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https://www.readbyqxmd.com/read/27893017/evaluation-of-cognitive-deficits-and-structural-hippocampal-damage-in-encephalitis-with-leucine-rich-glioma-inactivated-1-antibodies
#1
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/27781030/seizures-and-epilepsies-due-to-channelopathies-and-neurotransmitter-receptor-dysfunction-a-parallel-between-genetic-and-immune-aspects
#2
REVIEW
Agustina M Lascano, Christian M Korff, Fabienne Picard
Despite intensive research activity leading to many important discoveries, the pathophysiological mechanisms underlying seizures and epilepsy remain poorly understood. An important number of specific gene defects have been related to various forms of epilepsies, and autoimmunity and epilepsy have been associated for a long time. Certain central nervous system proteins have been involved in epilepsy or acute neurological diseases with seizures either due to underlying gene defects or immune dysfunction. Here, we focus on 2 of them that have been the object of particular attention and in-depth research over the past years: the N-methyl-D-aspartate receptor and the leucin-rich glioma-inactivated protein 1 (LGI1)...
September 2016: Molecular Syndromology
https://www.readbyqxmd.com/read/27777346/the-relevance-of-vgkc-positivity-in-the-absence-of-lgi1-and-caspr2-antibodies
#3
James B Lilleker, Matthew S Jones, Rajiv Mohanraj, Agnes van Sonderen, Marco W J Schreurs, Marienke A A M de Bruijn, Peter A E Sillevis Smitt, Maarten J Titulaer
No abstract text is available yet for this article.
October 25, 2016: Neurology
https://www.readbyqxmd.com/read/27760137/secretion-positive-lgi1-mutations-linked-to-lateral-temporal-epilepsy-impair-binding-to-adam22-and-adam23-receptors
#4
Emanuela Dazzo, Emanuela Leonardi, Elisa Belluzzi, Sandro Malacrida, Libero Vitiello, Elisa Greggio, Silvio C E Tosatto, Carlo Nobile
Autosomal dominant lateral temporal epilepsy (ADTLE) is a focal epilepsy syndrome caused by mutations in the LGI1 gene, which encodes a secreted protein. Most ADLTE-causing mutations inhibit LGI1 protein secretion, and only a few secretion-positive missense mutations have been reported. Here we describe the effects of four disease-causing nonsynonymous LGI1 mutations, T380A, R407C, S473L, and R474Q, on protein secretion and extracellular interactions. Expression of LGI1 mutant proteins in cultured cells shows that these mutations do not inhibit protein secretion...
October 2016: PLoS Genetics
https://www.readbyqxmd.com/read/27737869/lgi1-encephalitis-a-disease-of-jerks-and-confusion
#5
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/27717669/the-lgi1-adam22-protein-complex-in-synaptic-transmission-and-synaptic-disorders
#6
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/27713024/microrna-877-5p-is-involved-in-the-trovafloxacin-induced-liver-injury
#7
Ryo Mitsugi, Tomoo Itoh, Ryoichi Fujiwara
Trovafloxacin develops severe hepatotoxicity; however, the underlying mechanism of the trovafloxacin-induced liver injury has not been cleared. It has been shown that microRNAs (miRNAs) can be involved in the development of drug-induced liver injuries. We performed a miRNA microarray analysis to identify hepatic miRNAs that were induced or reduced by trovafloxacin in mice. It was demonstrated that miR-877-5p was the most increased miRNA in the mouse liver 24h after the trovafloxacin administration. To investigate the role of miR-877-5p in the liver, we established miR-877-5p-overexpressed HepG2 cells...
October 3, 2016: Toxicology Letters
https://www.readbyqxmd.com/read/27667487/-voltage-gated-potassium-channel-complex-antibodies-associated-encephalopathy-and-related-diseases
#8
Osamu Watanabe
Voltage-gated potassium channel (VGKC) complex antibodies are auto-antibodies, initially identified in acquired neuromyotonia (aNMT; Isaacs' syndrome), which cause muscle cramps and difficulty in opening the palm of the hands. Subsequently, these antibodies were found in patients presenting with aNMT along with psychosis, insomnia, and dysautonomia, collectively termed Morvan's syndrome (MoS), and in a limbic encephalopathy (LE) patient with prominent amnesia and frequent seizures. Typical LE cases have a distinctive adult-onset, frequent, brief dystonic seizure semiology that predominantly affects the arms and ipsilateral face...
September 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/27609625/prevalence-of-serum-anti-neuronal-autoantibodies-in-patients-admitted-to-acute-psychiatric-care
#9
M Schou, S G Sæther, K Borowski, B Teegen, D Kondziella, W Stoecker, A Vaaler, S K Reitan
BACKGROUND: Autoimmune encephalitis associated with anti-neuronal antibodies may be challenging to distinguish from primary psychiatric disorders. The significance of anti-neuronal antibodies in psychiatric patients without clear evidence of autoimmune encephalitis is unknown. We investigated the serum prevalence of six anti-neuronal autoantibodies in a cohort of unselected patients admitted to acute psychiatric care. METHOD: Serum was drawn from 925 patients admitted to acute psychiatric in-patient care...
September 9, 2016: Psychological Medicine
https://www.readbyqxmd.com/read/27604620/immunoadsorption-or-plasma-exchange-in-the-treatment-of-autoimmune-encephalitis-a-pilot-study
#10
Josephine Heine, Lam-Thanh Ly, Ina Lieker, Torsten Slowinski, Carsten Finke, Harald Prüss, Lutz Harms
Therapeutic apheresis has emerged as a major treatment option for autoantibody-associated inflammatory diseases of the nervous system. This includes patients with autoimmune encephalitides caused by antibodies against neuronal proteins. Plasma exchange (PE) and immunoadsorption (IA) constitute two possibilities to eliminate pathogenic antibodies from patients' plasma, but their efficacy and safety has not been prospectively assessed in larger patient groups of autoimmune encephalitides. In a prospective observational case control study, we, therefore, investigated the disease courses and treatment effects of 21 patients with autoimmune encephalitis associated with NMDAR, LGI1, CASPR2, GAD, mGluR5 and Hu antibodies...
December 2016: Journal of Neurology
https://www.readbyqxmd.com/read/27590293/anti-lgi1-encephalitis-clinical-syndrome-and-long-term-follow-up
#11
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/27577085/mapping-autoantigen-epitopes-molecular-insights-into-autoantibody-associated-disorders-of-the-nervous-system
#12
REVIEW
Nese Sinmaz, Tina Nguyen, Fiona Tea, Russell C Dale, Fabienne Brilot
BACKGROUND: Our knowledge of autoantibody-associated diseases of the central (CNS) and peripheral (PNS) nervous systems has expanded greatly over the recent years. A number of extracellular and intracellular autoantigens have been identified, and there is no doubt that this field will continue to expand as more autoantigens are discovered as a result of improved clinical awareness and methodological practice. In recent years, interest has shifted to uncover the target epitopes of these autoantibodies...
August 30, 2016: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/27566001/neuropsychological-and-fdg-pet-profiles-in-vgkc-autoimmune-limbic-encephalitis
#13
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/27511450/clinical-pathologic-correlations-in-vgkc-subtyped-autoimmune-painful-polyneuropathy
#14
Rajat Lahoria, Sean J Pittock, Avi Gadoth, JaNean K Engelstad, Vanda A Lennon, Christopher J Klein
INTRODUCTION: Voltage-gated Kv1 potassium channel-complex (VGKC) autoantibodies subtyped for leucine-rich-glioma-inactivated-1 (LGI1), contactin-associated-protein-like-2 (CASPR2), and Kv-IgGs have a spectrum of neurological presentations. Painful polyneuropathy is seen in some patients, but nerve pathology descriptions are lacking. METHODS: Clinicopathologic features were studied in subtyped VGKC-autoantibody seropositive patients who had undergone nerve biopsies...
August 11, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27489774/lgi1-negative-faciobrachial-dystonic-like-seizures-originating-from-the-insula
#15
Riddhi Patira, Vidita Khatri, Camilo Gutierrez, Sarah Zubkov
We expand the differential diagnosis of LGI1-positive faciobrachial dystonic seizures (FBDS) by presenting a 67-year-old woman affected by seizures of similar semiology who was found to have insular epilepsy. We report the distinct characteristics of insular faciobrachial dystonic-like seizures that would help clinicians to differentiate them from typical LGI1-positive FBDS, thus, guiding therapy while awaiting antibody results. LGI1-negative faciobrachial dystonic-like seizures should be considered when the seizure semiology includes unilateral and prolonged dystonia without loss of awareness, there is an ictal EEG correlate, MRI is suggestive of insular lesion, and when there is neither clearly associated memory impairment nor hyponatremia...
2016: Epilepsy & Behavior Case Reports
https://www.readbyqxmd.com/read/27485013/from-vgkc-to-lgi1-and-caspr2-encephalitis-the-evolution-of-a-disease-entity-over-time
#16
REVIEW
A van Sonderen, M W J Schreurs, P W Wirtz, P A E Sillevis Smitt, M J Titulaer
A wide variety of clinical syndromes has been associated with antibodies to voltage-gated potassium channels (VGKCs). Six years ago, it was discovered that patients do not truly have antibodies to potassium channels, but to associated proteins. This enabled the distinction of three VGKC-positive subgroups: anti-LGI1 patients, anti-Caspr2 patients and VGKC-positive patients lacking both antibodies. Patients with LGI1-antibodies have a limbic encephalitis, often with hyponatremia, and about half of the patients have typical faciobrachial dystonic seizures...
October 2016: Autoimmunity Reviews
https://www.readbyqxmd.com/read/27468420/identification-of-genetic-variants-of-lgi1-and-rtn4r-ngr1-linked-to-schizophrenia-that-are-defective-in-ngr1-lgi1-signaling
#17
Rhalena A Thomas, Amirthagowri Ambalavanan, Guy A Rouleau, Philip A Barker
BACKGROUND: The protein NgR1 is encoded by RTN4R, a gene linked to schizophrenia. We previously reported NgR1 as receptor for the epilepsy-linked protein LGI1. NgR1 regulates synapse number and synaptic plasticity, whereas LGI1 antagonizes NgR1 signaling and promotes synapse formation. Impairments in synapse formation are common in neurological disease and we hypothesized that an LGI1-NgR1 signaling pathway may contribute to the development of schizophrenia. METHODS: We screened two unrelated schizophrenic populations for variants in RTN4R and LGI1 using whole exome sequencing and Sanger sequencing...
July 2016: Molecular Genetics & Genomic Medicine
https://www.readbyqxmd.com/read/27466467/anti-lgi1-associated-cognitive-impairment-presentation-and-long-term-outcome
#18
Helena Ariño, Thais Armangué, Mar Petit-Pedrol, Lidia Sabater, Eugenia Martinez-Hernandez, Makoto Hara, Eric Lancaster, Albert Saiz, Josep Dalmau, Francesc Graus
OBJECTIVE: We investigated a series of patients with LGI1 antibody (Ab)-related cognitive deterioration to determine the clinical presentation, long-term outcome, and LGI1 Ab evolution. METHODS: We retrospectively analyzed the clinical information of 76 patients with LGI1 Ab-related cognitive deterioration. Presenting syndromes were classified as limbic encephalitis (LE), non-LE, or encephalopathy (normal MRI and no CSF pleocytosis). Frequency of relapses and clinical outcome were assessed in 48 patients with prolonged follow-up (median 39 months, range 18-200)...
August 23, 2016: Neurology
https://www.readbyqxmd.com/read/27462038/serial-arterial-spin-labeling-mri-in-autonomic-status-epilepticus-due-to-anti-lgi1-encephalitis
#19
Camilo Espinosa-Jovel, Rafael Toledano, Irene García-Morales, Juan Álvarez-Linera, Antonio Gil-Nagel
No abstract text is available yet for this article.
July 26, 2016: Neurology
https://www.readbyqxmd.com/read/27450643/antibody-associated-epilepsies-clinical-features-evidence-for-immunotherapies-and-future-research-questions
#20
REVIEW
Ochuko D Bakpa, Markus Reuber, Sarosh R Irani
PURPOSE: The growing recognition of epilepsies and encephalopathies associated with autoantibodies against surface neuronal proteins (LGI1, NMDAR, CASPR2, GABABR, and AMPAR) means that epileptologists are increasingly asking questions about mechanisms of antibody-mediated epileptogenesis, and about the use of immunotherapies. This review summarizes clinical and paraclinical observations related to autoimmune epilepsies, examines the current evidence for the effectiveness of immunotherapy, and makes epilepsy-specific recommendations for future research...
October 2016: Seizure: the Journal of the British Epilepsy Association
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