indicating demyelination. Both of his serum anti-MOG and CSF anti-NMDAR antibodies were proved positive by transfected cell based assays...
November 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/29141790/cerebral-toxoplasmosis-in-an-ms-patient-receiving-fingolimod
#17
Alejandro Enriquez-Marulanda, Jaime Valderrama-Chaparro, Laura Parrado, Juan Diego Vélez, Ana Maria Granados, Jorge Luis Orozco, Jairo Quiñones
Multiple Sclerosis (MS) is an autoimmune disease in which lymphocytes target putative myelin antigens in the CNS, causing inflammation and neurodegeneration. Fingolimod (FTY720) is an immunosuppressive drug used as a second line therapy for relapsing forms of MS due to its safety profile and good response to treatment. Despite its safety, there are still concerns about the possibility of Fingolimod being linked to the development of opportunistic infections like disseminated varicella zoster infections and herpes simplex encephalitis...
November 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/29136601/no-neuronal-autoantibodies-detected-in-plasma-of-patients-with-a-bipolar-i-disorder
#18
Gijsje Snijders, Maarten J Titulaer, Veerle Bergink, Anna E Bastiaansen, Marco W J Schreurs, Roel A Ophoff, Marco P Boks, René S Kahn, Lot D de Witte
A subpopulation of patients with bipolar disorder type I (BD-I) might suffer from undiagnosed autoimmune encephalitis. We tested plasma of 104 BD-I patients with a current or recent manic episode in the past 2 years for the presence of neuronal autoantibodies using immunohistochemistry, immunocytochemistry and cell-based assay (CBA). Neuronal antibodies were not detected in any of the BD type I. This finding suggests that the frequency of an undiagnosed autoimmune encephalitis in patients with BD I is less than 1%...
November 2, 2017: Psychiatry Research
https://www.readbyqxmd.com/read/29118423/tolerogenic-dendritic-cells-are-efficiently-generated-using-minocycline-and-dexamethasone
#19
Jae-Hee Lee, Chan-Su Park, Sundong Jang, Ji-Wan Kim, Sang-Hyeon Kim, Sukgil Song, Kyungjae Kim, Chong-Kil Lee
Tolerogenic dendritic cells (tDCs) represent a promising tool for cellular therapy against autoimmune diseases, allergies, and transplantation rejection. Numerous pharmacological agents are known to induce tDC generation. Minocycline, which has long been used as a broad-spectrum antibiotic, was recently shown to significantly increase the generation of DCs with regulatory properties. Here, we examined the effect of the combination of minocycline with dexamethasone, rapamycin, vitamin D3, and interleukin (IL)-10, which are all known inducers of tDC generation...
November 8, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29110776/psychiatric-syndromes-other-than-dementia
#20
Karl Bechter, Florian Deisenhammer
There is wide variability in how psychiatry guidelines and textbooks address the question of cerebrospinal fluid (CSF) diagnostics in the screening of psychiatric disorders. A United States-based textbook confirms that there is no consensus about which laboratory investigation should be routinely performed in psychiatric patients, but with respect to CSF diagnostics, the differences are even more striking. A survey among European experts showed a wide variety of opinions regarding clinical use and criteria in various countries of Europe and worldwide: some psychiatrists, mostly university hospital-based, recommended performing CSF diagnostics in every patient first experiencing severe mental illness (SMI), but especially in patients from the schizophrenia spectrum, whereas others almost never perform CSF examinations themselves and usually refer patients to neurology departments if necessary...
2017: Handbook of Clinical Neurology
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