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limbic encephalitis

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https://www.readbyqxmd.com/read/27919412/anti-nmda-a-nmdar-receptor-encephalitis-related-to-acute-consumption-of-metamphetamine-relevance-of-differential-diagnosis
#1
O Iriondo, J Zaldibar-Gerrikagoitia, T Rodríguez, J M García, L Aguilera
A 19-year-old male came to the Emergency Room of our hospital due to an episode of dystonic movements and disorientation 4 days after consuming methamphetamine, which evolved to a catatonic frank syndrome and eventually to status epilepticus. Definitive diagnosis was anti-NMDA receptor encephalitis, an acute inflammation of the limbic area of autoimmune origin in which early diagnosis and treatment are key elements for the final outcome. In this case, initial normal tests and previous methamphetamine poisoning delayed diagnosis, because inhaled-methamphetamine poisoning causes similar clinical symptoms to anti-NMDA receptor encephalitis...
December 2, 2016: Revista Española de Anestesiología y Reanimación
https://www.readbyqxmd.com/read/27893017/evaluation-of-cognitive-deficits-and-structural-hippocampal-damage-in-encephalitis-with-leucine-rich-glioma-inactivated-1-antibodies
#2
Carsten Finke, Harald Prüss, Josephine Heine, Sigrid Reuter, Ute A Kopp, Florian Wegner, Florian Then Bergh, Sebastian Koch, Olav Jansen, Thomas Münte, Günther Deuschl, Klemens Ruprecht, Winfried Stöcker, Klaus-Peter Wandinger, Friedemann Paul, Thorsten Bartsch
Importance: Limbic encephalitis with leucine-rich, glioma-inactivated 1 (LGI1) antibodies is one of the most frequent variants of autoimmune encephalitis with antibodies targeting neuronal surface antigens. However, the neuroimaging pattern and long-term cognitive outcome are not well understood. Objective: To study cognitive outcome and structural magnetic resonance imaging (MRI) alterations in patients with anti-LGI1 encephalitis. Design, Setting, and Participants: A cross-sectional study was conducted at the Departments of Neurology at Charité-Universitätsmedizin Berlin and University Hospital Schleswig-Holstein, Kiel, Germany...
November 21, 2016: JAMA Neurology
https://www.readbyqxmd.com/read/27855355/seizure-control-and-cognitive-improvement-via-immunotherapy-in-late-onset-epilepsy-patients-with-paraneoplastic-versus-gad65-autoantibody-associated-limbic-encephalitis
#3
N Hansen, G Widman, J-A Witt, J Wagner, A J Becker, C E Elger, C Helmstaedter
OBJECTIVE: To determine the efficacy of immunotherapy in limbic encephalitis (LE) associated epilepsies with autoantibodies against intracellular antigens in the forms of paraneoplastic autoantibodies versus glutamic acid decarboxylase 65 (GAD)-autoantibodies. METHODS: Eleven paraneoplastic-antibodies+ and eleven age- and gender-matched GAD-antibodies+ patients with LE were compared regarding EEG, seizure frequency, MRI volumetry of the brain, and cognition. All patients received immunotherapy with corticosteroids add-on to antiepileptic therapy...
November 14, 2016: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/27847683/clinical-utility-of-seropositive-voltage-gated-potassium-channel-complex-antibody
#4
Adham Jammoul, Luay Shayya, Karin Mente, Jianbo Li, Alexander Rae-Grant, Yuebing Li
BACKGROUND: Antibodies against voltage-gated potassium channel (VGKC)-complex are implicated in the pathogenesis of acquired neuromyotonia, limbic encephalitis, faciobrachial dystonic seizure, and Morvan syndrome. Outside these entities, the clinical value of VGKC-complex antibodies remains unclear. METHODS: We conducted a single-center review of patients positive for VGKC-complex antibodies over an 8-year period. RESULTS: Among 114 patients positive for VGKC-complex antibody, 11 (9...
October 2016: Neurology. Clinical Practice
https://www.readbyqxmd.com/read/27786401/anti-contactin-associated-protein-2-encephalitis-relevance-of-antibody-titres-presentation-and-outcome
#5
C G Bien, Z Mirzadjanova, C Baumgartner, M D Onugoren, T Grunwald, M Holtkamp, S Isenmann, P Kermer, N Melzer, M Naumann, M Riepe, W R Schäbitz, T J von Oertzen, F von Podewils, H Rauschka, T W May
BACKGROUND AND PURPOSE: To clarify the relevance of titres of IgG antibodies against contactin-associated protein-2 (CASPR2) in diagnosing anti-CASPR2 encephalitis and to describe features and outcomes. METHODS: This was a retrospective analysis of 64 patients with CASPR2 antibodies, categorized independently as 'autoimmune encephalitis' or 'other disease'. Logistic regression methods were performed to identify potential predictors of 'autoimmune encephalitis' in addition to CASPR2 antibodies...
October 27, 2016: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/27776544/anti-n-methyl-d-aspartate-receptor-encephalitis-with-an-imaging-invisible-ovarian-teratoma-a-case-report
#6
Zainab M Abdul-Rahman, Peter K Panegyres, Margareta Roeck, David Hawkins, Jude Bharath, Paul Grolman, Cliffe Neppe, David Palmer
BACKGROUND: Anti-N-methyl-D-aspartate receptor encephalitis is a recently discovered disease entity of paraneoplastic limbic encephalitis. It largely affects young women and is often associated with an ovarian teratoma. It is a serious yet treatable condition if diagnosed early. Its remedy involves immunotherapy and surgical removal of the teratoma of the ovaries. This case of anti-N-methyl-D-aspartate receptor encephalitis involves an early surgical intervention with bilateral oophorectomy, despite negative imaging evidence of a teratoma...
October 24, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27737869/lgi1-encephalitis-a-disease-of-jerks-and-confusion
#7
Deepa Dash, Madhavi Tripathi, Kavish Ihtisham, Manjari Tripathi
Limbic encephalitis is a group of immune-mediated disorders that includes the classic paraneoplastic encephalitic syndrome and the recently described non-paraneoplastic autoimmune encephalitis most of which target the extracellular antigens. We present a case of 70-year-old man who presented with rapidly progressive cognitive decline and refractory faciobrachial dystonic seizures and demonstrated seropositivity for leucine-rich, glioma-inactivated protein 1 antibodies. After immunomodulation, the patient had dramatic improvement in the cognitive functioning and in seizure control...
October 13, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27728547/anti-nmda-receptor-encephalitis-limbic-encephalitis-with-corticospinal-involvement-in-a-middle-aged-indian-female
#8
Kanchana S Pillai, Pratik Padole, Uma Sundar
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27717669/the-lgi1-adam22-protein-complex-in-synaptic-transmission-and-synaptic-disorders
#9
Yuko Fukata, Norihiko Yokoi, Yuri Miyazaki, Masaki Fukata
Physiological functioning of the brain requires fine-tuned synaptic transmission, and its dysfunction causes various brain disorders such as autism, dementia, and epilepsy. It is therefore extremely important to identify and characterize key regulators of synaptic function. In particular, disease-related synaptic proteins, such as autism-related neurexin-neuroligin and psychiatric disorder-related NMDA receptor, have attracted considerable attention. Recent basic and clinical research has highlighted critical roles of a ligand-receptor complex, LGI1-ADAM22, in synaptic transmission and brain function, as mutations in the LGI1 gene cause autosomal dominant lateral temporal lobe epilepsy and autoantibodies to LGI1 cause limbic encephalitis which is characterized by memory loss and seizures...
October 4, 2016: Neuroscience Research
https://www.readbyqxmd.com/read/27710962/voltage-gated-potassium-channel-antibodies-in-slow-progression-motor-neuron-disease
#10
Massimiliano Godani, Marco Zoccarato, Alessandro Beronio, Luigi Zuliani, Luana Benedetti, Bruno Giometto, Massimo Del Sette, Elisa Raggio, Roberta Baldi, Angela Vincent
BACKGROUND: The spectrum of autoimmune neurological diseases associated with voltage-gated potassium channel (VGKC)-complex antibodies (Abs) ranges from peripheral nerve disorders to limbic encephalitis. Recently, low titers of VGKC-complex Abs have also been reported in neurodegenerative disorders, but their clinical relevance is unknown. OBJECTIVE: The aim of the study was to explore the prevalence of VGKC-complex Abs in slow-progression motor neuron disease (MND)...
October 7, 2016: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/27709866/relapsing-polychondritis-with-central-nervous-system-involvement-experience-of-three-different-cases-in-a-single-center
#11
Chan Hong Jeon
Relapsing polychondritis (RP) is an autoimmune disorder characterized by inflammation in cartilaginous structures including the ears, noses, peripheral joints, and tracheobronchial tree. It rarely involves the central nervous system (CNS) but diagnosis of CNS complication of RP is challenging because it can present with varying clinical features. Herein we report 3 cases of relapsing polychondritis involving CNS with distinct manifestations and clinical courses. The first patient presented with rhombencephalitis resulting in brain edema and death...
November 2016: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/27667490/-autoimmune-encephalitis-associated-with-malignant-tumors
#12
Takashi Inuzuka
Autoimmune encephalitis consists of limbic symptoms and signs associated with antibodies against neuronal cell-surface antigens or intracellular antigens. Some cases are known to be associated with anti-channel or anti-receptor-related molecule antibodies. Whether these cases are paraneoplastic depends on the kinds of antigens that the antibodies are produced against. Other cases due to well-characterized onco-neural antibodies are almost always paraneoplastic and are generally resistant to anti-tumor therapy and/or immunotherapy...
September 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/27629954/paraneoplastic-limbic-encephalitis-in-a-human-epidermal-growth-factor-receptor-2-positive-gastric-cancer-patient-treated-with-trastuzumab-combined-chemotherapy-a-case-report-and-literature-review
#13
Yu Uneno, Akira Yokoyama, Yoshitaka Nishikawa, Taro Funakoshi, Yoshinao Ozaki, Ikuo Aoyama, Kiichiro Baba, Daisuke Yamaguchi, Shuko Morita, Yukiko Mori, Masashi Kanai, Hisanori Kinoshita, Takeshi Inoue, Nobukatsu Sawamoto, Riki Matsumoto, Shigemi Matsumoto, Manabu Muto
Paraneoplastic neurological syndromes (PNSs) are rare nervous system dysfunctions in cancer patients, which are primarily observed with small-cell lung cancer, gynecological cancer, and thymoma. We herein present an uncommon case of PNS in an anti-Hu antibody-positive patient with human epidermal growth factor receptor (HER)-2-positive gastric cancer (GC), who developed limbic encephalitis and a worsening cognitive function. Trastuzumab-combined chemotherapy was initiated and appeared to be partially effective for controlling the neurological symptoms and tumor volume...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27592059/autoimmune-neurological-syndromes-associated-limbic-encephalitis-and-paraneoplastic-cerebellar-degeneration
#14
Zeynep Özözen Ayas, Dilcan Kotan, Yeşim Güzey Aras
INTRODUCTION: Autoimmune neurological syndrome is a group of disorders caused by cancer affecting nervous system by different immunological mechanisms. In this study, we aim to study the clinical symptoms, cerebrospinal fluid (CSF) findings, autoantibody tests, computed tomography (CT), magnetic resonance imaging (MRI) signs and treatment outcome of patients with autoimmune syndromes. METHODS: In this study, 7 patients (4 male, 3 female) diagnosed with autoimmune neurological syndrome were retrospectively examined...
October 6, 2016: Neuroscience Letters
https://www.readbyqxmd.com/read/27590293/anti-lgi1-encephalitis-clinical-syndrome-and-long-term-follow-up
#15
Agnes van Sonderen, Roland D Thijs, Elias C Coenders, Lize C Jiskoot, Esther Sanchez, Marienke A A M de Bruijn, Marleen H van Coevorden-Hameete, Paul W Wirtz, Marco W J Schreurs, Peter A E Sillevis Smitt, Maarten J Titulaer
OBJECTIVE: This nationwide study gives a detailed description of the clinical features and long-term outcome of anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis. METHODS: We collected patients prospectively from October 2013, and retrospectively from samples sent to our laboratory from January 2007. LGI1 antibodies were confirmed with both cell-based assay and immunohistochemistry. Clinical information was obtained in interviews with patients and their relatives and from medical records...
October 4, 2016: Neurology
https://www.readbyqxmd.com/read/27590077/voltage-gated-potassium-channel-antibody-limbic-encephalitis-a-case-illustrating-the-neuropsychiatric-and-pet-ct-features-with-clinical-and-imaging-follow-up
#16
Liesl Celliers, Te-Jui Hung, Zeyad Al-Ogaili, Girolamo Moschilla, Wally Knezevic
OBJECTIVE: To illustrate the neuropsychiatric and imaging findings in a confirmed case of voltage-gated potassium channel antibody limbic encephalitis. METHOD: Case report and review of the literature. RESULTS: A 64-year-old man presented with several months' history of obsessive thoughts and compulsions associated with faciobrachial dystonic seizures. He had no significant past medical and psychiatric history. Physical examinations revealed only mildly increased tone in the left upper limb...
September 2, 2016: Australasian Psychiatry: Bulletin of Royal Australian and New Zealand College of Psychiatrists
https://www.readbyqxmd.com/read/27566001/neuropsychological-and-fdg-pet-profiles-in-vgkc-autoimmune-limbic-encephalitis
#17
Alessandra Dodich, Chiara Cerami, Sandro Iannaccone, Alessandra Marcone, Pierpaolo Alongi, Chiara Crespi, Nicola Canessa, Francesca Andreetta, Andrea Falini, Stefano F Cappa, Daniela Perani
BACKGROUND: Limbic encephalitis (LE) is characterized by an acute or subacute onset with memory impairments, confusional state, behavioral disorders, variably associated with seizures and dystonic movements. It is due to inflammatory processes that selectively affect the medial temporal lobe structures. Voltage-gate potassium channel (VGKC) autoantibodies are frequently observed. In this study, we assessed at the individual level FDG-PET brain metabolic dysfunctions and neuropsychological profiles in three autoimmune LE cases seropositive for neuronal VGKC-complex autoantibodies...
October 2016: Brain and Cognition
https://www.readbyqxmd.com/read/27559482/-18-f-fluoro-deoxy-glucose-positron-emission-tomography-scan-should-be-obtained-early-in-cases-of-autoimmune-encephalitis
#18
C R Newey, A Sarwal, S Hantus
Introduction. Autoimmune encephalitis (AE) is a clinically challenging diagnosis with nonspecific neurological symptoms. Prompt diagnosis is important and often relies on neuroimaging. We present a case series of AE highlighting the importance of an early [(18)F]-fluoro-deoxy-glucose positron emission tomography (FDG-PET) scan. Methods. Retrospective review of seven consecutive cases of autoimmune encephalitis. Results. All patients had both magnetic resonance imaging (MRI) and FDG-PET scans. Initial clinical presentations included altered mental status and/or new onset seizures...
2016: Autoimmune Diseases
https://www.readbyqxmd.com/read/27538995/roseolovirus-associated-encephalitis-in-immunocompetent-and-immunocompromised-individuals
#19
Joseph Ongrádi, Dharam V Ablashi, Tetsushi Yoshikawa, Balázs Stercz, Masao Ogata
The roseoloviruses, human herpesvirus (HHV)-6A, HHV-6B, and HHV-7, can cause severe encephalitis or encephalopathy. In immunocompetent children, primary HHV-6B infection is occasionally accompanied by diverse clinical forms of encephalitis. Roseolovirus coinfections with heterologous viruses and delayed primary HHV-7 infection in immunocompetent adults result in very severe neurological and generalized symptoms. Recovery from neurological sequelae is slow and sometimes incomplete. In immunocompromised patients with underlying hematological malignancies and transplantation, frequent single or simultaneous reactivation of roseoloviruses elicit severe, lethal organ dysfunctions, including damages in the limbic system, brain stem, and hippocampus...
August 18, 2016: Journal of Neurovirology
https://www.readbyqxmd.com/read/27523010/limbic-encephalitis-associated-with-sj%C3%A3-gren-s-syndrome-report-of-three-cases
#20
Arzu Çoban, Selen Özyurt, Kaan Meriç, Handan Mısırlı, Erdem Tüzün, Recai Türkoğlu
Sjögren's syndrome (SS) may be complicated by neurological manifestations. We herein report three women (age range 26-60 years old) who all presented with limbic encephalitis (LE) as the predominant clinical feature 3 months to 15 years after the diagnosis of SS. The 26-year-old patient also developed acute motor axonal neuropathy one week after autoimmune encephalitis. All three patients showed contrast-enhanced MRI lesions and inflammatory cerebrospinal fluid findings, while not displaying any anti-neuronal antibodies and showing a remarkable response to immunotherapy...
2016: Internal Medicine
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