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anti--NMDA Receptor Encephalitis

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...
November 29, 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
Aurélien Maureille, Tanguy Fenouil, Bastien Joubert, Géraldine Picard, Véronique Rogemond, Anne-Laurie Pinto, Laure Thomas, François Ducray, Isabelle Quadrio, Dimitri Psimaras, Giulia Berzero, Jean-Christophe Antoine, Virginie Desestret, Jérôme Honnorat
OBJECTIVE: To report the clinical features and long-term outcome of 22 newly diagnosed paraneoplastic patients with GABAB receptor antibodies (GABAB R-Abs). METHODS: Retrospective clinical study of CSF-confirmed cases of GABAB R-Abs encephalitis. RESULTS: We identified 22 patients (4 female) with GABAB R-Abs, with a median age of 64 years (range 55-85). All were paraneoplastic: 20 small-cell lung cancer, one malignant thymoma, and one uncharacterized lung mass...
November 20, 2018: Journal of Neurology
Carlos Guevara, Gonzalo Farias, Carlos Silva-Rosas, Pablo Alarcon, Gabriel Abudinen, Julio Espinoza, Andrés Caro, Heather Angus-Leppan, Jose de Grazia
A 68-years-old Hispanic man, complained of night sweats, low grade fewer, unexplained weight loss, and memory problems over 3 months. Abdominal tomography showed multiple intra-abdominal adenopathy and biopsy confirmed classic Hodgkin's lymphoma. He commenced treatment with chemotherapy. Three months later, he had acute onset of inattention, auditory hallucinations and alterations of anterograde memory. The patient developed psychomotor agitation, unresponsive to a combination of neuroleptics and benzodiazepines...
2018: Frontiers in Immunology
Chaosheng Zeng, Lin Chen, Bocan Chen, Yi Cai, Pengxiang Li, Limin Yan, Dehua Zeng
Anti-N-methyl-D-aspartate-receptor (NMDAR) encephalitis is an autoimmune disorder characterized by memory deficits, psychiatric symptoms, and autonomic instability. The lack of suitable biomarkers targeting anti-NMDAR encephalitis makes the immunotherapy and prognosis challenging. In this study, we found that the Th17 cells were significantly accumulated in the cerebrospinal fluid (CSF) of anti-NMDAR encephalitis patients than that of control individuals. The concentration of the cytokines and chemokines including interleukin (IL)-1β, IL-17, IL-6, and CXCL-13 were significantly increased in the CSF of anti-NMDAR encephalitis patients...
November 9, 2018: Acta Biochimica et Biophysica Sinica
Yue-Wen Ding, Su-Yue Pan, Wei Xie, Hai-Ying Shen, Hong-Hao Wang
Objective: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe autoimmune disorder that mainly affects children and young women. The Fas system contains both membrane-bound versions of Fas (mFas) and Fas ligand (mFasL), and soluble versions (sFas and sFasL), which play important roles in apoptosis and regulation of the immune system. Both the levels of sFas and sFasL and the role they play in anti-NMDAR disease pathogenesis remain unclear. Methods: Forty-eight pairs of cerebrospinal fluid (CSF) and serum were collected from patients with anti-NMDAR encephalitis, encephalitis of other causes or peripheral neuropathy...
2018: Frontiers in Neurology
Morten B Schou, Sverre Georg Sæther, Ole Kristian Drange, Karoline Krane-Gartiser, Solveig K Reitan, Arne E Vaaler, Daniel Kondziella
BACKGROUND: The clinical significance of anti-neuronal antibodies in patients with psychiatric disorders, but without encephalitis, remains unknown. In patients admitted to acute psychiatric inpatient care we aimed to identify clinical features distinguishing anti-neuronal antibody positive patients from matched controls. RESULTS: Patients who were serum-positive to N-methyl D-aspartate receptor (NMDAR) (n = 21), contactin-associated protein 2 (CASPR2) (n = 14) and/or glutamic acid decarboxylase 65 (GAD65) (n = 9) antibodies (cases) were age and sex matched (1:2) with serum-negative patients from the same cohort (controls)...
November 3, 2018: BMC Neuroscience
Yasas Chandra Tanguturi, Allyson Witters Cundiff, Catherine Fuchs
Despite the majority of patients with anti-N-methyl d-aspartate receptor (NMDAR) antibody encephalitis presenting with catatonic symptoms, the literature has not focused on well-known treatments for catatonia, such as electroconvulsive therapy (ECT). The authors review the literature identifying case reports that document the effective use of ECT for anti-NMDAR encephalitis. They also identify gaps in the literature regarding use and documentation of ECT and review possible mechanisms of action for ECT. The authors propose identifying catatonia as a syndrome with multiple potential causes (including anti-NMDAR encephalitis) and suggest a standardized treatment approach using evidence-based catatonia treatments such as ECT and benzodiazepines...
January 2019: Child and Adolescent Psychiatric Clinics of North America
Jinyu Chen, Yuewen Ding, Dong Zheng, Zhanhang Wang, Suyue Pan, Teng Ji, Hai-Ying Shen, Honghao Wang
Objective: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis predominantly affects children and young women; the disease can have a multistage presentation and exhibit a wide variety of neuropsychiatric features. This study aimed to investigate the profile of YKL-40 (Chitinase 3-like 1) in anti-NMDAR encephalitis patients and evaluate its association with modified Rankin Scale (mRS) scores and expression of inflammatory cytokines. Methods: A total of 66 patients were enrolled in this study, 33 with anti-NMDAR encephalitis, 13 with viral meningitis and 20 with non-inflammatory neurological disease...
2018: Frontiers in Neurology
Marc Nikolaus, Jakob Kreye, Paul Turko, Imre Vida, Ellen Knierim, Harald Prüss
The y-aminobutyric acid A receptor (GABAA R) participates in most neurophysiological processes. Mutations cause epilepsy and neuropsychiatric pathologies. Recently a severe encephalitis with refractory seizures and antibodies against GABAA Rs has been described. Considering the complex subunit distribution of GABAA Rs, binding patterns of human GABAA R antibodies will help to understand the pathophysiology underlying diverse clinical pictures. We therefore investigated the cerebrospinal fluid (CSF) reactivity of a patient with GABAA R encephalitis using immunocytochemistry on murine brain sections and compared its specificity with commercial GABAA R antibodies...
October 19, 2018: Brain Research
Nan Jiang, Hongzhi Guan, Qiang Lu, Haitao Ren, Bin Peng
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a common cause of encephalitis in intensive care units. Until now, no reliable method has existed for predicting the outcome of anti-NMDAR encephalitis. In this study, we used quantitative electroencephalography (qEEG) to examine the brain function of anti-NMDAR encephalitis patients and assessed its predictive value. Twenty-six patients diagnosed with anti-NMDAR encephalitis were included and grouped according to whether they were treated in intensive care units (14 critically ill vs...
2018: Frontiers in Neurology
Margherita Nosadini, Shekeeb S Mohammad, Irene Toldo, Stefano Sartori, Russell C Dale
BACKGROUND: Available data on mycophenolate mofetil (MMF), azathioprine (AZA) and methotrexate (MTX) for paediatric-onset anti-N-methyl-d-aspartate receptor encephalitis (anti-NMDARE) is limited. METHODS: Systematic literature review on patients treated with MMF/AZA/MTX for paediatric-onset anti-NMDARE, with focus on modes of use, efficacy and safety. RESULTS: 87 patients were included (age at onset median 11 years, range 0.8-18 years; 69% females)...
September 27, 2018: European Journal of Paediatric Neurology: EJPN
Sabrina Kalam, Aravindhan Baheerathan, Cillian McNamara, Victoria Singh-Curry
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis was first reported in 2005 in four patients with ovarian teratomas; there have been many further cases reported since the antigen for the NMDAR antibody was confirmed in 2007. Patients characteristically have a well-defined set of features, characterised by psychiatric disturbance, seizures and cognitive disturbance, followed by movement disorders, disorders of consciousness and dysautonomia. To date, 14 cases of NMDAR encephalitis have been described in the context of pregnancy...
October 10, 2018: Practical Neurology
Sachi Tokunaga, Minako Ide, Takehiro Ishihara, Takako Matsumoto, Toshiro Maihara, Takeo Kato
Anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis is a type of immune-mediated encephalitis, which is a new category of treatment-responsive paraneoplastic encephalitis. In patients with this disease, electroencephalography (EEG) shows non-specific findings, but recently, a unique EEG pattern, named the extreme delta brush, was detected in 40% of adult patients and was suggested to be specific to this type of encephalitis. Here, we describe a two-year-old boy with anti-NMDAR encephalitis, who presented with speech arrest and disturbances of gait and cognition several weeks after developing febrile convulsions...
September 29, 2018: Brain & Development
Karine Beiruti, Anan Abu Awad, Galina Keigler, Chen Hanna Ryder, Radi Shahien
A previously healthy 35-year-old man suffering from behavioral and mental deterioration for three months was referred to our facility. On admission, the patient was dysphasic and had tonic-clonic seizures. Neurological examination showed mental confusion, euphoric mood, mania, paranoia, and mild motor dysphasia. Magnetic resonance imaging (MRI) was performed twice but no abnormalities were revealed. His acute confusional state, elevated cerebrospinal fluid (CSF) protein, epileptic seizure and electroencephalogram showing intermittent frontal slowness were all suggestive of encephalitis...
September 26, 2018: International Journal of STD & AIDS
Daniel Koh, Theodore Lau, Evonne Teoh, Kenneth K Lau
Anti-AMPA (anti-α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid) receptor limbic encephalitis is a rare autoimmune syndrome. It can be associated with an underlying malignancy, such as lung, breast, or thymus. We are reporting a case of a 19-year-old patient who presented with a 2-week history of confusion and short-term memory loss. A magnetic resonance imaging of brain showed increased T2 hyperintensity within the hippocampi bilaterally. Cerebrospinal fluid analysis was positive for anti-AMPA receptor antibodies...
October 2018: Journal of Pediatric Hematology/oncology
Ailiang Miao, Mingyang Du, Lingling Wang, Jianqing Ge, Hengdong Lu, Haiyan Xu, Hongxing Liu, Chuanyong Yu, Caiyun Wu, Yuan Gao, Jintao Sun, Qi Shi, Xiaoshan Wang
PURPOSE: This study aimed to determine the relation between electroclinical features and cerebrospinal fluid (CSF) antibody titers in patients with anti- N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis. METHOD: Clinical symptoms and electroencephalography (EEG) at different stages were analyzed in 51 hospitalized patients with anti-NMDAR encephalitis. RESULTS: Behavioral changes were the initial symptoms in 90.9% (20/22) of female patients with high (1:10 or 1:32) CSF antibody titers...
September 24, 2018: Clinical EEG and Neuroscience: Official Journal of the EEG and Clinical Neuroscience Society (ENCS)
Jindřich Kopecký, Ondřej Kubeček, Tomáš Geryk, Birgita Slováčková, Petr Hoffmann, Miroslav Žiaran, Peter Priester
BACKGROUND: Great progress has recently been made in the treatment of metastatic renal cell carcinoma, including the introduction of nivolumab, an immune checkpoint inhibitor. Despite promising results, this treatment brings a completely new spectrum of adverse events, distinct from those experienced with small-molecule kinase inhibitors. Neurologic immune-related adverse events may be serious and potentially life-threatening complications requiring immediate immunosuppressive therapy...
September 15, 2018: Journal of Medical Case Reports
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