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Matt Shirley
Daclizumab (Zinbryta(®); previously known as daclizumab high-yield process) is a therapeutic monoclonal antibody that has recently been approved for the treatment of relapsing forms of multiple sclerosis (MS) in adults. Daclizumab is a humanized IgG1 monoclonal antibody directed against CD25, the alpha subunit of the high-affinity interleukin-2 receptor. As demonstrated in the phase III DECIDE trial, once-monthly subcutaneous daclizumab was superior to once-weekly intramuscular interferon (IFN) β-1a in reducing the clinical relapse rate and radiological measures of disease in patients with relapsing-remitting MS...
February 17, 2017: Drugs
Xavier Montalban, Stephen L Hauser, Ludwig Kappos, Douglas L Arnold, Amit Bar-Or, Giancarlo Comi, Jérôme de Seze, Gavin Giovannoni, Hans-Peter Hartung, Bernhard Hemmer, Fred Lublin, Kottil W Rammohan, Krzysztof Selmaj, Anthony Traboulsee, Annette Sauter, Donna Masterman, Paulo Fontoura, Shibeshih Belachew, Hideki Garren, Nicole Mairon, Peter Chin, Jerry S Wolinsky
Background An evolving understanding of the immunopathogenesis of multiple sclerosis suggests that depleting B cells could be useful for treatment. We studied ocrelizumab, a humanized monoclonal antibody that selectively depletes CD20-expressing B cells, in the primary progressive form of the disease. Methods In this phase 3 trial, we randomly assigned 732 patients with primary progressive multiple sclerosis in a 2:1 ratio to receive intravenous ocrelizumab (600 mg) or placebo every 24 weeks for at least 120 weeks and until a prespecified number of confirmed disability progression events had occurred...
January 19, 2017: New England Journal of Medicine
Stephen L Hauser, Amit Bar-Or, Giancarlo Comi, Gavin Giovannoni, Hans-Peter Hartung, Bernhard Hemmer, Fred Lublin, Xavier Montalban, Kottil W Rammohan, Krzysztof Selmaj, Anthony Traboulsee, Jerry S Wolinsky, Douglas L Arnold, Gaelle Klingelschmitt, Donna Masterman, Paulo Fontoura, Shibeshih Belachew, Peter Chin, Nicole Mairon, Hideki Garren, Ludwig Kappos
Background B cells influence the pathogenesis of multiple sclerosis. Ocrelizumab is a humanized monoclonal antibody that selectively depletes CD20+ B cells. Methods In two identical phase 3 trials, we randomly assigned 821 and 835 patients with relapsing multiple sclerosis to receive intravenous ocrelizumab at a dose of 600 mg every 24 weeks or subcutaneous interferon beta-1a at a dose of 44 μg three times weekly for 96 weeks. The primary end point was the annualized relapse rate. Results The annualized relapse rate was lower with ocrelizumab than with interferon beta-1a in trial 1 (0...
January 19, 2017: New England Journal of Medicine
Ryuki Matsuura, Shin-Ichiro Hamano, Satoru Ikemoto, Yuko Hirata, Kotoko Suzuki, Kenjiro Kikuchi, Yukitoshi Takahashi
A 3-year-old boy with normal development presented with acute cerebellitis at one year and 10 months of age. His truncal ataxia resolved without treatment. He experienced a relapse of truncal ataxia and atonic seizures at 2 years and one month of age. Five months later, he experienced myoclonic atonic seizures. By 3 years of age, the truncal ataxia had become severe, and the frequency of myoclonic atonic seizures increased. Compared to controls, we found higher levels of anti-C-terminal GluN2B and anti-N terminal GluD2 antibodies in the serum, and anti-N terminal GluN2B and anti-C terminal GluD2 antibodies in the cerebrospinal fluid (CSF)...
February 27, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
Agnese Suppiej, Elisa Cainelli, Giulia Casara, Ambra Cappellari, Margherita Nosadini, Stefano Sartori
BACKGROUND: Acute disseminated encephalomyelitis is an inflammatory-demyelinating disorder of the central nervous system usually with a monophasic course and a favorable neurological outcome. Long-term neurocognitive sequelae and quality of life have not yet been fully investigated. AIM: To examine neurocognitive outcome and quality of life in pediatric monophasic acute disseminated encephalomyelitis. METHODS: Of the 36 patients diagnosed with acute disseminated encephalomyelitis at our institution, six were lost to follow-up and eight relapsed (two with multiphasic forms and six with multiple sclerosis)...
April 2014: Pediatric Neurology
Le Zhang, Meng-Qian Wu, Zi-Long Hao, Siew Mun Vance Chiang, Kun Shuang, Min-Tao Lin, Xiao-Sa Chi, Jia-Jia Fang, Dong Zhou, Jin-Mei Li
Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is a recently recognized autoimmune disorder which is responsive to immunotherapy. However, the outcomes of different immunotherapies have not been defined and there have been few studies that carried out a comparison among them. To provide an overview of the clinical characteristics, treatments, and outcomes of anti-NMDAR encephalitis, we systematically reviewed the literature in the PubMed, Medline, Embase, Cochrane Library, BioMedical Literature Database (CBM), China National Knowledge Infrastructure (CNKI), and Wan-fang databases...
March 2017: Epilepsy & Behavior: E&B
W Köhler, S Ehrlich, C Dohmen, M Haubitz, F Hoffmann, S Schmidt, R Klingel, A Kraft, T Neumann-Haefelin, H Topka, O Stich, A Baumgartner, C Fassbender
BACKGROUND AND PURPOSE: Detection of autoantibodies against neuronal surface antigens and their correlation with the pattern and severity of symptoms led to the definition of new autoimmune-mediated forms of encephalitis and was essential for the initiation of immunotherapies including plasma exchange. The elimination of autoantibodies using selective immunoadsorption (IA) is a pathophysiologically guided therapeutic approach but has not yet been evaluated in a separate analysis. METHODS: A retrospective analysis was performed of patients with autoimmune encephalitis who were treated with tryptophan IA in six neurological clinics between 2009 and 2013...
January 2015: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
Andrea Mann, Nikolas Mata Machado, Ni Liu, Abdul-Hussein Mazin, Kenneth Silver, Khalid I Afzal
Anti-NMDAR (N-methyl-d-aspartate receptor) encephalitis is a novel autoimmune and paraneoplastic disease often presenting as acute psychosis. Few studies exist in the psychiatric literature on neuroimmunity and behavioral management. This article reviews the epidemiology, diagnosis, pathophysiology, and management of this disease from a neuropsychiatric perspective. Patients have potential for near-complete recovery with early diagnosis and intervention. In addition to immune-suppression and tumor removal, electroconvulsive therapy may be an important tool in treatment of the underlying process in cases developing life-threatening catatonia...
2012: Journal of Neuropsychiatry and Clinical Neurosciences
Hsiuying Wang
Treatments for anti-N-methyl-D-aspartate (NMDA) receptor encephalitis include immunotherapy with steroids, intravenous immunoglobulin, plasma exchange, or plasmapheresis as first-line treatments, immunotherapy with rituximab or cyclophosphamide as second-line treatments, and tumor removal. In this systematic review, we evaluated previous studies and examined the association between certain microRNAs and anti-NMDA receptor encephalitis to investigate the performance of different treatment combinations. The efficacies of different combinations of treatments classified into the following four categories were compared: (I) intravenous immunoglobulin administration, (II) plasmapheresis or plasma exchange, (III) treatment with rituximab or cyclophosphamide and (IV) tumor removal...
January 1, 2016: Frontiers in Bioscience (Landmark Edition)
Takahiro Iizuka, Shintaro Yoshii, Shinichi Kan, Junichi Hamada, Josep Dalmau, Fumihiko Sakai, Hideki Mochizuki
The long-term neuroimaging correlates of clinical recovery have not been described in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. The aim of the study is to evaluate the long-term outcome of brain atrophy in anti-NMDAR encephalitis. Patients were two women (ages 17 and 33 years) with severe anti-NMDAR encephalitis resulting in decreased level of consciousness, autonomic instability, hypoventilation, and dyskinesias requiring continuous infusion of anesthetic agents for 6-7 months. Brain MRI and cerebral blood flow SPECT obtained at the time of maximal neurological disability were compared with similar studies obtained 5-7 years later...
October 2010: Journal of Neurology
H M H Braakman, V M P Moers-Hornikx, B M G Arts, R M M Hupperts, J Nicolai
No abstract text is available yet for this article.
September 7, 2010: Neurology
Pawan Kashyape, Ellen Taylor, Joanne Ng, Deepa Krishnakumar, Fenella Kirkham, Andrea Whitney
We describe the clinical course and treatment of three unrelated female patients ranging in age from 27 months to 14 years with anti-NMDA receptor encephalitis. The third case is reported as an addendum to the paper. None of the cases were paraneoplastic. All received initial immunotherapy consisting of steroids and IVIg, and two of them received 3 and 8 plasma exchanges respectively, without consistent or sustained clinical improvement. All three girls were then treated with monthly cycles of Cyclophosphamide...
January 2012: European Journal of Paediatric Neurology: EJPN
Antonio Bravo-Oro, Danae Acosta-Yebra, Ilse P Grimaldo-Zapata, Guillermo Reyes-Vaca
INTRODUCTION: Anti-N-methyl-D-aspartate (NMDA) receptor antibody encephalitis was initially described as a paraneoplastic syndrome associated to ovarian teratomas. Yet, an increasing number of reports are being published involving cases of young women and children with signs and symptoms of an autoimmune encephalopathy, in 40-50% of the cases secondary to a viral infection. Clinically, it is characterised by a progressive picture of psychiatric manifestations, convulsive seizures, dyskinesias and dysautonomias...
May 16, 2015: Revista de Neurologia
Kumaran Deiva, Maria Carmela Pera, Hélène Maurey, Pascale Chrétien, Frédérique Archambaud, Viviane Bouilleret, Marc Tardieu
BACKGROUND: Anti NMDA receptor (anti NMDAR) encephalitis is a well-characterized entity in children associating movement disorders, psychiatric features and speech difficulties. Novel phenotypes have been described in adults. METHODS AND RESULTS: A 4-year-old girl presented partial seizures which evolved towards sudden and isolated Broca's aphasia. Anti NMDAR antibodies were positive in CSF and serum confirming anti NMDAR encephalitis. Clinical recovery was observed after a specific treatment...
November 2014: European Journal of Paediatric Neurology: EJPN
Kristin C Jones, Ann C Schwartz, Adriana P Hermida, David A Kahn
We describe the case of a 17-year-old male who presented with acute onset of seizures and malignant catatonia with psychosis, agitation, and hypermetabolism, who responded to electroconvulsive therapy (ECT). Soon after he began to respond, he was diagnosed with anti-N-methyl-D-aspartate (NMDA) receptor encephalitis and then given immunosuppressive therapy. Anti-NMDA receptor encephalitis is an increasingly recognized autoimmune disorder that often presents with neuropsychiatric symptoms. The mainstays for treatment have been early diagnosis, tumor work-up and removal if found, and initiation of immunosuppressive therapy...
September 2015: Journal of Psychiatric Practice
David Mechelhoff, Betteke Maria van Noort, Bernhard Weschke, Christian J Bachmann, Christiane Wagner, Ernst Pfeiffer, Sibylle Winter
Since 2007, more than 600 patients have been diagnosed with anti-N-methyl-D-aspartate (NMDA) receptor encephalitis, with almost 40 % of those affected being children or adolescents. In early phases of the illness, this life-threatening disease is characterized by psychiatric symptoms, such as depression, anxiety, obsessions, hallucinations or delusions. Consequently, a high percentage of patients receive psychiatric diagnoses at first, hindering the crucial early diagnosis and treatment of the anti-NMDA receptor encephalitis...
November 2015: European Child & Adolescent Psychiatry
J Nicholas Brenton, Joshua Kim, Richard H Schwartz
Anti-N-methyl-d-aspartate (anti-NMDA) receptor encephalitis is a treatable cause of autoimmune encephalitis. It remains unclear if the natural history of this disease is altered by choice of acute therapy or the employment of chronic immunotherapy. Chart review was undertaken for pediatric patients diagnosed with anti-NMDA receptor encephalitis. Data obtained included patient demographics, disease manifestations, treatment course, and clinical outcomes. Ten patients with anti-NMDA receptor encephalitis were identified...
August 2016: Journal of Child Neurology
Maarten J Titulaer, Lindsey McCracken, Iñigo Gabilondo, Thaís Armangué, Carol Glaser, Takahiro Iizuka, Lawrence S Honig, Susanne M Benseler, Izumi Kawachi, Eugenia Martinez-Hernandez, Esther Aguilar, Núria Gresa-Arribas, Nicole Ryan-Florance, Abiguei Torrents, Albert Saiz, Myrna R Rosenfeld, Rita Balice-Gordon, Francesc Graus, Josep Dalmau
BACKGROUND: Anti-NMDA receptor (NMDAR) encephalitis is an autoimmune disorder in which the use of immunotherapy and the long-term outcome have not been defined. We aimed to assess the presentation of the disease, the spectrum of symptoms, immunotherapies used, timing of improvement, and long-term outcome. METHODS: In this multi-institutional observational study, we tested for the presence of NMDAR antibodies in serum or CSF samples of patients with encephalitis between Jan 1, 2007, and Jan 1, 2012...
February 2013: Lancet Neurology
Koji Yokoyama, Mutsuki Hori, Akira Yoshida
No abstract text is available yet for this article.
September 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
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
2017-02-07 20:44:27
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