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NMDAR encephalitis

Katherine McIvor, Perry Moore
OBJECTIVE: Anti N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is an autoimmune disorder that was only fully discovered recently and neuropsychological outcome data remains sparse. We present the case of BA, a 19-year-old male, which illustrates the cognitive outcome in an untreated case over a time period of over 2½ years. METHOD: We conducted three cognitive assessments, including tests of memory and executive functioning, over this time period and considered the evidence for reliable change in memory function using the Wechsler Advanced Clinical Solutions (ACS) serial assessment package...
October 20, 2016: Clinical Neuropsychologist
Shigemi Nagayama, Keiko Tanaka
Recently, the search for diagnostic antibody markers has drawn considerable attention in relation to autoimmune encephalitis. Among the antibody markers, the most frequently detected is the anti-N-methyl-D-aspartate receptor (NMDAR)antibody. Patients with this antibody develop characteristic clinical features. This disease tends to affect young women, and starts with psychiatric symptoms followed by seizures, involuntary movements, autonomic failure, and respiratory failure. Nearly half of these female patients have ovarian teratoma...
September 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
Atsurou Yamada, Nobuhiro Miyachi, Toshiyasu Miura, Masako Suzuki, Norio Watanabe, Tatsuo Akechi
INTRODUCTION: Post-infectious encephalitis/encephalopathy is a neurological syndrome that sometimes develops following common viral or bacterial infections. The most common form is acute disseminated encephalomyelitis (ADEM). ADEM is a demyelinating disease of the central nervous system that typically presents as a monophasic disorder associated with multifocal neurologic symptoms and encephalitis. Anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis is another type of severe autoimmune disorder, characterized by seizures, movement disorders and psychiatric symptoms...
2016: SpringerPlus
Qi Huang, Yuan Wu, Rongfa Qin, Xing Wei, Meigang Ma
Anti-N-Methyl-D-Aspartate receptor (NMDAR) encephalitis is an acute neurological disorder affecting children and adults. We aimed to compare the clinical characteristics, treatments, and outcomes between children and adults with anti-NMDAR encephalitis and to assess the probable risk factors. In this observational study, patients who tested positive for anti-NMDAR antibody in the cerebrospinal fluid were enrolled. The patients were divided into children and adults group on the basis of age (whether <16 or not)...
September 15, 2016: Journal of Neurology
Bi-Chun Duan, Wen-Chin Weng, Kuang-Lin Lin, Lee Chin Wong, Sung-Tse Li, Mei-Hsin Hsu, Jainn-Jim Lin, Pi-Chaun Fan, Ming-I Lin, Nan-Chang Chiu, Yu-Ching Lin, Huei-Shyong Wang, Kun-Long Hung, Wang-Tso Lee
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is one of the most common autoimmune encephalitis that presents with a wide variety of movement disorders. The purpose of our study is to review the manifestations and duration of movement disorders in different ages with NMDAR encephalitis.A retrospective cohort of 28 patients (20 females and 8 males) with positive cerebrospinal fluid (CSF) anti-NMDAR antibody in a 5-year period from major hospitals in Taiwan was enrolled. They were categorized into 3 age groups: 7 patients were ≤10 years, 14 patients were 10 to 18 years, and 7 patients were >18 years...
September 2016: Medicine (Baltimore)
Xueping Chen, Jin-Mei Li, Fan Liu, Qiong Wang, Dong Zhou, Xiaohui Lai
Anti-N-methyl-D-aspartate receptor encephalitis (anti-NMDAR encephalitis) is the most common type of immune-mediated encephalitis. This study aimed to assess the incidence and mortality of anti-NMDAR encephalitis in intensive care unit (ICU) to evaluate the clinical manifestations, laboratory findings, managements and outcomes, and to compare these characteristics with patients with non-anti-NMDAR encephalitis admitted to ICU. Patients admitted to the neurological ICU with suspected encephalitis were included between January 1, 2012 and July 31, 2015...
September 12, 2016: Neurological Sciences
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
Jung-Ah Lim, Soon-Tae Lee, Tae-Joon Kim, Jangsup Moon, Jun-Sang Sunwoo, Jung-Ick Byun, Keun-Hwa Jung, Ki-Young Jung, Kon Chu, Sang Kun Lee
The aim of this study was to analyze the clinical presentation and provocation factors of rhabdomyolysis in anti-NMDAR encephalitis. Among the 16 patients with anti-NMDAR encephalitis in our institutional cohort, nine patients had elevated CK enzyme levels and clinical evidence of rhabdomyolysis. Rhabdomyolysis was more frequent after immunotherapy. The use of dopamine receptor blocker (DRB) increased the risk of rhabdomyolysis. None of the patients without rhabdomyolysis received DRBs. Rhabdomyolysis is a frequent complication in anti-NMDAR encephalitis and more common after immunotherapy and the use of DRBs increases the risk...
September 15, 2016: Journal of Neuroimmunology
Florian Lejuste, Laure Thomas, Géraldine Picard, Virginie Desestret, François Ducray, Veronique Rogemond, Dimitri Psimaras, Jean-Christophe Antoine, Jean-Yves Delattre, Laurent Groc, Marion Leboyer, Jerome Honnorat
OBJECTIVE: To precisely describe the initial psychiatric presentation of patients with anti-NMDA receptor (NMDAR) antibodies encephalitis (anti-NMDAR encephalitis) to identify potential clues enhancing its early diagnosis. METHODS: We retrospectively studied the French Reference Centre medical records of every adult patient with anti-NMDAR encephalitis to specify the patients' initial psychiatric symptoms leading to hospitalization in a psychiatric department and the reasons underlying the diagnosis of anti-NMDAR encephalitis...
October 2016: Neurology® Neuroimmunology & Neuroinflammation
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...
September 7, 2016: Journal of Neurology
Kavitha Kothur, Louise Wienholt, Shekeeb S Mohammad, Esther M Tantsis, Sekhar Pillai, Philip N Britton, Cheryl A Jones, Rajeshwar R Angiti, Elizabeth H Barnes, Timothy Schlub, Sushil Bandodkar, Fabienne Brilot, Russell C Dale
BACKGROUND: Despite the discovery of CSF and serum diagnostic autoantibodies in autoimmune encephalitis, there are still very limited CSF biomarkers for diagnostic and monitoring purposes in children with inflammatory or autoimmune brain disease. The cause of encephalitis is unknown in up to a third of encephalitis cohorts, and it is important to differentiate infective from autoimmune encephalitis given the therapeutic implications. AIM: To study CSF cytokines and chemokines as diagnostic biomarkers of active neuroinflammation, and assess their role in differentiating demyelinating, autoimmune, and viral encephalitis...
2016: PloS One
Jessica L Gough, Jan Coebergh, Brunda Chandra, Ramin Nilforooshan
Autoimmune encephalitis is a poorly understood condition that can present with a combination of neurological and psychiatric symptoms, either of which may predominate. There are many autoantibodies associated with a variety of clinical syndromes - anti-N-Methyl-D-Aspartate receptor (NMDAR) is the commonest. Currently, the most widely used therapy is prompt plasmapheresis and steroid treatment (and tumour resection if indicated), followed by second line immunosuppression if this fails. Given the growing awareness of autoimmune encephalitis as an entity, it is increasingly important that we consider it as a potential diagnosis in order to provide timely, effective treatment...
August 16, 2016: World Journal of Clinical Cases
Étienne de Montmollin, Sophie Demeret, Noëlle Brulé, Marie Conrad, Frédéric Dailler, Nicolas Lerolle, Jean-Christophe Navellou, Carole Schwebel, Mikaël Alves, Martin Cour, Nicolas Engrand, Jean-Marie Tonnelier, Eric Maury, Stéphane Ruckly, Géraldine Picard, Véronique Rogemond, Éric Magalhaes, Tarek Sharshar, Jean-François Timsit, Jérôme Honnorat, Romain Sonneville
RATIONALE: Encephalitis due to anti-N-methyl-D-aspartate receptor (NMDAR) antibodies is the leading cause of immune-mediated encephalitis. There is limited data on intensive care unit (ICU) management of these patients. OBJECTIVES: To identify prognostic factors of good neurologic outcome in patients admitted to an ICU with anti-NMDAR encephalitis. METHODS: Observational multicenter study of all consecutive adult patients diagnosed with anti-NMDAR encephalitis, admitted to an ICU between 2008 and 2014...
August 23, 2016: American Journal of Respiratory and Critical Care Medicine
Gemma L McKeon, James G Scott, Donna M Spooner, Alexander E Ryan, Stefan Blum, David Gillis, Daman Langguth, Gail A Robinson
BACKGROUND: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a recently described life-threatening autoimmune disorder associated with a characteristic multi-stage neuropsychiatric syndrome. Although it is known that the majority of patients experience neuropsychological disturbance post-treatment, some aspects of the cognitive profile remain unclear. METHODS: This study sought to investigate patterns of cognitive functioning in a sample of anti-NMDAR encephalitis patients...
September 2016: Journal of the International Neuropsychological Society: JINS
Carlo Selmi, Jobert G Barin, Noel R Rose
In the broad field of autoimmunity and clinical immunology, experimental evidence over the past few years have demonstrated several connections between the immune system and the nervous system, both central and peripheral, leading to the definition of neuroimmunology and of an immune-brain axis. Indeed, the central nervous system as an immune-privileged site, thanks to the blood-brain barrier, is no longer a dogma as the barrier may be altered during chronic inflammation with disruptive changes of endothelial cells and tight junctions, largely mediated by adenosine receptors and the expression of CD39/CD73...
August 18, 2016: Journal of Autoimmunity
Jakob Kreye, Nina K Wenke, Mariya Chayka, Jonas Leubner, Rajagopal Murugan, Nikolaus Maier, Betty Jurek, Lam-Thanh Ly, Doreen Brandl, Benjamin R Rost, Alexander Stumpf, Paulina Schulz, Helena Radbruch, Anja E Hauser, Florence Pache, Andreas Meisel, Lutz Harms, Friedemann Paul, Ulrich Dirnagl, Craig Garner, Dietmar Schmitz, Hedda Wardemann, Harald Prüss
SEE ZEKERIDOU AND LENNON DOI101093/AWW213 FOR A SCIENTIFIC COMMENTARY ON THIS ARTICLE: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a recently discovered autoimmune syndrome associated with psychosis, dyskinesias, and seizures. Little is known about the cerebrospinal fluid autoantibody repertoire. Antibodies against the NR1 subunit of the NMDAR are thought to be pathogenic; however, direct proof is lacking as previous experiments could not distinguish the contribution of further anti-neuronal antibodies...
October 2016: Brain: a Journal of Neurology
Anusha K Yeshokumar, Lisa R Sun, Jessica L Klein, Kristin W Baranano, Carlos A Pardo
This case series demonstrates a novel clinical phenotype of gait disturbance as an initial symptom in children <3 years old with anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis. Anti-NMDAR encephalitis is one of the most common causes of encephalitis in children, more common than any of the viral encephalitides and the second most common autoimmune cause after acute disseminated encephalomyelitis. Anti-NMDAR encephalitis in children often presents with disrupted speech and sleep patterns followed by progression to motor dysfunction, dyskinesias, and seizures...
September 2016: Pediatrics
Charuwan Tantipalakorn, Atiwat Soontornpun, Tip Pongsuvareeyakul, Theera Tongsong
This report is aimed to describe a life-threatening case of anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis secondary to ovarian teratoma with rapid recovery in 1 day after the removal of the tumour. A 23-year-old woman presented with sudden headache, personality changes and seizure. After neurological assessment, limbic or herpes encephalitis was provisionally diagnosed and treated with intravenous immunoglobulin, acyclovir and steroids. The patient had progressive severe neurological symptoms, requiring prolonged intubation and mechanical ventilation...
2016: BMJ Case Reports
E Castillo-Gómez, B Oliveira, D Tapken, S Bertrand, C Klein-Schmidt, H Pan, P Zafeiriou, J Steiner, B Jurek, R Trippe, H Prüss, W-H Zimmermann, D Bertrand, H Ehrenreich, M Hollmann
Autoantibodies of the IgG class against N-methyl-d-aspartate-receptor subunit NR1 (NMDAR1) were first described in anti-NMDAR encephalitis and seen as disease indicators. Recent work on together over 5000 individuals challenged this exclusive view by showing age-dependently up to >20% NMDAR1-autoantibody seroprevalence with comparable immunoglobulin class and titer distribution across health and disease. The key question therefore is to understand the properties of these autoantibodies, also in healthy carriers, in order to assess secondary complications and possible contributions to neuropsychiatric disease...
August 9, 2016: Molecular Psychiatry
G Westman, M Studahl, C Ahlm, B M Eriksson, B Persson, J Rönnelid, S Schliamser, E Aurelius
OBJECTIVES: To investigate the prevalence and temporal development of N-methyl-d-aspartate receptor (NMDAR) autoantibodies in relation to neurocognitive performance in patients with herpes simplex encephalitis (HSE). METHODS: This prospective observational study enrolled a total of 49 HSE patients within a randomized controlled trial of valacyclovir. Cerebrospinal fluid and serum samples were drawn in the initial stage of disease, after 2 to 3 weeks and after 3 months...
August 3, 2016: Clinical Microbiology and Infection
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