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limbic encephalitis

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https://www.readbyqxmd.com/read/28096524/a-case-of-seronegative-limbic-encephalitis-with-multiple-sclerosis-a-possible-overlapping-syndrome
#1
Zerrin Karaaslan, Özlem Mercan, Erdem Tüzün, Handan Mısırlı, Recai Türkoğlu
BACKGROUND Autoimmune encephalitis might coexist in patients with autoimmune demyelinating disorders. CASE REPORT We report on a case of a 45-year-old female multiple sclerosis (MS) patient presenting with acute onset short-term memory loss, altered mental status, inflammatory cerebrospinal fluid (CSF) findings and an MRI lesion on the left temporal lobe. An extensive panel for neuronal autoantibodies proved negative. Neuropsychological symptoms gave a prompt response to immunotherapy but nevertheless control MRI showed left hippocampal atrophy...
January 18, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28063151/gad65-neurological-autoimmunity
#2
Andrew McKeon, Jennifer A Tracy
The glutamic acid decarboxylase 65-isoform (GAD65) antibody is a biomarker of autoimmune central nervous system (CNS) disorders and, more commonly, non-neurological autoimmune diseases. Type 1 diabetes, autoimmune thyroid disease, and pernicious anemia are the most frequent GAD65 autoimmune associations. One or more of these disorders coexists in approximately 70% of patients with GAD65 neurological autoimmunity. Neurological phenotypes have CNS localization and include limbic encephalitis, epilepsy, cerebellar ataxia, and stiff-person syndrome (SPS), among others...
January 7, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28062719/characteristics-in-limbic-encephalitis-with-anti-adenylate-kinase-5-autoantibodies
#3
Le-Duy Do, Eve Chanson, Virginie Desestret, Bastien Joubert, François Ducray, Sabine Brugière, Yohann Couté, Maité Formaglio, Veronique Rogemond, Catherine Thomas-Antérion, Laura Borrega, Brice Laurens, Francois Tison, Jonathan Curot, Thomas De Brouker, Christine Lebrun-Frenay, Jean-Yves Delattre, Jean-Christophe Antoine, Jerome Honnorat
OBJECTIVE: To report 10 patients with limbic encephalitis (LE) and adenylate kinase 5 autoantibodies (AK5-Abs). METHODS: We conducted a retrospective study in a cohort of 50 patients with LE with uncharacterized autoantibodies and identified a specific target using immunohistochemistry, Western blotting, immunoprecipitation, mass spectrometry, and cell-based assay. RESULTS: AK5 (a known autoantigen of LE) was identified as the target of antibodies in the CSFs and sera of 10 patients with LE (median age 64 years; range 57-80), which was characterized by subacute anterograde amnesia without seizure and sometimes preceded by a prodromal phase of asthenia or mood disturbances...
January 6, 2017: Neurology
https://www.readbyqxmd.com/read/28062718/do-we-need-to-measure-specific-antibodies-in-patients-with-limbic-encephalitis
#4
Josep Dalmau, Angela Vincent
No abstract text is available yet for this article.
January 6, 2017: Neurology
https://www.readbyqxmd.com/read/28026046/anti-lgi1-encephalitis-is-strongly-associated-with-hla-dr7-and-hla-drb4
#5
Agnes van Sonderen, Dave L Roelen, Johannes A Stoop, Robert M Verdijk, Geert W Haasnoot, Roland D Thijs, Paul W Wirtz, Marco W J Schreurs, Frans H J Claas, Peter A E Sillevis Smitt, Maarten J Titulaer
Leucine-rich glioma-inactivated1 (LGI1)-encephalitis is an antibody-associated inflammation of the limbic area. An autoimmune etiology is suspected but not proven yet. We performed HLA-analysis in 25 non-tumor anti-LGI1 patients and discovered a remarkably strong HLA-association. HLA-DR7 was present in 88% compared to 19.6% in healthy controls (p=4.1*10-11). HLA-DRB4 was present in all patients and in 46.5% controls (p=1.19*10-7). These findings support the autoimmune hypothesis. An exploratory analysis was performed in a small group of four tumor-LGI1 patients...
December 27, 2016: Annals of Neurology
https://www.readbyqxmd.com/read/28026032/the-major-histocompatibility-complex-and-antibody-mediated-limbic-encephalitis
#6
Olaf Stuve, Steven Vernino
No abstract text is available yet for this article.
December 27, 2016: Annals of Neurology
https://www.readbyqxmd.com/read/27980287/a-case-of-neurosyphilis-presenting-with-limbic-encephalitis
#7
Kazuto Tsukita, Akihiro Shimotake, Mitsuyoshi Nakatani, Yukitoshi Takahashi, Akio Ikeda, Ryosuke Takahashi
A 46-year-old man presented with a history of personality change and memory disturbance for 5 months. He gradually became difficult in doing a daily job. Brain MRI showed abnormal hyperintensity within bilateral mesial temporal lobes on T2 weighted image. Therefore, viral and autoimmune limbic encephalitis was initially suspected. However, because treponemal and non-treponemal specific antibodies were positive in serum and cerebrospinal fluid (CSF), the diagnosis of neurosyphilis was made. Patients of neurosyphilis with mesiotemporal T2 weighted hyperintensity reportedly showed common features such as relatively young age, HIV-negative, subacute cognitive impairment and seizure, as seen in our patient...
December 16, 2016: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/27955671/pro-epileptogenic-effects-of-viral-like-inflammation-in-both-mature-and-immature-brains
#8
Nina Dupuis, Andrey Mazarati, Béatrice Desnous, Vibol Chhor, Bobbi Fleiss, Tifenn Le Charpentier, Sophie Lebon, Zsolt Csaba, Pierre Gressens, Pascal Dournaud, Stéphane Auvin
BACKGROUND: Infectious encephalitides are most often associated with acute seizures during the infection period and are risk factors for the development of epilepsy at later times. Mechanisms of viral encephalitis-induced epileptogenesis are poorly understood. Here, we evaluated the contribution of viral encephalitis-associated inflammation to ictogenesis and epileptogenesis using a rapid kindling protocol in rats. In addition, we examined whether minocycline can improve outcomes of viral-like brain inflammation...
December 12, 2016: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/27919412/anti-nmda-a-nmdar-receptor-encephalitis-related-to-acute-consumption-of-metamphetamine-relevance-of-differential-diagnosis
#9
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
#10
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...
January 1, 2017: 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
#11
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...
December 2016: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/27847683/clinical-utility-of-seropositive-voltage-gated-potassium-channel-complex-antibody
#12
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
#13
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...
January 2017: 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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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