Franziska Wagner, Angelika Goertzen, Orsolya Kiraly, Gregor Laube, Jakob Kreye, Otto W Witte, Harald Prüss, Rüdiger W Veh
Anti-NMDA receptor encephalitis was first described about thirteen years ago and has become one of the most important differential diagnoses for new-onset psychosis. The disease is mediated by autoantibodies against the subunit 1 of the N-methyl-D-aspartate receptor (NMDA-R1) in patients presenting with variable clinical symptoms. Patients often profit from immunmodulatory therapy, independent of their individual symptoms. In this study CSF samples as well as monoclonal antibodies derived from patients diagnosed with NMDA-R1 encephalitis were applied to rat hippocampus and visualized by immunocytochemistry...
July 28, 2020: Brain Research
Deborah Young
N-methyl-D-aspartate receptors (NMDAR) play a key role in brain development and function, including contributing to the pathogenesis of many neurological disorders. Immunization against the GluN1 subunit of the NMDAR and the production of GluN1 antibodies is associated with neuroprotective and seizure-protective effects in rodent models of stroke and epilepsy, respectively. Whilst these data suggest the potential for the development of GluN1 antibody therapy, paradoxically GluN1 autoantibodies in humans are associated with the pathogenesis of the autoimmune disease anti-NMDA receptor encephalitis...
2020: Frontiers in Neurology
Talal Alkayali, Stephen Bell, Caitlin Bass, Natalia Lattanzio, Natan Kraitman
Encephalitis is an inflammatory process of the brain that is most commonly related to infectious etiology; nonetheless, autoimmune encephalitis has been an increasingly identified entity that can cause it as well and should be considered. N-methyl-D-aspartate (NMDA) receptor encephalitis is a recently identified process but remains less recognized than autoimmune encephalitis. We report a case of an 18-year old female who initially presented with seizures and later developed behavioral symptoms of agitation, crying, screaming, and emotional lability...
July 8, 2020: Curēus
Lauren E Gillespie, Amanda Dave, Amy Goldstein
This is the first reported case of familial voltage-gated potassium channel (VGKC) autoimmune encephalitis. The symptoms of autoimmune encephalitis can mimic infectious encephalitis with headache, fatigue, and neuropsychiatric symptoms. Autoimmunity is emerging as a distinct cause of encephalitis in the children. Prompt recognition, diagnosis, and treatment are important to prevent brain damage. Two brothers presented two years apart with different symptoms. The explanation for their distinct symptoms lies in the multifactorial development of autoimmunity...
June 20, 2020: Curēus
Giulia Monti, Giada Giovannini, Andrea Marudi, Roberta Bedin, Alessandra Melegari, Anna Maria Simone, Mario Santangelo, Alessandro Pignatti, Elisabetta Bertellini, Tommaso Trenti, Stefano Meletti
No abstract text is available yet for this article.
July 15, 2020: Seizure: the Journal of the British Epilepsy Association
A N Belova, V N Grygorieva, M V Rasteryaeva, E A Ruina, E M Belova, V S Solovieva, A N Boyko
Autoimmune encephalitis with antibodies to NMDA receptors (anti-NMDAR encephalitis), is the most common form of autoimmune encephalitis. The disease is curable, however, the lack of timely therapy can lead to the disability of patients or to the death. Difficulties in the diagnosis of anti-NMDAR encephalitis are caused by the heterogeneity of its manifestations, a possible overlapping with other autoimmune diseases and insufficient awareness about this form of encephalitis. This article describes the case of anti-NMDAR encephalitis associated with recurrent optic neuritis which might be an atypical manifestation for this disease...
2020: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
Jose Gabriel Solis, Arturo Olascoaga Lugo, Marco Antonio Rodríguez Florido, Bayron Alexander Sandoval Bonilla, Jose Malagón Rangel
Sarcoidosis is a multisystemic inflammatory disease of unknown cause. It is characterized by the presence of noncaseating granuloma on a biopsy specimen. Clinical presentation varies across case report series with myriad of symptoms ranging from fever, respiratory symptoms, and skin lesions, or atypical symptoms like heart block or neurological symptoms. Hence, we report the case of a 22-year-old woman with encephalitis, a pituitary mass, and adipsic diabetes insipidus. The diagnostic approach did not end on the biopsy of the lesion, which reported noncaseating granulomas; on the contrary, it was the beginning of a path to exclude other causes of the central nervous system granulomas that ended with the diagnosis of the isolated central nervous system sarcoidosis...
2020: Case Reports in Neurological Medicine
Irina Chernyshkova, Bebsy Estefan, Md Rezaul Hoque, Alice Lee
We describe a case of probable autoimmune encephalitis developed as a result of paraneoplastic syndrome in a woman with an ovarian teratoma. Patients may present with psychiatric and neurological symptoms, which are caused by anti-N-methyl-D-aspartate (anti-NMDA) receptor antibodies produced in response to a teratoma that crosses the blood-brain barrier and damages brain tissue in the limbic area, causing encephalitis. Our patient presented with seizures, myoclonus, and memory problems. This is a relatively newly discovered and rare problem; however, it can be quite debilitating if left untreated...
June 7, 2020: Curēus
Zina H Stavitsky, Colin H Nguyen, James Martin Johnston
Anti-N-methyl-D-aspartate receptor encephalitis (NMDARE) is an autoimmune form of encephalitis, first described in 2005 and now recognized as among the more common causes of encephalitis. While NMDARE can result in permanent neurologic deficits or even mortality, the prognosis in children is generally more favorable; 75-85% of children and teenagers achieve a full or substantial recovery. We describe here a preadolescent female, whose course of NMDARE was complicated by a unilateral stroke, resulting in permanent deficits...
May 2020: Case Reports in Neurology
Mette Scheller Nissen, Matias Ryding, Morten Meyer, Morten Blaabjerg
Autoimmune encephalitides (AE) comprises a group of diseases with antibodies against neuronal synaptic and cell surface antigens. Since the discovery of the most common subtype, N-Methyl-D-Aspartate (NMDA) receptor encephalitis, an astonishing number of novel disease-causing antibodies have been described. This includes other glutamatergic and GABAergic receptor antibodies and antibodies against various other surface proteins. Many of these novel conditions present as limbic encephalitis with memory impairment, psychiatric features and epileptic seizures, often alongside subtype specific clinical features...
July 8, 2020: CNS & Neurological Disorders Drug Targets
Felipe Alberto Gómez Ávila, Maricarmen Fernández González-Aragón, Álvaro Moreno Avellán, Daniel San-Juan
PURPOSE: Anti-N-methyl-D-aspartate receptor encephalitis is produced by an autoimmune reaction against macromolecular structures that form ionotropic receptors for glutamic acid NR2A and NR2B subunits. Other important findings are the multiple clinical and paraclinical manifestations, among which the EEG stands out. We characterized EEG patterns and their association with functional disability and hospitalization time in patients with anti-N-methyl-D-aspartate receptor encephalitis admitted in our center...
July 1, 2020: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
Z Y Fu, H T Ren, L P Xue, Y N Tian, S Y Fan, Y H Zhao, N Lin, H X Yin, N Jiang, B Peng, L Y Cui, H Z Guan
Objective: To explore the clinical features of post-viral-encephalitis autoimmune encephalitis (PVEAE). Methods: Ten cases of PVEAE, who were hospitalized in the Neurology Department of Peking Union Medical College Hospital (PUMCH) between November 2014 and October 2019, were retrospectively reviewed. Clinical manifestation, immunology, neuroradiology, treatment and outcomes were analyzed. Results: There were 5 males and 5 females, with a median age of 44 (18, 66) years. Of 9 cases, the median interval between the two onsets of encephalitis was 37 (24, 60) days, and there was no obvious interval in case 7...
July 7, 2020: Zhonghua Yi Xue za Zhi [Chinese medical journal]
Xiaoxiao Liu, Liqiong Guo, Cheng Liang
Anti-N-methyl-d-aspartate (NMDA) receptor encephalitis is a kind of autoimmune disease aiming at NR1 subunit of NMDA receptor. In the early stage, functional damage is the main cause. Electroencephalogram (EEG) can reflect the abnormal brain function by recording the changes of EEG signals. The common EEG patterns of anti NMDA receptor encephalitis are slow wave abnormality, epileptic discharge, a large number of β activity, extreme delta brush, etc. Here we review the waveform characteristics, origin, pathogenesis and clinical value of EEG in patients with NMDA receptor encephalitis...
May 25, 2020: Zhejiang da Xue Xue Bao. Yi Xue Ban, Journal of Zhejiang University. Medical Sciences
Vladimir Ferrafiat, Elise Riquin, Elena Freri, Tiziana Granata, Nardo Nardocci, François Medjkane, Claire Corfiotti, Alessandra Tozzo, Huges Pellerin, Xavier Benarous, Julien Haroche, Zahir Amoura, Philippe Duverger, Renaud Jardri, Priscille Gerardin, David Cohen, Angèle Consoli, Marie Raffin
OBJECTIVES: Patients with autoimmune encephalitis (AE) are likely to exhibit an acute onset of severe psychiatric features, including psychosis and/or catatonia. Based on the high prevalence of catatonia in AE and our clinical experience, we hypothesized that catatonia might be a marker of severity requiring more aggressive treatment approaches. METHODS: To reach a sufficient number of cases with brain-autoimmune conditions, we pooled two samples (N = 58): the first from the French National Network of Rare Psychiatric diseases and the second from the largest Italian neuro-pediatrics center for encephalopathies...
June 30, 2020: Progress in Neuro-psychopharmacology & Biological Psychiatry
Mihai Ceanga, Ha-Yeun Chung, Christian Geis
No abstract text is available yet for this article.
June 2020: Annals of Translational Medicine
Dongming Liu, Hongwei Xu, Fang Yi
Cryptococcal encephalitis is a fatal central nervous system infectious disease,whereas anti-N-methyl-D-aspartate(NMDA)receptor encephalitis(NMDARE)is an autoimmune syndrome associated with psychological symptoms,behavioural abnormalities,seizures,and dyskinesias.Despite their distinct pathologies and pathogenic mechanisms,both of them can lead to cognitive dysfunction and abnormal behaviors,although anti-NMDARE can also have mood and mental disorders as its core manifestations.A patient with nephrotic syndrome accompanied by both cryptococcal encephalitis and anti-NMDARE was treated in our center,which for the first confirmed that these two conditions could coexist in one patient...
June 30, 2020: Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
Shilpa Devamare, Kavish Ihtisham, Himani Bhasin, Vikram Bhasker, Suvasini Sharma, Manjari Tripathi
No abstract text is available yet for this article.
May 2020: Annals of Indian Academy of Neurology
Akira Kurozumi, Yosuke Okada, Kosuke Nishio, Yoshiya Tanaka
Anti-N-methyl-D-aspartate receptor (NMDA-R) encephalitis is an autoimmune disorder in which autoantibodies in the limbic system bind to GluN1 subunits of NMDA-Rs in the brain. We report a rare case of autoimmune polyendocrine syndrome type 3 (APS-3) complicated by anti-NMDA-R encephalitis. After hospitalization for type 1 diabetes the 39-year-old patient developed various schizophreniform symptoms and seizures following cold-like symptoms. These findings are consistent with the diagnosis of anti-NMDA-R encephalitis...
June 30, 2020: Journal of Diabetes Investigation
Aaron Nizam, Andrew W Menzin, Jill S Whyte
Background: Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis has been described in increasing frequency in association with benign, mature ovarian teratoma. Affected patients typically present with paraneoplastic limbic encephalitis with flu-like symptoms followed by altered mental status, acute psychiatric symptoms, seizures and amnesia. These symptoms can rapidly progressive if not treated aggressively with surgical resection. Profound neurological symptoms may require immunotherapy...
August 2020: Gynecologic Oncology Reports
Namita Neerukonda, Michael Bliss, Abtin Jafroodifar, Luba Leontieva
Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis has become one of the more well-known autoimmune diseases affecting the brain and it is characterized by a multitude of progressive neuropsychiatric symptoms. The following case describes the clinical course of an 18-year-old female with excited type catatonia secondary to anti-NMDA receptor encephalitis. The patient had been brought to the ED by her parents in an acutely psychotic state characterized by profound disorganization and vivid visual hallucinations...
June 18, 2020: Curēus
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