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Anti-NMDAR encephalitis

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https://www.readbyqxmd.com/read/29774053/postherpes-simplex-encephalitis-a-case-series-of-viral-triggered-autoimmunity-synaptic-autoantibodies-and-response-to-therapy
#1
Harry Alexopoulos, Sofia Akrivou, Sotiria Mastroyanni, Maria Antonopoulou, Argirios Dinopoulos, Melpo Giorgi, Kostas Konstantinou, Evangelos Kouremenos, Maria Lariou, Dimitrios Naoumis, Efterpi Pavlidou, Evaggelos Pavlou, Konstantinos Voudris, Panayotis Vlachoyiannopoulos, Marinos C Dalakas
Background: Recent evidence suggests that patients with herpes simplex virus (HSV) encephalitis may relapse because of autoimmunity against the N-methyl-D-aspartate receptor (NMDAR). We present a case series of post-HSV relapsing encephalopathy associated with antibodies to central nervous system (CNS) synaptic antigens. Patient/Methods: Sera and cerebrospinal fluid (CSF) from five patients with HSV encephalitis who relapsed after antiviral therapy were tested for anti-NMDAR, gamma-aminobutyric acid b receptor (GABAbR), α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), Leucine-rich, glioma inactivated 1 (LGI1), anti -contactin-associated protein-like 2 (CASPR2) and dipeptidyl-peptidase-like protein-6 (DDPX) antibodies using cell-based assays...
2018: Therapeutic Advances in Neurological Disorders
https://www.readbyqxmd.com/read/29735495/anaesthetic-management-of-a-patient-with-a-unique-combination-of-anti-n-methyl-d-aspartate-receptor-encephalitis-and-stiff-person-syndrome
#2
Mohammad Hadi Gharedaghi, Arjang Khorasani, Nebojsa Nick Knezevic, Farzad Ebrahimi
Stiff-person syndrome (SPS) and anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis are rare paraneoplastic syndromes caused by antibodies that target the central nervous system. Here, we describe a 26-year-old woman who presented with psychosis, amnesia, rigidity and fever. After extensive diagnostic and laboratory workup, she was diagnosed with an ovarian teratoma which was causing the symptoms of anti-NMDAR encephalitis and SPS. The patient was successfully treated with laparoscopic removal of the ovarian tumour under general anaesthesia...
May 7, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29734104/late-onset-anti-n-methyl-d-aspartate-receptor-encephalitis-in-china
#3
Le Zhang, Xu Liu, Xin-Yue Jiang, Yun-Hui Wang, Jin-Mei Li, Dong Zhou
PURPOSE: This study aimed to summarize the clinical characteristics and outcome of late-onset anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis in China. METHOD: All cases of people with a definitive diagnosis of anti-NMDAR encephalitis in West China Hospital between June 2012 and April 2017 were retrospectively reviewed. The demographics, clinical characteristics, and outcome of those patients (age≥45years old) were summarized. Comparisons were conducted between older (≥45years old) and younger (18-44years old) adults...
May 4, 2018: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/29703768/long-term-neuropsychological-outcome-following-pediatric-anti-nmdar-encephalitis
#4
Marienke A A M de Bruijn, Femke K Aarsen, Marielle P van Oosterhout, Marieke M van der Knoop, Coriene E Catsman-Berrevoets, Marco W J Schreurs, Danielle E M Bastiaansen, Peter A E Sillevis Smitt, Rinze F Neuteboom, Maarten J Titulaer
OBJECTIVE: To provide detailed long-term outcome data of children and adolescents following pediatric anti- N -methyl-d-aspartate receptor (anti-NMDAR) encephalitis, to identify neuropsychological impairments, and to evaluate the influence of these factors on quality of life (QoL). METHODS: All Dutch children diagnosed with anti-NMDAR encephalitis were identified. Patients currently aged 4 years or older were included in the follow-up study, consisting of a visit to our clinic for a detailed interview and a standardized neuropsychological assessment...
April 27, 2018: Neurology
https://www.readbyqxmd.com/read/29698873/clinical-significance-of-igm-and-iga-class-anti-nmdar-antibodies-in-herpes-simplex-encephalitis
#5
Gabriel Westman, Azita Sohrabian, Elisabeth Aurelius, Clas Ahlm, Silvia Schliamser, Fredrik Sund, Marie Studahl, Johan Rönnelid
BACKGROUND: Herpes simplex encephalitis (HSE) is a devastating disease, often leaving patients with severe disabilities. It has been shown that IgG anti-N-methyl-d-aspartate receptor (NMDAR) antibodies appear in approximately 25% of HSE patients and could be associated with impaired recovery of cognitive performance. OBJECTIVES: To characterize the prevalence of IgM and IgA anti-NMDAR antibodies in HSE patients, in relation to subsequent development of IgG anti-NMDAR and correlation to cognitive performance...
April 10, 2018: Journal of Clinical Virology: the Official Publication of the Pan American Society for Clinical Virology
https://www.readbyqxmd.com/read/29661538/an-unusual-case-of-anti-mog-cns-demyelination-with-concomitant-mild-anti-nmdar-encephalitis
#6
Jiajia Zhou, Winwen Tan, Suyin Elaine Tan, Jing Hu, Zhongqin Chen, Kang Wang
We report the case of a patient who presented with progressive unsteadiness and narcoleptic attacks followed by behavioral change and psychosis, without visual disturbances or seizures. MRI revealed multiple areas of fluid attenuation inversion recovery (FLAIR) high-intensity lesions involving the cerebellum, brainstem, thalamus and third ventricular peri-ependymal region consistent with demyelination. Both the serum myelin oligodendrocyte glycoprotein-antibodies (MOG-Abs) and cerebral spinal fluid (CSF) anti-N-methyl-d-as-partate receptor (NMDAR) antibodies were positive using transfected cell based assays...
April 6, 2018: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/29651625/clinical-characteristics-and-prognosis-of-severe-anti-n-methyl-d-aspartate-receptor-encephalitis-patients
#7
Yan Zhang, Gang Liu, Mengdi Jiang, Weibi Chen, Yanbo He, Yingying Su
BACKGROUND AND PURPOSE: Data concerning the characteristics and duration of the critical manifestations, treatment response, and long-term outcomes of severe anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis patients compared to those of non-severe patients are limited. This observational study was performed to explore the clinical characteristics and long-term outcomes of severe anti-NMDAR encephalitis patients. METHODS: According to their characteristics on admission to the neurology intensive care unit, patients with anti-NMDAR encephalitis were divided into a severe group and a non-severe group...
April 12, 2018: Neurocritical Care
https://www.readbyqxmd.com/read/29628174/early-identification-of-anti-nmda-receptor-encephalitis-presenting-cerebral-lesions-in-unconventional-locations-on-magnetic-resonance-imaging
#8
Weihe Zhang, Lei Cui, Wei Wang, Yujuan Jiao, Yeqiong Zhang, Jinsong Jiao
To facilitate the diagnosis of anti-NMDAR encephalitis presenting with brain lesions in unconventional locations (BLUL) on MRI, we retrospectively analyzed forty-five Chinese patients. Eighteen (40.0%) of their MRI initially exhibited one or more BLUL. These locations predominantly included cerebral gray matter (cortex, basal ganglia and thalamus), as well as white matter and brainstem. Due to these BLUL, thirteen (72.2%) patients were originally misdiagnosed with other diseases and developed poor clinical and imaging outcomes...
March 29, 2018: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/29617708/a-systematic-review-of-the-neuropsychological-sequelae-of-people-diagnosed-with-anti-n-methyl-d-aspartate-receptor-encephalitis-in-the-acute-and-chronic-phases
#9
Della C M Nicolle, Jennifer L Moses
Objective: A systematic review was conducted to investigate the emerging cognitive profile for people diagnosed with anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis. Method: Ten papers met the review criteria including five neuropsychological case studies and five case series; three of the 10 studies used matched controls. The cognitive functioning of 54 participants (46 female: eight male) was studied. Paper quality assessment was undertaken and outcomes summarized...
March 31, 2018: Archives of Clinical Neuropsychology: the Official Journal of the National Academy of Neuropsychologists
https://www.readbyqxmd.com/read/29607915/-a-case-of-limbic-encephalitis-associated-with-sj%C3%A3-gren-s-syndrome-mimicking-anti-n-methyl-d-aspartate-receptor-encephalitis
#10
Kenji Yoshimura, Rie Kanki, Satoshi Nakano
A 25-year-old woman in her 37 weeks and 5 days pregnant presented with abnormal behavior and memory impairment following a high fever. Her manifestations were diagnosed as limbic encephalitis, and she delivered a baby by Cesarean section. In the operation, bilateral ovarian tumors were found and resected, though they were revealed as non-teratoma afterward. After operation, she became agitated, and started to present oral dyskinesia, intractable systemic myoclonus, central hypoventilation, and autonomic manifestations such as labile blood pressure, but her symptoms responded well to immunotherapy...
March 31, 2018: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/29599011/anti-n-methyl-d-aspartate-receptor-encephalitis-in-children-incidence-and-experience-in-hong-kong
#11
Alvin Chi-Chung Ho, Sophelia Hoi-Shan Chan, Eric Chan, Sheila Suet-Na Wong, Sharon Tsui-Hang Fung, Sharon Wan-Wah Cherk, Eva Lai-Wah Fung, Kam-Hung Ma, Kwing-Wan Tsui, Eric Kin-Cheong Yau, Virginia Chun-Nei Wong
AIM: The study aims to analyze the incidence, clinical features, investigation findings and treatment outcomes of anti-N-methyl-d-aspartate receptor encephalitis in children from Hong Kong. METHOD: A retrospective study was carried out on paediatric patients diagnosed with anti-NMDAR encephalitis in Hong Kong from January 2009 to December 2015. RESULTS: Fifteen patients (67% female, 93% Chinese) were identified over seven years and the estimated incidence in Hong Kong was 2...
March 26, 2018: Brain & Development
https://www.readbyqxmd.com/read/29572931/genetic-predisposition-in-anti-lgi1-and-anti-nmda-receptor-encephalitis
#12
Stefanie H Mueller, Anna Färber, Harald Prüss, Nico Melzer, Kristin S Golombeck, Tania Kümpfel, Franziska Thaler, Martin Elisak, Jan Lewerenz, Max Kaufmann, Kurt-Wolfram Sühs, Marius Ringelstein, Christoph Kellinghaus, Christian G Bien, Andrea Kraft, Uwe K Zettl, Sven Ehrlich, Robert Handreka, Kevin Rostásy, Florian Then Bergh, Jürgen H Faiss, Wolfgang Lieb, Andre Franke, Gregor Kuhlenbäumer, Klaus-Peter Wandinger, Frank Leypoldt
We performed a genome-wide-association study in 1,194 controls and 150 patients with anti-N-methyl-D-aspartate receptor (anti-NMDAR, n=96) or anti-leucine-rich glioma-inactivated1 (anti-LGI1, n=54) autoimmune encephalitis. Anti-LGI1 encephalitis was highly associated with 27 SNPs in the HLA-II region (leading SNP rs2858870 P=1.22x10-17 , OR=13.66 [7.50-24.87]). Potential associations, below genome-wide significance, were found with rs72961463 close to the doublecortin-like kinase 2 gene (DCLK2) and rs62110161 in a cluster of zink-finger genes...
March 23, 2018: Annals of Neurology
https://www.readbyqxmd.com/read/29561731/paraneoplastic-movement-disorders
#13
Karolina Popławska-Domaszewicz, Jolanta Florczak-Wyspiańska, Wojciech Kozubski, Sławomir Michalak
Paraneoplastic movement disorders are rare, autoimmune-mediated, nonmetastatic complications of malignant neoplasms. Common paraneoplastic movement disorders include paraneoplastic chorea, dystonia, cerebellar degeneration, different types of encephalitis, opsoclonus-myoclonus syndrome, stiff person syndrome, and neuromyotonia. Syndromes usually develop before tumor diagnosis, have subacute onset, and are associated with serum or cerebrospinal fluid antibodies. Two types of antibodies can be distinguished: antibodies against nuclear and cytoplasmic neuronal antigens (anti-Hu, anti-Ri, anti-Yo, anti-Ma, anti-CV2/CRMP5, anti-Gephrin, and anti-GABATRAP) and antibodies recently identified against cell surface and synaptic proteins (anti-NMDAR, anti-LGI1, and anti-Caspr2)...
March 21, 2018: Reviews in the Neurosciences
https://www.readbyqxmd.com/read/29549218/clinical-and-pathogenic-significance-of-igg-iga-and-igm-antibodies-against-the-nmda-receptor
#14
Makoto Hara, Eugenia Martinez-Hernandez, Helena Ariño, Thais Armangué, Marianna Spatola, Mar Petit-Pedrol, Albert Saiz, Myrna R Rosenfeld, Francesc Graus, Josep Dalmau
OBJECTIVE: To determine the frequency and clinical relevance of immunoglobulin (Ig)G, IgA, and IgM N -methyl-d-aspartate receptor (NMDAR) antibodies in several diseases, and whether the IgG antibodies occur in disorders other than anti-NMDAR encephalitis. METHODS: Evaluation of IgG, IgA, and IgM NMDAR antibodies in serum of 300 patients with anti-NMDAR encephalitis, stroke, dementia, schizophrenia, or seronegative autoimmune encephalitis. Antibodies and their effect on cultured neurons were examined with cell-based assays and brain and live neuronal immunostaining...
April 17, 2018: Neurology
https://www.readbyqxmd.com/read/29535092/ovarian-teratoma-associated-with-anti-nmda-n-methyl-d-aspartate-receptor-encephalitis
#15
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
https://www.readbyqxmd.com/read/29523295/molecular-hypotheses-to-explain-the-shared-pathways-and-underlying-pathobiological-causes-in-catatonia-and-in-catatonic-presentations-in-neuropsychiatric-disorders
#16
E M Peter-Ross
The pathobiological causes, the shared cellular and molecular pathways in catatonia and in catatonic presentation in neuropsychiatric disorders are yet to be determined. The hypotheses in this paper have been deduced from the latest scientific research findings and clinical observations of patients with genetic disorders, behavioral phenotypes and other family members suffering mental disorders. The first hypothesis postulates that catatonia and the heterogeneity of catatonic signs and symptoms involve nucleolar dysfunction arising from abnormalities of the brain-specific, non-coding micro-RNA, SNORD115 genes (either duplications or deletions) which result in pathobiological dysfunction of various combinations in the downstream pathways (possibly along with other genes in these shared pathways)...
April 2018: Medical Hypotheses
https://www.readbyqxmd.com/read/29520252/management-of-refractory-orofacial-dyskinesia-caused-by-anti-n-methyl-d-aspartate-receptor-encephalitis-using-botulinum-toxin
#17
Feixia Zheng, Xiuyun Ye, Xulai Shi, Neha Devi Poonit, Zhongdong Lin
The use of botulinum neurotoxin serotype A (BoNT-A) injections for the treatment of orofacial dyskinesia secondary to anti- N -methyl-d-aspartate receptor (NMDAR) encephalitis is rarely reported. Here, we report a case of an urgent, successful management of severe orofacial dyskinesia in an 8-year-old girl with anti-NMDAR encephalitis using BoNT-A injection. The patient presented with de novo unilateral paroxysmal movement disorder progressing to generalized dystonia and repetitive orofacial dyskinesia. Diagnosis was confirmed by the presence of NMDAR antibodies in serum and cerebrospinal fluid...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29484082/arterial-spin-labeling-perfusion-imaging-demonstrates-cerebral-hyperperfusion-in-anti-nmdar-encephalitis
#18
Jeffrey R Sachs, Michael E Zapadka, Gautam S Popli, Jonathan H Burdette
Anti-N-methyl-d-aspartate receptor encephalitis is an increasingly recognized autoimmune disorder that results in substantial morbidity, prolonged hospital stays, and even death. The diagnosis is often delayed or unrecognized entirely as a result of absent or only subtle initial magnetic resonance imaging findings and a nonspecific clinical syndrome. The discovery of early imaging findings in this disease may help clinicians to more aggressively treat this autoimmune encephalitis and to potentially lessen morbidity and mortality...
December 2017: Radiology case reports
https://www.readbyqxmd.com/read/29476014/positive-allosteric-modulation-as-a-potential-therapeutic-strategy-in-anti-nmda-receptor-encephalitis
#19
Natasha Warikoo, Samuel J Brunwasser, Ann Benz, Hong-Jin Shu, Steven M Paul, Michael Lewis, James Doherty, Michael Quirk, 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 CNS associated with GluN1 antibody-mediated NMDAR internalization. Here we characterize the functional/pharmacological consequences of exposure to 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...
March 28, 2018: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/29474316/autoantibodies-against-n-methyl-d-aspartate-receptor-1-in-health-and-disease
#20
Hannelore Ehrenreich
PURPOSE OF REVIEW: Humoral autoimmunity has gained highest interest in neurology and psychiatry. Despite numerous recent articles on this hot topic, however, the biological significance of natural autoantibodies (AB) and the normal autoimmune repertoire of mammals remained quite obscure. AB may contribute to disorder-relevant phenotypes and are even believed to induce diseases themselves, but the circumstances under which AB become pathogenic are not fully understood. This review will focus on the highly frequent AB against the N-methyl-d-aspartate receptor 1 (NMDAR1-AB) as an illustrating example and provide a critical overview of current work (please note that the new nomenclature, GluN1, is disregarded here for consistency with the AB literature)...
June 2018: Current Opinion in Neurology
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