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encephalitis antibodies dalmau

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https://www.readbyqxmd.com/read/27920282/nmda-receptor-encephalitis-and-other-antibody-mediated-disorders-of-the-synapse-the-2016-cotzias-lecture
#1
Josep Dalmau
Investigations during the last 10 years have revealed a group of disorders mediated by antibodies against ion channels and synaptic receptors, which cause both neurologic and psychiatric symptoms. In this review, I discuss the process of discovery and immunologic triggers of these disorders, and use anti-NMDA receptor encephalitis to emphasize the importance of understanding the underlying physiopathologic mechanisms in those diseases. A better knowledge of these mechanisms reveals points of convergence with other disorders (e...
December 6, 2016: Neurology
https://www.readbyqxmd.com/read/27476614/-subacute-anti-n-methyl-d-aspartate-receptor-encephalitis-a-serie-of-13-paediatric-cases
#2
Ricardo Erazo, Jaime González, Consuelo Quintanilla, Claudia Devaud, Consuelo Gayoso, Ximena Toledo, Erna Rauch, Claudia Riffo, Carolina Alvarez, Marne Salazar, Daniela Salvo, Josep Dalmau, Orietta Carmona
INTRODUCTION: Subacute anti-NMDA receptor encephalitis was recognised in 2007 as a clinical entity, and was first described in young women with ovarian teratoma. The first paediatric series unrelated with tumours was reported in 2009. OBJECTIVE: To present the clinical features, treatment, and prognosis of 13 patients with anti-NMDA receptor encephalitis in Chile. PATIENTS AND METHOD: A description is presented of 13 children, 9 males, aged between 1 and 16 years, diagnosed between 2009 and 2016 in 7 hospitals...
July 29, 2016: Revista Chilena de Pediatría
https://www.readbyqxmd.com/read/27466467/anti-lgi1-associated-cognitive-impairment-presentation-and-long-term-outcome
#3
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/27399303/ephrin-b2-prevents-n-methyl-d-aspartate-receptor-antibody-effects-on-memory-and-neuroplasticity
#4
Jesús Planagumà, Holger Haselmann, Francesco Mannara, Mar Petit-Pedrol, Benedikt Grünewald, Esther Aguilar, Luise Röpke, Elena Martín-García, Maarten J Titulaer, Pablo Jercog, Francesc Graus, Rafael Maldonado, Christian Geis, Josep Dalmau
OBJECTIVE: To demonstrate that ephrin-B2 (the ligand of EphB2 receptor) antagonizes the pathogenic effects of patients' N-methyl-D-aspartate receptor (NMDAR) antibodies on memory and synaptic plasticity. METHODS: One hundred twenty-two C57BL/6J mice infused with cerebrospinal fluid (CSF) from patients with anti-NMDAR encephalitis or controls, with or without ephrin-B2, were investigated. CSF was infused through ventricular catheters connected to subcutaneous osmotic pumps over 14 days...
September 2016: Annals of Neurology
https://www.readbyqxmd.com/read/27371488/the-clinical-spectrum-of-caspr2-antibody-associated-disease
#5
Agnes van Sonderen, Helena Ariño, Mar Petit-Pedrol, Frank Leypoldt, Peter Körtvélyessy, Klaus-Peter Wandinger, Eric Lancaster, Paul W Wirtz, Marco W J Schreurs, Peter A E Sillevis Smitt, Francesc Graus, Josep Dalmau, Maarten J Titulaer
OBJECTIVE: To report a large cohort of patients with antibodies against contactin-associated protein-like 2 (Caspr2) and provide the clinical spectrum of this disorder. METHODS: Serum and CSF samples were assessed at 2 neuroimmunology centers in Barcelona and Rotterdam. Patients were included if Caspr2 antibodies were confirmed with 2 independent techniques, including brain immunohistochemistry and cell-based assay. Clinical information was obtained by the authors or provided by treating physicians after patients' informed consent...
August 2, 2016: Neurology
https://www.readbyqxmd.com/read/27271951/association-of-autoimmune-encephalitis-with-combined-immune-checkpoint-inhibitor-treatment-for-metastatic-cancer
#6
Tanya J Williams, David R Benavides, Kelly-Ann Patrice, Josep O Dalmau, Alexandre Leon Ribeiro de Ávila, Dung T Le, Evan J Lipson, John C Probasco, Ellen M Mowry
IMPORTANCE: Paraneoplastic encephalitides usually precede a diagnosis of cancer and are often refractory to immunosuppressive therapy. Conversely, autoimmune encephalitides are reversible conditions that can occur in the presence or absence of cancer. OBJECTIVE: To report the induction of autoimmune encephalitis in 2 patients after treatment of metastatic cancer with a combination of the immune checkpoint inhibitors nivolumab and ipilimumab. DESIGN, SETTING, AND PARTICIPANTS: A retrospective case study was conducted of the clinical and management course of 2 patients with progressive, treatment-refractory metastatic cancer who were treated with a single dose each (concomitantly) of the immune checkpoint inhibitors nivolumab, 1 mg/kg, and ipilimumab, 3 mg/kg...
August 1, 2016: JAMA Neurology
https://www.readbyqxmd.com/read/27170573/human-neurexin-3%C3%AE-antibodies-associate-with-encephalitis-and-alter-synapse-development
#7
Nuria Gresa-Arribas, Jesús Planagumà, Mar Petit-Pedrol, Izumi Kawachi, Shinichi Katada, Carol A Glaser, Mateus M Simabukuro, Thaís Armangué, Eugenia Martínez-Hernández, Francesc Graus, Josep Dalmau
OBJECTIVE: To report a novel autoimmune encephalitis in which the antibodies target neurexin-3α, a cell adhesion molecule involved in the development and function of synapses. METHODS: Five patients with encephalitis and antibodies with a similar pattern of brain reactivity were selected. Antigen precipitation and determination of antibody effects on cultured rat embryonic neurons were performed with reported techniques. RESULTS: Immunoprecipitation and cell-based assays identified neurexin-3α as the autoantigen of patients' antibodies...
June 14, 2016: Neurology
https://www.readbyqxmd.com/read/27111481/association-of-progressive-cerebellar-atrophy-with-long-term-outcome-in-patients-with-anti-n-methyl-d-aspartate-receptor-encephalitis
#8
Takahiro Iizuka, Juntaro Kaneko, Naomi Tominaga, Hidehiro Someko, Masaaki Nakamura, Daisuke Ishima, Eiji Kitamura, Ray Masuda, Eiichi Oguni, Toshiyuki Yanagisawa, Naomi Kanazawa, Josep Dalmau, Kazutoshi Nishiyama
IMPORTANCE: Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is an immune-mediated disorder that occurs with IgG antibodies against the GluN1 subunit of NMDAR. Some patients develop reversible diffuse cerebral atrophy (DCA), but the long-term clinical significance of progressive brain and cerebellar atrophy is unknown. OBJECTIVE: To report the long-term clinical implications of DCA and cerebellar atrophy in anti-NMDAR encephalitis. DESIGN, SETTING, AND PARTICIPANTS: A retrospective observational study and long-term imaging investigation was conducted in the Department of Neurology at Kitasato University...
June 1, 2016: JAMA Neurology
https://www.readbyqxmd.com/read/27065452/clinical-and-immunologic-investigations-in-patients-with-stiff-person-spectrum-disorder
#9
Eugenia Martinez-Hernandez, Helena Ariño, Andrew McKeon, Takahiro Iizuka, Maarten J Titulaer, Mateus M Simabukuro, Eric Lancaster, Mar Petit-Pedrol, Jesús Planagumà, Yolanda Blanco, Robert J Harvey, Albert Saiz, Francesc Graus, Josep Dalmau
IMPORTANCE: Symptoms of stiff-person syndrome (SPS), stiff-limb syndrome (SLS), or progressive encephalomyelitis with rigidity, myoclonus, or other symptoms (SPS-plus) can occur with several autoantibodies, but the relative frequency of each antibody, syndrome specificity, and prognostic implications are unclear. OBJECTIVE: To report the clinical and immunologic findings of a large cohort of patients with stiff-person spectrum disorder (SPSD), including SPS, SLS, and SPS-plus...
June 1, 2016: JAMA Neurology
https://www.readbyqxmd.com/read/26906964/a-clinical-approach-to-diagnosis-of-autoimmune-encephalitis
#10
REVIEW
Francesc Graus, Maarten J Titulaer, Ramani Balu, Susanne Benseler, Christian G Bien, Tania Cellucci, Irene Cortese, Russell C Dale, Jeffrey M Gelfand, Michael Geschwind, Carol A Glaser, Jerome Honnorat, Romana Höftberger, Takahiro Iizuka, Sarosh R Irani, Eric Lancaster, Frank Leypoldt, Harald Prüss, Alexander Rae-Grant, Markus Reindl, Myrna R Rosenfeld, Kevin Rostásy, Albert Saiz, Arun Venkatesan, Angela Vincent, Klaus-Peter Wandinger, Patrick Waters, Josep Dalmau
Encephalitis is a severe inflammatory disorder of the brain with many possible causes and a complex differential diagnosis. Advances in autoimmune encephalitis research in the past 10 years have led to the identification of new syndromes and biomarkers that have transformed the diagnostic approach to these disorders. However, existing criteria for autoimmune encephalitis are too reliant on antibody testing and response to immunotherapy, which might delay the diagnosis. We reviewed the literature and gathered the experience of a team of experts with the aims of developing a practical, syndrome-based diagnostic approach to autoimmune encephalitis and providing guidelines to navigate through the differential diagnosis...
April 2016: Lancet Neurology
https://www.readbyqxmd.com/read/26491084/autoimmune-post-herpes-simplex-encephalitis-of-adults-and-teenagers
#11
MULTICENTER STUDY
Thaís Armangue, Germán Moris, Verónica Cantarín-Extremera, Carlos Enrique Conde, Kevin Rostasy, Maria Elena Erro, Juan Carlos Portilla-Cuenca, Eulàlia Turón-Viñas, Ignacio Málaga, Beatriz Muñoz-Cabello, Carmen Torres-Torres, Sara Llufriu, Luis González-Gutiérrez-Solana, Guillermo González, Ignacio Casado-Naranjo, Myrna Rosenfeld, Francesc Graus, Josep Dalmau
OBJECTIVE: To report 14 patients with immune-mediated relapsing symptoms post-herpes simplex encephalitis (HSE) and to compare the clinical and immunologic features of the teenage and adult group with those of young children. METHODS: Prospective observational study of patients diagnosed between June 2013 and February 2015. Immunologic techniques have been reported previously. RESULTS: Among the teenage and adult group (8 patients, median age 40 years, range 13-69; 5 male), 3 had an acute symptom presentation suggesting a viral relapse, and 5 a presentation contiguous with HSE suggesting a recrudescence of previous deficits...
November 17, 2015: Neurology
https://www.readbyqxmd.com/read/26439959/clinico-pathological-correlation-in-adenylate-kinase-5-autoimmune-limbic-encephalitis
#12
Adeline S L Ng, Joel Kramer, Alejandro Centurion, Josep Dalmau, Eric Huang, Jennifer A Cotter, Michael D Geschwind
Autoantibodies associated with autoimmune limbic encephalitis (ALE) have been well-characterized, with intracellular neuronal antibodies being less responsive to immunotherapy than antibodies to cell surface antigens. Adenylate kinase 5 (AK5) is a nucleoside monophosphate kinase vital for neuronal-specific metabolism and is located intracellularly in the cytosol and expressed exclusively in the brain. Antibodies to AK5 had been previously identified but were not known to be associated with human disease prior to the report of two patients with AK5-related ALE (Tuzun et al...
October 15, 2015: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/26335665/update-on-neurological-paraneoplastic-syndromes
#13
REVIEW
Romana Höftberger, Myrna R Rosenfeld, Josep Dalmau
PURPOSE OF REVIEW: To provide an update on paraneoplastic neurologic syndromes (PNS), the involved tumors, and types of immune responses. RECENT FINDINGS: PNS are a diverse group of syndromes that may present as a relatively isolated syndrome such as predominant cerebellar degeneration or limbic encephalitis, or with more complex phenotypes such as diffuse encephalomyelitis that affects different levels of the neuraxis producing a variety of clinical manifestations...
November 2015: Current Opinion in Oncology
https://www.readbyqxmd.com/read/26291285/anti-dppx-encephalitis-pathogenic-effects-of-antibodies-on-gut-and-brain-neurons
#14
Johannes Piepgras, Markus Höltje, Klaus Michel, Qin Li, Carolin Otto, Christoph Drenckhahn, Christian Probst, Michael Schemann, Sven Jarius, Winfried Stöcker, Bettina Balint, Hans-Michael Meinck, Ralph Buchert, Josep Dalmau, Gudrun Ahnert-Hilger, Klemens Ruprecht
OBJECTIVE: To characterize pathogenic effects of antibodies to dipeptidyl-peptidase-like protein 6 (DPPX), a subunit of Kv4.2 potassium channels, on gut and brain neurons. METHODS: We identified a new patient with anti-DPPX encephalitis and analyzed the effects of the patient's serum and purified immunoglobulin G (IgG), and of serum of a previous patient with anti-DPPX encephalitis, on the activity of enteric neurons by voltage-sensitive dye imaging in guinea pig myenteric and human submucous plexus preparations...
September 8, 2015: Neurology
https://www.readbyqxmd.com/read/26183699/autoimmune-encephalitis-in-postpartum-psychosis
#15
Veerle Bergink, Thaís Armangue, Maarten J Titulaer, Sander Markx, Josep Dalmau, Steven A Kushner
OBJECTIVE: Significant immunological alterations have been observed in women with first-onset affective psychosis during the postpartum period. Recent studies have highlighted the possibility that a subset of patients with first-onset severe psychiatric episodes might suffer from undiagnosed autoimmune encephalitis. Therefore, the authors performed a three-step immunohistochemistry-based screening for CNS autoantibodies in a large cohort of patients with postpartum psychosis and matched postpartum comparison subjects...
September 1, 2015: American Journal of Psychiatry
https://www.readbyqxmd.com/read/26099072/paraneoplastic-neurological-syndromes-and-glutamic-acid-decarboxylase-antibodies
#16
Helena Ariño, Romana Höftberger, Nuria Gresa-Arribas, Eugenia Martínez-Hernández, Thaís Armangue, Michael C Kruer, Javier Arpa, Julio Domingo, Bojan Rojc, Luis Bataller, Albert Saiz, Josep Dalmau, Francesc Graus
IMPORTANCE: Little is known of glutamic acid decarboxylase antibodies (GAD-abs) in the paraneoplastic context. Clinical recognition of such cases will lead to prompt tumor diagnosis and appropriate treatment. OBJECTIVE: To report the clinical and immunological features of patients with paraneoplastic neurological syndromes (PNS) and GAD-abs. DESIGN, SETTING, AND PARTICIPANTS: Retrospective case series study and immunological investigations conducted in February 2014 in a center for autoimmune neurological disorders...
August 2015: JAMA Neurology
https://www.readbyqxmd.com/read/26014349/bipolar-disorder-and-antibodies-against-the-n-methyl-d-aspartate-receptor-a-gate-to-the-involvement-of-autoimmunity-in-the-pathophysiology-of-bipolar-illness
#17
REVIEW
J León-Caballero, I Pacchiarotti, A Murru, M Valentí, F Colom, B Benach, V Pérez, J Dalmau, E Vieta
The high prevalence of comorbidity between bipolar disorder (BD) and other medical conditions, including autoimmune diseases, supports the hypothesis of the nature of BD as a biological illness category. Hence, an immune dysregulation process may play an important role in the development of at least certain subtypes of BD. Increasing evidence also suggests that the N-methyl-d-aspartate receptor (NMDAR) may be relevant in the pathophysiology of BD. A possible key mechanism underlying the physiopathology of certain autoimmune diseases that may present with affective symptoms might be the production of anti-NMDAR auto-antibodies (auto-Abs)...
August 2015: Neuroscience and Biobehavioral Reviews
https://www.readbyqxmd.com/read/25979696/encephalitis-and-ampa-receptor-antibodies-novel-findings-in-a-case-series-of-22-patients
#18
Romana Höftberger, Agnes van Sonderen, Frank Leypoldt, David Houghton, Michael Geschwind, Jeffrey Gelfand, Mercedes Paredes, Lidia Sabater, Albert Saiz, Maarten J Titulaer, Francesc Graus, Josep Dalmau
OBJECTIVE: We report the clinical features, comorbidities, and outcome of 22 newly identified patients with antibodies to the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR). METHODS: This was a retrospective review of patients diagnosed between May 2009 and March 2014. Immunologic techniques have been reported previously. RESULTS: Patients' median age was 62 years (range 23-81; 14 female). Four syndromes were identified: 12 (55%) patients presented with distinctive limbic encephalitis (LE), 8 (36%) with limbic dysfunction along with multifocal/diffuse encephalopathy, one with LE preceded by motor deficits, and one with psychosis with bipolar features...
June 16, 2015: Neurology
https://www.readbyqxmd.com/read/25909091/the-growing-spectrum-of-antibody-associated-inflammatory-brain-diseases-in-children
#19
Sandra Bigi, Manisha Hladio, Marinka Twilt, Josep Dalmau, Susanne M Benseler
OBJECTIVE: To describe the clinical spectrum, diagnostic evaluation, current management, and neurologic outcome of pediatric antibody-associated inflammatory brain diseases (AB-associated IBrainD). METHODS: We performed a single-center retrospective cohort study of consecutive patients aged ≤18 years diagnosed with an AB-associated IBrainD at The Hospital for Sick Children, Toronto, Ontario, Canada, between January 2005 and June 2013. Standardized clinical data, laboratory test results, neuroimaging features, and treatment regimens were captured...
June 2015: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/25820508/a-rare-case-of-anti-n-methyl-d-aspartate-receptor-encephalitis-during-pregnancy
#20
Lai Wan Chan, Christer Nilsson, Jan Schepel, Christopher Lynch
Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis was first described as a severe form of encephalitis by Dalmau et al in 2006. This is an autoimmune disorder usually associated with paraneoplastic mechanism that manifests as neuropsychiatric disorder affecting mainly women of child-bearing age. Nevertheless anti- NMDA receptor encephalitis is a rare condition during pregnancy. To -date, there have been only four reported cases during pregnancy. We report a case of a 23-year-old primigravida in first trimester pregnancy with altered mental status and multiple neurological symptoms related to anti-NMDA receptor encephalitis...
March 27, 2015: New Zealand Medical Journal
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