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
Helena Ariño, Amaia Muñoz-Lopetegi, Eugenia Martinez-Hernandez, Thaís Armangue, Mireia Rosa-Justicia, Domingo Escudero, Nuria Matos, Francesc Graus, Gisela Sugranyes, Josefina Castro-Fornieles, Albert Compte, Josep Dalmau, Joan Santamaria
OBJECTIVE: To describe the sleep disorders in anti-NMDA receptor encephalitis (anti-NMDARe). METHODS: Patients recovering from anti-NMDARe were invited to participate in a prospective observational single center study including comprehensive clinical, video-polysomnography (V-PSG) sleep assessment and neuropsychological evaluation. Age and sex-matched healthy participants served as controls. RESULTS: Eighteen patients (89% female, median age 26 years, IQR 21-29) and 21 controls (81% female, median age 23 years, IQR 18-26) were included...
June 23, 2020: Neurology
Anh-Vu Nguyen, Keith A Young, James A Bourgeois
Objective: To present a review of the literature on the clinical presentation and pathophysiology of anti-N-methyl-d-aspartate receptor encephalopathy (ANMDARE) with attention to both the more commonly recognized psychotic symptom prodrome and the less well-understood depressive symptom prodrome. Data Sources: The search for clinical neuropsychiatric phenomena and proposed mechanisms involved in ANMDARE pathophysiology was conducted in PubMed. English-language articles published up to September 2019 were identified using a combination of the following search terms: N-methyl-d-aspartate, anti-NMDA receptor encephalitis, schizophrenia, psychosis, depression, major depressive disorder, bipolar I disorder, bipolar II disorder, anxiety, and posttraumatic stress disorder...
June 18, 2020: Primary Care Companion to CNS Disorders
Romain Delangle, Sophie Demeret, Geoffroy Canlorbe, Leslie Chelon, Jérémie Belghiti, Clémentine Gonthier, Marianne Nikpayam, Catherine Uzan, Henri Azaïs
BACKGROUND: Anti-NMDA receptor antibody (anti-NMDAr) encephalitis, although still a rare condition, is well known to neurologists as it is the leading cause of non-infectious acute encephalitis in young women. However, this is less well known to gynecologists, who may have a decisive role in etiological management. Indeed, in 30-60% of cases in women of childbearing age, it is associated with the presence of an ovarian teratoma, whose removal is crucial in the resolution of symptomatology...
June 17, 2020: Archives of Gynecology and Obstetrics
Serena Marita Lazzarin, Marco Vabanesi, Giordano Cecchetti, Raffaella Fazio, Giovanna Franca Fanelli, Maria Antonietta Volonté, Angela Genchi, Antonino Giordano, Vittorio Martinelli, Sergio Colombo, Paolo Beccaria, Milena Mucci, Jacopo Peccatori, Massimo Filippi, Fabio Minicucci
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a potentially fatal autoimmune disease, characterized by autoantibody-mediated neurotransmission impairment in multiple brain locations. The course of this condition often comprises altered mental status, autonomic dysfunctions, refractory seizures and hyperkinetic movement disorders. Available disease-modifying therapies include corticosteroids, i.v. immunoglobulins, plasma exchange, rituximab and cyclophosphamide. In a subgroup of patients not responding to B-cell depletion, bortezomib, a proteasome inhibitor, has shown promising evidence of efficacy...
June 13, 2020: Journal of Neurology
Yusuke Sakiyama, Eiji Matsuura, Ayano Shigehisa, Yuki Hamada, Mika Dozono, Satoshi Nozuma, Tomonori Nakamura, Keiko Higashi, Akihiro Hashiguchi, Yukitoshi Takahashi, Hiroshi Takashima
We herein report a 50-year-old man with alcoholic cirrhosis who developed loss of consciousness and tremor of the upper limbs. Magnetic resonance imaging findings were suggestive of limbic encephalitis with bilateral hippocampal damage, and a cerebrospinal fluid (CSF) examination confirmed anti-N-methyl-D-aspartate (NMDA) and anti-glutamate receptor antibodies. Despite initial corticosteroid therapy, meningeal irritation symptoms appeared, owing to the development of cryptococcal meningitis (CM), diagnosed by the detection of cryptococcal capsular polysaccharide antigen in the follow-up CSF analysis...
June 9, 2020: Internal Medicine
Yuya Kobayashi, Mitsuto Sato, Michiaki Kinoshita, Masaki Kakizawa, Kayoko Higuchi, Yamada Mitsunori, Minori Kodaira, Yoshiki Sekijima
A 61-year-old Japanese man presented with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. NR1 antibodies were detected in his cerebrospinal fluid. Chest computed tomography revealed lung tumor. The patient was diagnosed with paraneoplastic anti-NMDAR encephalitis associated with lung cancer and treated with two cycles of intravenous high-dose methylprednisolone and one cycle of intravenous immunoglobulin. However, he died one year later without improvement. An autopsy confirmed small-cell lung cancer (SCLC)...
June 2, 2020: Internal Medicine
David Moreno-Ajona, Elena Prieto, Fabiana Grisanti, Inés Esparragosa, Lizeth Sánchez Orduz, Jaime Gállego Pérez-Larraya, Javier Arbizu, Mario Riverol
Brain positron emission tomography imaging with 18Fluorine-fluorodeoxyglucose (FDG-PET) has demonstrated utility in suspected autoimmune encephalitis. Visual and/or assisted image reading is not well established to evaluate hypometabolism/hypermetabolism. We retrospectively evaluated patients with autoimmune encephalitis between 2003 and 2018. Patients underwent EEG, brain magnetic resonance imaging (MRI), cerebrospinal fluid (CSF) sampling and autoantibodies testing. Individual FDG-PET images were evaluated by standard visual reading and assisted by voxel-based analyses, compared to a normal database...
May 29, 2020: Diagnostics
Ana Paula Bornes da Silva, Rodrigo Braccini Madeira Silva, Leise Daniele Sckenal Goi, Rachel Dias Molina, Denise Cantarelli Machado, Douglas Kazutoshi Sato
Immune-mediated inflammatory diseases of the central nervous system (CNS) are a group of neurological disorders in which inflammation and/or demyelination are induced by cellular and humoral immune responses specific to CNS antigens. They include diseases such as multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD), acute disseminated encephalomyelitis (ADEM) and anti-NMDA receptor encephalitis (NMDAR encephalitis). Over the years, many in vivo and in vitro models were used to study clinical, pathological, physiological and immunological features of these neuroimmunological disorders...
2020: Frontiers in Neurology
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