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NMDAR encephalitis

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https://www.readbyqxmd.com/read/29332416/-autoimmune-encephalitis-possibilities-in-the-laboratory-investigation
#1
Katalin Böröcz, Zsófia Hayden, Viktória Mészáros, Zsuzsanna Csizmadia, Kornélia Farkas, Zoltán Kellermayer, Péter Balogh, Ferenc Nagy, Tímea Berki
INTRODUCTION: The role of autoimmune responses against central nervous system (CNS) antigens in encephalitis presenting with non-classified neurologic or psychiatric symptoms has been appreciated in the past decade. Paraneoplastic limbic encephalitis has a poor prognosis and is most commonly associated with lung, ovarium, and testicular neoplasms, leading to immune reactions against intracellular antigens (anti-Hu/ANNA1, anti-Ri/ANNA2, anti-CV2/CRMP5 and anti-Ma2/Ta). In contrast, the recently described autoimmune encephalitis subtypes present with a broad spectrum of symptoms, respond to autoimmune therapies well and usually associate with autoantibodies against neuronal cell surface receptors (NMDAR, GABABR, AMPAR) or synaptic proteins (LGI1, CASPR2)...
January 2018: Orvosi Hetilap
https://www.readbyqxmd.com/read/29329729/elevated-neuron-specific-enolase-and-s100-calcium-binding-protein-b-concentrations-in-cerebrospinal-fluid-of-patients-with-anti-n-methyl-d-aspartate-receptor-encephalitis
#2
Baozhu Liu, Zuoshan Xie, Guanghui Liu, Yong Gu, Suyue Pan, Honghao Wang
BACKGROUND: Anti‑N‑methyl‑d‑aspartate receptor (NMDAR) encephalitis is a relatively common autoimmune neurological disease of the central nervous system (CNS). Neuron-specific enolase (NSE) and S100 calcium-binding protein B (S100B) are structural proteins of the central nervous system (CNS). In patients with CNS injury accompanied by nervous tissue and cellular damage, these structural proteins are released from cells; their extracellular concentrations, including those in cerebrospinal fluid (CSF) and blood, subsequently increase...
January 9, 2018: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/29327271/-paraneoplastic-neurological-syndromes-a-current-summary
#3
REVIEW
D De Simoni, R Höftberger
BACKGROUND: Paraneoplastic neurological syndromes (PNNS) are remote effects of a tumor and mediated by an altered immune reaction. In the last ten years, the spectrum of PNNS has changed profoundly with the discovery of a new category of neurological diseases that are associated with antibodies against surface or synaptic antigens. In contrast to classical PNNS, patients with surface receptor autoimmunity are often highly responsive to immunotherapy. OBJECTIVES: This article provides an update on the most relevant PNNS, focusing on specific syndromes associated with antibodies against classical onconeuronal antigens as well as surface and synaptic proteins...
January 11, 2018: Der Internist
https://www.readbyqxmd.com/read/29315460/serum-cystatin-c-and-anti-n-methyl-d-aspartate-receptor-encephalitis
#4
Y Shu, Y Chang, H Wu, J Li, B Cao, X Sun, J Wang, L Peng, X Hu, X Yu, W Qiu
BACKGROUND: Cystatin C (CysC) is associated with many neurodegenerative disorders and autoimmune diseases, but its relationship with anti-N-Methyl-D-aspartate receptor (anti-NMDAR) encephalitis is unknown. METHODS: Serum levels of CysC were determined in 66 patients with anti-NMDAR encephalitis and 115 healthy controls. Of the 66 patients, 30 had a follow-up evaluation at 3 months after admission. Association of CysC with anti-NMDAR encephalitis and its clinical parameters were evaluated in the patients...
January 7, 2018: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/29312777/anti-n-methyl-d-aspartate-receptor-encephalitis-associated-with-mediastinal-teratoma-a-rare-case-report-and-literature-review
#5
Chuan Li, Chengwu Liu, Feng Lin, Lunxu Liu
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a recently identified neuroautoimmune disorder characterized by antibodies against the heteromeric NR1-NR2 receptor complex. To our best knowledge, anti-NMDAR encephalitis combined with mediastinal teratoma is extremely rare with only three cases been reported in literature worldwide. In this article, we present an 18 years old female diagnosed as anti-NMDAR encephalitis caused by a large mature mediastinal teratoma whose initial manifestations were acute headache and seizures...
December 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29310908/an-infant-born-to-a-mother-with-anti-n-methyl-d-aspartate-receptor-encephalitis
#6
Nitish Chourasia, Michael W Watkins, Jeremy E Lankford, Joseph S Kass, Ankur Kamdar
BACKGROUND: Anti-N-Methyl-D-Aspartate receptor (NMDAR) encephalitis is an autoimmune disorder that often affects women of childbearing age, and maternal-fetal transfer of anti-NMDAR antibodies during pregnancy has been documented in both symptomatic and asymptomatic women. The effects of these antibodies on the fetus, however, are incompletely understood. PATIENT DESCRIPTION: This term infant exhibited depressed respiratory effort, poor feeding, and abnormal movements after birth...
November 22, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/29306850/allergy-in-patients-with-anti-n-methyl-d-aspartate-receptor-encephalitis
#7
Xin-Yue Jiang, Le Zhang, Xian Jiang, Ammar Taha Abdullah Abdulaziz, Yun-Hui Wang, Jin-Mei Li, Dong Zhou
BACKGROUND AND OBJECTIVE: Allergy is a potential outcome of dysregulated immune system. Previous studies have shown the association of allergy and autoimmune diseases, however, there is few study to investigate the relationship between allergy and anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis. Thus, we investigate the rate of allergy in patients with anti-NMDAR encephalitis and analyze the risk factors. METHOD: The rate of allergy was investigated in patients with anti-NMDAR encephalitis and was compared with patients with virus encephalitis...
January 4, 2018: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/29306404/cerebrospinal-fluid-pentraxin-3-and-cd40-ligand-in-anti-n-menthyl-d-aspartate-receptor-encephalitis
#8
Baozhu Liu, Peiying Ai, Dong Zheng, Ying Jiang, Xiaojia Liu, Suyue Pan, Honghao Wang
Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder of the central nervous system whose pathogenesis involves interleukin (IL)-6 and IL-17A. We examined the correlations between CSF concentrations of the acute-phase protein pentraxin 3 (PTX3), the chronic inflammatory mediator CD40L, IL-6, and IL-17A in anti-NMDAR encephalitis, and the impact on clinical outcome. PTX3, CD40L, IL-6, and IL-17A were significantly higher in the CSF of patients with anti-NMDAR encephalitis than in controls...
February 15, 2018: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/29289523/principles-and-approaches-to-the-treatment-of-immune-mediated-movement-disorders
#9
REVIEW
Shekeeb S Mohammad, Russell C Dale
Immune mediated movement disorders include movement disorders in the context of autoimmune encephalitis such as anti-NMDAR encephalitis, post-infectious autoimmune movement disorders such as Sydenham chorea, paraneoplastic autoimmune movement disorders such as opsoclonus myoclonus ataxia syndrome, and infection triggered conditions such as paediatric acute neuropsychiatric syndrome. This review focuses on the approach to treatment of immune mediated movement disorders, which requires an understanding of the immunopathogenesis, whether the disease is destructive or 'altering', and the natural history of disease...
December 19, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29282340/-from-involuntary-movements-to-movement-disorders
#10
Toshio Fukutake
Apart from the term 'involuntary movements', the term 'movement disorders' encompasses not only classical hyperkinesias but also hypokinesias including catalepsy or apraxia. It enables us to understand abnormal movements by their phenomena instead of by their localization. To advance the grasping of movement disorders, we discuss the clinical and pathophysiological features of abnormal movements in catatonia/catalepsy, anti-NMDAR encephalitis, paroxysmal dyskinesias, stiff person/leg syndrome, corticobasal degeneration/syndrome, and hysteria...
December 2017: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/29258969/a-case-of-anti-nmda-receptor-encephalitis-revealed-by-insular-epilepsy
#11
Charlotte De Maeseneire, Riem El Tahry, Susana Ferrao Santos
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder of the central nervous system that typically manifests predominantly as a psychiatric disorder. However, other manifestations such as epileptic seizures, abnormal movements, and memory or language complications are not unusual. Here, we report the case of a young man who presented with a new-onset epilepsy, with ictal semiology suggestive of insular involvement; this hypothesis was supported by a PET-CT study. Anti-NMDAR antibodies were found in the CSF, confirming the diagnosis of anti-NMDAR encephalitis...
December 20, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29249055/brain-magnetic-resonance-imaging-findings-of-anti-n-methyl-d-aspartate-receptor-encephalitis-a-cohort-follow-up-study-in-chinese-patients
#12
Rui Wang, Xiao-Hui Lai, Xu Liu, Yu-Jia Li, Chu Chen, Chen Li, Xiao-Sa Chi, Dong Zhou, Zhen Hong
The aim of this report was to assess routine clinical brain magnetic resonance imaging (MRI) and its relation to clinical characteristics and disease prognosis. Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis patients were consecutively recruited from West China Hospital between October 1, 2011 and April 1, 2016. Brain MRI findings of 106 patients were analysed, and outcomes were assessed at 4, 8, and 12 months after discharge from the hospital using the modified Rankin scale (mRS). An MRI of the brain was normal in 52/106 (49...
December 16, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/29245365/teratoma-associated-anti-nmdar-encephalitis-two-cases-report-and-literature-review
#13
Zhigang Liang, Shaowan Yang, Xuwen Sun, Bing Li, Wei Li, Zhuli Liu, Guoping Yu
OBJECTIVE: This study aimed to discuss the pathogenesis, clinical manifestation, diagnosis, and treatment of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. CASE REPORT: The diagnosis and treatment of 2 cases with teratoma-associated anti-NMDAR encephalitis were summarized and the clinical data of patients reported by domestic and international studies were reviewed in this study. The 2 cases were both adolescent females who showed mental abnormalities as their main clinical manifestation...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29226117/pediatric-anti-n-methyl-d-aspartate-receptor-encephalitis-a-review-with-pooled-analysis-and-critical-care-emphasis
#14
REVIEW
Kenneth E Remy, Jason W Custer, Joshua Cappell, Cortney B Foster, Nan A Garber, L Kyle Walker, Liliana Simon, Dayanand Bagdure
Purpose: Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is being recognized with increasing frequency among children. Given the paucity of evidence to guide the critical care management of these complex patients, we provide a comprehensive review of the literature with pooled analysis of published case reports and case series. Methods: We performed a comprehensive literature search using PubMed, Scopus, EMBASE, and Web of Science for relevant published studies...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29197887/important-differential-in-a-patient-presenting-with-neuropsychiatric-symptoms-anti-n-methyl-d-aspartate-receptor-encephalitis
#15
J M Low
Anti-N-Methyl-D-Aspartate receptor (NMDAR) encephalitis is an immune mediated condition, which remains relatively unknown in Malaysia outside tertiary hospitals with neurology unit. It is often misdiagnosed as a psychiatric illness before definitive treatment is instituted. We report here an 18-year-old man who initially presented to the psychiatry unit before he was subsequently diagnosed as having anti-NMDAR encephalitis. To our knowledge, this is the first reported case of anti-NMDAR encephalitis in the east coast of Peninsular Malaysia...
October 2017: Medical Journal of Malaysia
https://www.readbyqxmd.com/read/29186977/ictal-rhythmic-alpha-sinusoidal-waves-in-3-cases-of-anti-nmdar-encephalitis
#16
Ailiang Miao, Xiaoshan Wang
Previous studies have shown that extreme delta brush and high beta/delta power ratio on electroencephalogram are suggestive of anti- N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis. Here we report 3 anti-NMDAR encephalitis patients with ictal rhythmic alpha sinusoidal waves in temporal regions, which suggested electrographic seizures in anti-NMDAR encephalitis and indicated potential for seizure occurrence in the future. Rhythmic alpha sinusoidal waves may be an electrographic feature and helpful in distinguishing anti-NMDAR encephalitis...
November 1, 2017: Clinical EEG and Neuroscience: Official Journal of the EEG and Clinical Neuroscience Society (ENCS)
https://www.readbyqxmd.com/read/29183618/encephalitis-associated-with-antibodies-against-the-nmda-receptor
#17
REVIEW
Mar Guasp, Josep Dalmau
The encephalitis associated with antibodies against the N-methyl-D-aspartate receptor (NMDAR) is characterized by the presence of antibodies against the GluN1 subunit of this receptor, resulting in symptoms that are similar to those observed in models of genetic or pharmacologic reduction of NMDARs. Patients are usually young adults, predominantly women, and children who develop, in a sequential manner, rapidly progressive symptoms including psychosis, abnormal movements, autonomic dysfunction, and coma. Epileptic seizures are variable and can occur throughout the course of the disease...
November 25, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/29161898/delta-brush-pattern-is-not-unique-to-nmdar-encephalitis-evaluation-of-two-independent-long-term-eeg-cohorts
#18
Betul Baykan, Ozlem Gungor Tuncer, Ebru Nur Vanli-Yavuz, Leyla Baysal Kirac, Gokcen Gundogdu, Nerses Bebek, Candan Gurses, Ebru Altindag, Erdem Tuzun
PURPOSE: Although its specificity has not previously been investigated in other cohorts, delta brush pattern (DBP) is increasingly reported in the EEGs of patients with anti- N-methyl-d-aspartate receptor (NMDAR) encephalitis. METHODS: We aimed to investigate the DBP in the EEGs of 2 cohorts; patients with change in consciousness for various causes monitored in the intensive care unit (ICU) (n = 106) and patients with mesial temporal lobe epilepsy (MTLE) with or without antineuronal antibodies (n = 76)...
February 1, 2017: Clinical EEG and Neuroscience: Official Journal of the EEG and Clinical Neuroscience Society (ENCS)
https://www.readbyqxmd.com/read/29159196/botulinum-toxin-treatment-for-hypersalivation-in-anti-nmda-receptor-encephalitis
#19
Jin-Sun Jun, Han Gil Seo, Soon-Tae Lee, Kon Chu, Sang Kun Lee
Hypersalivation is one of the intractable symptoms of anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis. While anticholinergic medications partially improve the hypersalivation, they can aggravate the autonomic dysfunctions associated with anti-NMDAR encephalitis. Thus, we investigated the efficacy and safety of botulinum toxin type A injection on hypersalivation refractory to anticholinergics in six patients with anti-NMDAR encephalitis. Hypersalivation was well-controlled without remarkable adverse reaction over 16 weeks after botulinum toxin type A, although two patients were reinjected at 12 weeks due to reaggravation of hypersalivation...
November 2017: Annals of Clinical and Translational Neurology
https://www.readbyqxmd.com/read/29159189/nmdar-encephalitis-passive-transfer-from-man-to-mouse-by-a-recombinant-antibody
#20
Manish Malviya, Sumanta Barman, Kristin S Golombeck, Jesús Planagumà, Francesco Mannara, Nathalie Strutz-Seebohm, Claudia Wrzos, Fatih Demir, Christine Baksmeier, Julia Steckel, Kim Kristin Falk, Catharina C Gross, Stjepana Kovac, Kathrin Bönte, Andreas Johnen, Klaus-Peter Wandinger, Elena Martín-García, Albert J Becker, Christian E Elger, Nikolaj Klöcker, Heinz Wiendl, Sven G Meuth, Hans-Peter Hartung, Guiscard Seebohm, Frank Leypoldt, Rafael Maldonado, Christine Stadelmann, Josep Dalmau, Nico Melzer, Norbert Goebels
Objective: Autoimmune encephalitis is most frequently associated with anti-NMDAR autoantibodies. Their pathogenic relevance has been suggested by passive transfer of patients' cerebrospinal fluid (CSF) in mice in vivo. We aimed to analyze the intrathecal plasma cell repertoire, identify autoantibody-producing clones, and characterize their antibody signatures in recombinant form. Methods: Patients with recent onset typical anti-NMDAR encephalitis were subjected to flow cytometry analysis of the peripheral and intrathecal immune response before, during, and after immunotherapy...
November 2017: Annals of Clinical and Translational Neurology
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