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anti--NMDA Receptor Encephalitis

Olga Taraschenko, Howard S Fox, Sean J Pittock, Anastasia Zekeridou, Maftuna Gafurova, Ember Eldridge, Jinxu Liu, Shashank M Dravid, Raymond Dingledine
OBJECTIVE: Seizures develop in 80% of patients with anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis, and these represent a major cause of morbidity and mortality. Anti-NMDAR antibodies have been linked to memory loss in encephalitis; however, their role in seizures has not been established. We determined whether anti-NMDAR antibodies from autoimmune encephalitis patients are pathogenic for seizures. METHODS: We performed continuous intracerebroventricular infusion of cerebrospinal fluid (CSF) or purified immunoglobulin (IgG) from the CSF of patients with anti-NMDAR encephalitis or polyclonal rabbit anti-NMDAR IgG, in male C57BL/6 mice...
February 11, 2019: Epilepsia
Joseph J Cooper, Khalid I Afzal
No abstract text is available yet for this article.
February 1, 2019: Journal of ECT
Kristin M Galetta, Shamik Bhattacharyya
Autoimmune disorders of the central nervous system are common and often affect people in the most productive years of their lives. Among primary autoimmune diseases of the central nervous system, multiple sclerosis is most prevalent in the United States. Many other autoantibody-mediated neurologic syndromes have been identified within the past 2 to 3 decades, including neuromyelitis optica and anti-N-methyl-D aspartate receptor encephalitis. Finally, the central nervous system can also be affected by systemic autoimmune diseases such as sarcoidosis...
March 2019: Medical Clinics of North America
G L Wang, F Yin, Y Wang, W X Zhang, F He, L F Yang, L W Wu, J Peng
Objective: To investigate the clinical features, treatment strategies and long term outcomes of children with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. Methods: The data of clinical features, auxiliary examinations, treatments and prognosis in children with anti-NMDAR encephalitis in Xiangya Hospital of Central South University from March 2014 to October 2017 were collected and retrospectively analyzed. A total of 71 patients were enrolled, including 33 males and 38 females. The youngest age of onset was 4 months old, and the age of onset was (9±4) years...
February 2, 2019: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Stefanie Kar Yan Hung, Fu Liong Hiew, Shanthi Viswanathan
Multiple co-infections can predispose a patient to autoimmune encephalitis. Out of thirty cases of N-methyl-D-aspartate receptor (NMDAR) encephalitis seen at a single tertiary referral center, only two cases of co-infection with NMDAR encephalitis were identified. One of these cases was highly interesting due to the presence of more than one co-infections along with the presence of cortical dysfunction, seizures, and orofacial dyskinesias at the onset in a male in the absence of tumors, which was refractory to initial treatment...
January 2019: Annals of Indian Academy of Neurology
Michitaka Funayama, Jin Mizushima, Taketo Takata, Akihiro Koreki, Masaru Mimura
Most patients with N-methyl-D-aspartate receptor (NMDAR) encephalitis initially present with psychiatric symptoms. Although a delayed diagnosis may lead to a poor outcome, psychiatric symptoms that could differentiate anti-NMDAR encephalitis from other psychoses have not been fully investigated. We evaluated two patients with anti-NMDAR encephalitis who were observed by psychiatrists from onset throughout the course of disease. Both patients exhibited disorientation, memory deficits, perceptual disturbances, hallucinations, and mood liability...
January 25, 2019: Neurocase
Zhihua Si, Aihua Wang, Jinzhi Liu, Zixian Zhang, Ke Hu
OBJECTIVE: To explore the clinical, imaging, and electroencephalogram (EEG) findings, as well as the treatment and prognosis of five patients with anti-γ-aminobutyric acid B receptor (GABAB R) encephalitis and review the current literature to gain a deeper understanding and improve the clinical diagnostic ability of the disease. METHODS: Clinical data such as blood examination, imaging, computed tomography (CT), EEG, and magnetic resonance imaging (MRI) findings from five patients with anti-GABAB R encephalitis were retrospectively analyzed...
January 22, 2019: Neurological Sciences
Joanne E Davies, Sarah Johnson
A middle-aged woman presented with a history of symptoms of depression with psychotic features severely affecting her physical health. Neuroimaging of her brain suggested pathological changes out of keeping with her age, leading to further investigations including genetic testing for cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis was also considered as a differential diagnosis as an initial serum test for anti-NMDAR autoantibodies was positive...
January 20, 2019: BMJ Case Reports
Byung-Soo Park, Gyung Mo Son, Hyun Sung Kim, Jacob Medina, Jae Wook Cho
No abstract text is available yet for this article.
February 2019: Clinical Neurology and Neurosurgery
Lucas Treidl, Shahriar Izadi, Angelika Rießland-Seifert
Anti-N-methyl-D-aspartate receptor encephalitis (anti-NMDAR encephalitis) is a rare and recently discovered disease. Affected patients are initially often referred to psychiatric departments due to prominent neuropsychiatric symptoms.We present the course of the illness of an 18-year-old female patient, who was primarily and for the first time admitted to the 1. Psychiatric Department of the Otto Wagner Hospital Vienna due to a psychotic disorder. With fluctuating clinical course, further diagnostics were performed and the patient was diagnosed with anti-NMDAR encephalitis...
January 17, 2019: Neuropsychiatrie: Klinik, Diagnostik, Therapie und Rehabilitation
Yaqing Shu, Wei Qiu, Junfeng Zheng, Xiaobo Sun, Junping Yin, Xiaoli Yang, Xiaoyang Yue, Chen Chen, Zhihui Deng, Shasha Li, Yu Yang, Fuhua Peng, Zhengqi Lu, Xueqiang Hu, Frank Petersen, Xinhua Yu
BACKGROUND AND OBJECTIVE: Aetiology and pathogenesis of anti- N -methyl-D-aspartate receptor (anti-NMDAR) encephalitis, the most common autoimmune encephalitis, is largely unknown. Since an association of the disease with the human leucocyte antigen (HLA) has not been shown so far, we here investigated whether anti-NMDAR encephalitis is associated with the HLA locus. METHODS: HLA loci of 61 patients with anti-NMDAR encephalitis and 571 healthy controls from the Chinese Han population were genotyped and analysed for this study...
January 13, 2019: Journal of Neurology, Neurosurgery, and Psychiatry
Hisanao Akiyama, Rie Sasaki, Yasuhiro Hasegawa
RATIONALE: We report this 1st case because perampanel may be effective against the seizures and abnormal behavior that occur in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. PATIENT CONCERNS: The patient was a healthy 26-year-old woman who suddenly developed seizures and exhibited abnormal behavior. DIAGNOSES: NMDAR encephalitis was diagnosed based on positive NMDAR antibody on cerebrospinal fluid analysis. INTERVENTIONS: Treatment with anticonvulsants and sedatives was started immediately, along with steroid pulse therapy and plasmapheresis, but these measures did not adequately control the repeated seizures and abnormal behavior...
January 2019: Medicine (Baltimore)
Maoqiang Tian, Juan Li, Wenting Lei, Xiaomei Shu
Anti-N-methyl-D-aspartate receptor encephalitis (anti-NMDARe) was originally described as a paraneoplastic disease with more than 50% cases involving a tumor. However, tumor incidence in anti-NMDARe in children is much lower. Herpes simplex virus-induced anti-NMDARe has been well-described; however, findings on Japanese encephalitis virus (JEV)-induced anti-NMDARe are scarce. Here, we describe a 7-year-old boy who presented with fever and headache that progressed to seizures and disturbance of consciousness...
January 8, 2019: Neuropediatrics
Bojan Rojc, Barbara Podnar, Francesc Graus
We report the clinical association of myelin-oligodendrocyte-glycoprotein (MOG) IgG-associated encephalomyelitis (MOG-EM) and anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis. A 47-year old male presented to our hospital with right eye optic neuritis in February 2018. He had a history of recurrent bilateral optic neuritis since 2001 and in 2013 was treated of a anti-NMDAR encephalitis. He never had any CNS demyelination event besides optic neuritis. At the time of anti-NMDAR encephalitis both NMDAR and MOG antibodies were positive in serum and CSF...
March 15, 2019: Journal of Neuroimmunology
Rani A Sarkis, M Justin Coffey, Joseph J Cooper, Islam Hassan, Belinda Lennox
OBJECTIVE: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder characterized by prominent neuropsychiatric symptoms. Given the nature of its pathophysiology, psychiatrists tend to be one of the first clinicians encountering patients with the disease. METHODS: In the present review of patients described in the literature with psychiatric symptoms, the authors aimed to characterize the psychiatric symptoms of the disease and its management in adults and adolescents as well as children (≤12 years old)...
December 18, 2018: Journal of Neuropsychiatry and Clinical Neurosciences
Hua Li, Yan-Ke Guo, Ying-Lin Cui, Tao Peng
RATIONALE: Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is an auto-immune and paraneoplastic encephalitis with prominent neuropsychiatric manifestations. The N-methyl-D-aspartate receptor is located in the forebrain and hippocampus and plays a role in learning and memory. PATIENT CONCERNS: A 29-year-old female patient with anti-NMDAR encephalitis, was reported and we also reviewed the literature and summarised the characteristics of the cases. DIAGNOSES: In the present study, we reported 1 patient with anti-NMDAR encephalitis diagnosed by the detection of anti-NMDAR antibodies in serum and cerebrospinal fluid (CSF)...
December 2018: Medicine (Baltimore)
Yaqing Shu, Yue Xu, Chen Chen, Jing Li, Rui Li, Haotian Wu, Xueqiang Hu, Zhengqi Lu, Xinhua Yu, Wei Qiu
BACKGROUND AND OBJECTIVE: Low serum levels of bilirubin and albumin are associated with multiple autoimmune diseases, but their role in anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is unknown. METHODS: Serum bilirubin and albumin levels were evaluated in 60 patients with anti-NMDAR encephalitis, 50 cryptococcal encephalitis, and 145 healthy controls (CTLs). Of the 60 anti-NMDAR encephalitis patients, 30 had a follow-up evaluation at 3 months after admission...
2018: Neuroimmunomodulation
Shu Pin Yu, Kien Chai Ong, David Perera, Kum Thong Wong
Japanese encephalitis virus (JEV) causes central nervous system neuronal injury and inflammation. A clear understanding of neuronal responses to JEV infection remains elusive. Using the Affymetrix array to investigate the transcriptome of infected SK-N-MC cells, 1316 and 2737 dysregulated genes (≥ 2/-2 fold change, P < 0.05) were found at 48 hours post-infection (hpi) and 60 hpi, respectively. The genes were mainly involved in anti-microbial responses, cell signalling, cellular function and maintenance, and cell death and survival...
November 24, 2018: Virology
B P Kelley, J J Corrigan, S C Patel, B D Griffith
Systemic lupus erythematosus is a chronic autoimmune disease characterized by the production of autoantibodies resulting in tissue injury across multiple organs; up to 50% of patients develop neurologic involvement, collectively referred to as neuropsychiatric systemic lupus erythematosus. The cases in this clinical report will highlight a subtype of neuropsychiatric systemic lupus erythematosus demonstrating imaging findings of striatal inflammation responsive to plasmapheresis similar to those in the subset of N -methyl-D-aspartate receptor autoimmune encephalitis that involves the striatum...
November 22, 2018: AJNR. American Journal of Neuroradiology
Daniel Almeida do Valle, Joselainy Stela Pires Galeazzi, Mayara de Rezende Machado, Vanessa Catarine Silva Abreu Ribeiro Dos Santos, Alcir Francisco da Silva, Alfredo Lohr Júnior, Mara Lúcia Schmitz Ferreira Santos, Rosana Herminia Scola
PURPOSE: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an immune-mediated disease of the central nervous system (CNS). The aim of this study was to describe the variability of clinical presentation in anti-NMDAR encephalitis, treatment and outcomes in a case series of children and adolescents. METHODS: Retrospectively analyse patients diagnosed with anti-NMDAR encephalitis, from 2010 to 2018. RESULTS: The study population consisted of nine children with anti-NMDAR encephalitis from southern Brazil, six females and three males, aged 5 months to 16 years (mean 5 years)...
November 20, 2018: Neurological Sciences
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