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Nellihela Leel, Hemanshoo S Thakkar, David Drake, Nordeen Bouhadiba
Anti-N-methyl D-aspartate receptor (NMDAR) encephalitis is a paraneoplastic, immune-mediated encephalopathy with a known association with ovarian teratomas. We present the first case in the UK of a 7-year-old patient presenting with this condition. Having been previously fit and healthy, the patient presented with an acute onset of cognitive disturbances. The initial suspicion was that of an infective encephalopathy; however, the lumbar puncture results were negative. Electroencephalography reported diffuse background slowing, supporting a diagnosis of encephalopathy...
March 13, 2018: BMJ Case Reports
Hideyuki Matsumoto, Hideji Hashida, Yukitoshi Takahashi
This report describes a rare case presenting with dystonic seizures due to anti-N-methyl-D-aspartate (NMDA) receptor encephalitis. The patient was an 18-year-old woman with repeated right-dominant dystonic seizures even under sedation. Single-photon emission computed tomography (SPECT) showed intense hyperperfusion of the caudate nuclei, putamen, globus pallidus, thalamus, and insula on the left side, suggesting encephalitis. Antibodies against NMDA receptors were detected in the sera and cerebrospinal fluids...
September 2017: Case Reports in Neurology
Natasha Warikoo, Samuel J Brunwasser, Ann Benz, Hong-Jin Shu, Steven M Paul, Michael Lewis, James Doherty, Michael Quick, Laura Piccio, Charles F Zorumski, Gregory S Day, Steven Mennerick
N-methyl-D-aspartate receptors (NMDARs) are ionotropic glutamate receptors important for synaptic plasticity, memory, and neuropsychiatric health. NMDAR hypofunction contributes to multiple disorders, including anti-NMDAR encephalitis (NMDARE), an autoimmune disease of the central nervous system associated with GluN1 antibody-mediated NMDAR internalization. Here we characterize the functional/pharmacological consequences of exposure to cerebrospinal fluid (CSF) from female human NMDARE patients on NMDAR function, and we characterize the effects of intervention with recently described positive allosteric modulators (PAMs) of NMDARs...
February 23, 2018: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
Yoshiyuki Arinuma
PURPOSE OF REVIEW: Neuropsychiatric manifestations are one of the fatal complications in patients with systemic lupus erythematosus (SLE). However, the diagnosis and monitoring of that aspect of SLE is still challenging, as there are no reliable biomarkers linked to central nervous system (CNS) damage. This review emphasizes potential candidate autoantibodies that appear to be associated with development of behavioral and psychiatric manifestations in SLE patients. RECENT FINDINGS: Developments in the pathogenesis in SLE, not surprising for this immune disorder, point to specific, autoantibody toxicity...
February 22, 2018: Current Opinion in Neurology
Hsiuying Wang
Treatments for the anti-NMDA receptor encephalitis usually include steroids, intravenous immunoglobulin, plasma exchange, plasmapheresis, rituximab, cyclophosphamide and tumor resection. We aimed to compare the efficacy of the treatments including intravenous immunoglobulin, plasma exchange, plasmapheresis, rituximab or cyclophosphamide for male anti-NMDA receptor encephalitis patients without tumor and to discuss potential biomarkers for this disease. The Fisher exact test and the contingency table analysis were used to analyze the treatment efficacy for both male and female these patients...
February 21, 2018: Current Medicinal Chemistry
Rui-Juan Lv, Hai-Tao Ren, Hong-Zhi Guan, Tao Cui, Xiao-Qiu Shao
Objective: The purpose of this study is to analyze the seizure semiologic characteristics of patients with autoimmune epilepsy (AE) and describe the investigation characteristics of AE using a larger sample size. Methods: This observational retrospective case series study was conducted from a tertiary epilepsy center between May 2014 and March 2017. Cases of new-onset seizures were selected based on laboratory evidence of autoimmunity. At the same time, typical mesial temporal lobe epilepsy (MTLE) patients with hippocampal sclerosis (HS) were recruited as the control group from the subjects who underwent presurgical evaluation during the same period...
February 2018: Annals of Clinical and Translational Neurology
Xiaotang Cai, Hui Zhou, Yongmei Xie, Dan Yu, Zhiling Wang, Haitao Ren
RATIONALE: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis has been recognized as the most frequent autoimmune encephalitis in children. Several infectious agents have been implicated in anti-NMDA encephalitis. PATIENT CONCERNS: A previously healthy immunocompetent 9-year-old girl first presented with seizures, headaches and vomiting. Cerebrospinal fluid and brain magnetic resonance imaging were normal. After one week onset, the patient gradually developed unexplained personality and behavior changes, accompanied by fever and seizures again...
February 2018: Medicine (Baltimore)
Mukku Shiva Shanker Reddy, Harish Thippeswamy, Sundarnag Ganjekar, Madhu Nagappa, Anita Mahadevan, H R Arvinda, Prabha S Chandra, Arun B Taly
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is increasingly being recognised to be associated with protean neuropsychiatric manifestations. Anti-NMDAR encephalitis is considered to be the most common amongst the autoimmune-mediated encephalitic disorders. It is caused by the autoantibodies against GluN1 subunits of N-methyl-D-aspartate (NMDA) receptor and manifests with prominent psychiatric symptoms, especially during the initial phase of illness. Literature anti-NMDAR encephalitis presenting with postpartum psychosis is scant...
February 7, 2018: Archives of Women's Mental Health
Siyuan Fan, Yan Xu, Haitao Ren, Hongzhi Guan, Feng Feng, Xuehui Gao, Ding Ding, Fang Fang, Guangliang Shan, Tianjia Guan, Yao Zhang, Yi Dai, Ming Yao, Bin Peng, Yicheng Zhu, Liying Cui
BACKGROUND: Myelin oligodendrocyte glycoprotein (MOG)-antibody (ab) disease and AQP4-IgG-positive neuromyelitis optica spectrum disorder (NMOSD) can co-exist with anti-NMDA (N-methyl-D-aspartate) receptor encephalitis (NMDARe). OBJECTIVES: To characterize MOG-ab disease and AQP4-IgG-positive NMOSD during NMDARe. METHODS: We analyzed all the patients with overlapping MOG-ab disease and NMDARe (MNOS) and patients with AQP4-IgG-positive NMOSD and NMDARe (ANOS) in our hospital and compared those data with data from systematically review of previously published reports...
January 30, 2018: Multiple Sclerosis and related Disorders
David R Lynch, Amy Rattelle, Yi Na Dong, Kylie Roslin, Amy J Gleichman, Jessica A Panzer
In slightly more than 10 years, anti-NMDA receptor (NMDAR) encephalitis has changed from a rare paraneoplastic syndrome to the most common cause of nonviral encephalitis. It presents fulminantly with progressive psychosis, seizures, and autonomic dysfunction, leading to death if untreated. However, rapid recognition and treatment can lead to survival and a return to baseline levels of functioning in many patients. While initially associated with ovarian teratomas, it is now associated with other tumors and can reflect a postviral event...
2018: Advances in Pharmacology
José Fidel Baizabal-Carvallo, Joseph Jankovic
Movement disorders (MDs) are common in patients with autoimmune disorders affecting the central and peripheral nervous system. They may be observed in autoimmune disorders triggered by an infectious agent, such as streptococcus in Sydenham's chorea, or in basal ganglia encephalitis with antibodies against the dopamine-D2 receptors. In these patients chorea or dystonia are usually the most prominent hyperkinetic MDs. MDs are also observed in patients with diffuse or limbic encephalitis with antibodies directed against neuronal cell-surface antigens...
February 15, 2018: Journal of the Neurological Sciences
Adeel Arshad, Shahzaib Nabi, Muhammad Zahid
No abstract text is available yet for this article.
February 2018: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
Nedia Ben Achour, Thouraya Ben Younes, Ibtihel Rebai, Melika Ben Ahmed, Ichraf Kraoua, Ilhem Ben Youssef-Turki
INTRODUCTION: Anti-glutamic acid decarboxylase (anti-GAD65) antibodies are a rare cause of autoimmune encephalitis. This entity is mainly recognized in adults and very few cases were reported in children. We report on a paediatric case of anti-GAD encephalitis with severe presentation and uncontrollable dysautonomia. CASE STUDY: A 9-year-old girl was referred to our department for refractory seizures and behavioral disturbances. Brain magnetic resonance imaging (MRI) was normal...
January 12, 2018: European Journal of Paediatric Neurology: EJPN
Chia-Hsiang Chen, Yu-Syuan Chang
The presence of anti-NMDA receptor autoantibody can cause various neuropsychiatric symptoms in the affected patients, termed anti-NMDA receptor autoimmune encephalitis. Detection of the specific autoantibody against the NMDA receptor in the blood or cerebrospinal fluid (CSF) is essential for the accurate diagnosis of this condition. The NMDA receptor is an ion channel protein complex that contains four subunits, including two mandatory NMDA receptor subunit 1 (NR1) and one or two NMDA receptor subunit 2A (NR2A), NMDA receptor subunit 2B (NR2B), NMDA receptor subunit 2C (NR2C), or NMDA receptor subunit 2D (NR2D)...
January 9, 2018: Journal of Visualized Experiments: JoVE
Kamlesh Kumari, Neeru Sahni, Vimla Kumari, Vikas Saini
Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is an immune-mediated disease commonly associated with ovarian teratoma. Anti-NMDA-receptor autoantibodies disrupt NMDA function leading to the development of psychosis, seizures and autonomic dysfunction. The treatment includes underlying tumour resection and immunosuppression. Slow recovery and unpredictable clinical course makes intensive care management of these patients challenging. We report the management of two young female patients with anti-NMDA-receptor encephalitis associated with ovarian teratoma...
December 2017: Turkish Journal of Anaesthesiology and Reanimation
Atsushi Kaneko, Juntaro Kaneko, Naomi Tominaga, Naomi Kanazawa, Kasumi Hattori, Yoshikazu Ugawa, Arata Moriya, Daisuke Kuzume, Daisuke Ishima, Eiji Kitamura, Kazutoshi Nishiyama, Takahiro Iizuka
OBJECTIVES: To report pitfalls in the clinical diagnosis of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. METHODS: We retrospectively reviewed the clinical information of 221 patients with clinically suspected autoimmune neurological disorders who underwent testing for autoantibodies against neuronal cell-surface antigens between January 1, 2007 and September 10, 2017. Forty-one patients met the diagnostic criteria for probable anti-NMDAR encephalitis (probable criteria), but one was excluded because neither serum nor CSF was examined at the active stage...
January 22, 2018: Journal of Neurology
Takanori Omae, Yoshiaki Saito, Hirokazu Tsuchie, Koyo Ohno, Yoshihiro Maegaki, Hiroshi Sakuma
A 3-year-old girl suffered from anti-N-methyl-d-aspartate (anti-NMDA) receptor encephalitis after resolution of herpes simplex virus encephalitis (HSE). Methylprednisolone pulse and immunoglobulin therapies showed little effect, but the patient completely recovered after six courses of monthly cyclophosphamide pulse therapy and successive maintenance on mycophenolate mofetil for one year. Anti-NMDA receptor antibody in the cerebrospinal fluid (CSF) was minimally detected during the prodromal febrile period and then was seen to be markedly elevated at the onset of second encephalopathy phase...
December 22, 2017: Brain & Development
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
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
Eric Lancaster
PURPOSE OF REVIEW: Paraneoplastic neurologic syndromes target specific areas of the nervous system with pathogenic autoantibodies or T-cell responses. Each syndrome conveys a risk of particular tumors. Expanded paraneoplastic antibody testing has led to improved diagnosis but created challenges involving appropriate interpretation of test results. RECENT FINDINGS: Peripheral nervous system paraneoplastic disorders such as myasthenia gravis and Lambert-Eaton myasthenic syndrome involve pathogenic autoantibodies...
December 2017: Continuum: Lifelong Learning in Neurology
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