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nmda receptor encephalitis iv immunoglobulins

C G Bien
The paraneoplastic and autoimmune encephalitides are now well-established entities. Detection of neural autoantibodies enables specific diagnoses, provides information on the underlying disease pathophysiology, immunological treatability and the likelihood of a tumor being the underlying cause. This is true for the "high ranking" neural antibodies that have been established in the context of circumscribed clinical images and in consideration of large control groups, have been found in the same way by other laboratories and they respond to immunotherapy...
March 13, 2018: Der Nervenarzt
Hsiuying Wang
Treatments for the anti-NMDA receptor encephalitis usually include steroids, intravenous immunoglobulin, plasma exchange, plasmapheresis, rituximab, cyclophosphamide and tumor resection. We aimed to compare the efficacy of the treatments including intravenous immunoglobulin, plasma exchange, plasmapheresis, rituximab or cyclophosphamide for male anti-NMDA receptor encephalitis patients without tumor and to discuss potential biomarkers for this disease. The Fisher exact test and the contingency table analysis were used to analyze the treatment efficacy for both male and female these patients...
February 21, 2018: Current Medicinal Chemistry
Siyuan Fan, Yan Xu, Haitao Ren, Hongzhi Guan, Feng Feng, Xuehui Gao, Ding Ding, Fang Fang, Guangliang Shan, Tianjia Guan, Yao Zhang, Yi Dai, Ming Yao, Bin Peng, Yicheng Zhu, Liying Cui
BACKGROUND: Myelin oligodendrocyte glycoprotein (MOG)-antibody (ab) disease and AQP4-IgG-positive neuromyelitis optica spectrum disorder (NMOSD) can co-exist with anti-NMDA (N-methyl-D-aspartate) receptor encephalitis (NMDARe). OBJECTIVES: To characterize MOG-ab disease and AQP4-IgG-positive NMOSD during NMDARe. METHODS: We analyzed all the patients with overlapping MOG-ab disease and NMDARe (MNOS) and patients with AQP4-IgG-positive NMOSD and NMDARe (ANOS) in our hospital and compared those data with data from systematically review of previously published reports...
January 30, 2018: Multiple Sclerosis and related Disorders
Kenneth E Remy, Jason W Custer, Joshua Cappell, Cortney B Foster, Nan A Garber, L Kyle Walker, Liliana Simon, Dayanand Bagdure
Purpose: Anti- N -methyl-d-aspartate receptor (NMDAR) encephalitis is being recognized with increasing frequency among children. Given the paucity of evidence to guide the critical care management of these complex patients, we provide a comprehensive review of the literature with pooled analysis of published case reports and case series. Methods: We performed a comprehensive literature search using PubMed, Scopus, EMBASE, and Web of Science for relevant published studies...
2017: Frontiers in Pediatrics
Ching Soong Khoo, Nurul Husna Zulkifli, Siti Soraya Ab Rahman
We describe a case of a young lady with anti-NMDA (N-methyl-D-aspartate) receptor encephalitis, who initially presented with status epilepticus. Her seizures and orofacial dyskinesia were refractory to four anticonvulsants. She received intravenous immunoglobulin and a left ovarian tumour (an associated feature) was resected. However, her outcome was poor because of delayed treatment, autonomic dysfunction and complications of prolonged hospitalisation. This case highlights the importance of an early recognition of this rare but increasingly recognised disease...
October 2017: Clinical Medicine: Journal of the Royal College of Physicians of London
Ajay Goenka, Vivek Jain, Hiroki Nariai, Alfred Spiro, Mitchell Steinschneider
OBJECTIVE: There is a wide spectrum of clinical manifestations in children with anti-N-methyl-d-aspartate (NMDA) receptor antibody encephalitis from two different health care settings. METHODS: We describe our experience with 13 patients (median age, 7 years; range, 5 months to 19 years) presenting to tertiary referral centers in India and the United States. RESULTS: Initial manifestations were neurological (seizures or movement disorders) in eight patients, and psychiatric (e...
July 2017: Pediatric Neurology
Xue Xiao, Shunping Gui, Peng Bai, Yi Bai, Dan Shan, Yayi Hu, Tri M Bui-Nguyen, Rong Zhou
Anti-N-methyl-d-aspartate receptor (anti-NMDA-R) encephalitis is an autoimmune disorder that was first described by Dr Vitaliani in 2005. In 2007, Dalmau et al. found anti-NMDA-R antibody expressed both in the hippocampus and prefrontal nerve cell membrane, finally proposing the diagnosis of autoimmune anti-NMDA-R encephalitis. Most of the patients are female (91%), with ages ranging from 4 to 76 years. The average age is 23 years, a birth peak age, although anti-NMDA-R encephalitis is rare during pregnancy...
April 2017: Journal of Obstetrics and Gynaecology Research
Joseph Fisher, Clifford Ellingson, Noah Tolby
Anti-N-methyl-d-aspartate antibody receptor (NMDAR) encephalitis is a newly recognized disease increasing in diagnostic frequency. A 27-year-old female presented with symptoms of oral dyskinesia, tachycardia, and altered mental status following a three-month history of depression, lethargy, catatonia, and auditory hallucinations. We utilized our facilities neurology and psychiatry consult services, performed a lumbar puncture (LP), and requested NMDAR antibody titers. Following admission the Anti-NMDAR antibody titer was elevated warranting treatment with intravenous immunoglobulin (IVIG), corticosteroids, and later rituximab...
July 2017: American Journal of Emergency Medicine
Mai Nguyen Thi Hoang, Phu Nguyen Hoan, Tan Le Van, Angela McBride, Nghia Ho Dang Trung, Thanh Tran Tan, Hong Nguyen Thi Thu, Dorothee Heemskerk, Jeremy Day, Angela Vincent, Chau Nguyen Van Vinh, Guy Thwaites
INTRODUCTION: Anti-NMDA receptor encephalitis is increasingly recognised as an important differential diagnosis in patients with encephalitis of unknown aetiology. We report the first case series of patients diagnosed in Vietnam. METHODS: Samples of CSF from patients with presumed encephalitis but negative microbiological investigations, who exhibited dyskinesia, autonomic instability or psychosis were tested for antibodies against the NR1 subunit of the glutamate (type-NMDA) receptor using an indirect immunofluorescence assay...
February 15, 2017: Journal of the Neurological Sciences
Franziska Scheibe, Harald Prüss, Annerose M Mengel, Siegfried Kohler, Astrid Nümann, Martin Köhnlein, Klemens Ruprecht, Tobias Alexander, Falk Hiepe, Andreas Meisel
OBJECTIVE: We assessed the therapeutic potential of the plasma-cell-depleting proteasome inhibitor bortezomib in severe and therapy-refractory cases of anti-NMDA receptor (anti-NMDAR) encephalitis. METHODS: Five severely affected patients with anti-NMDAR encephalitis with delayed treatment response or resistance to standard immunosuppressive and B-cell-depleting drugs (corticosteroids, IV immunoglobulins, plasma exchange, immunoadsorption, rituximab, cyclophosphamide) who required medical treatment and artificial ventilation on intensive care units were treated with 1-6 cycles of 1...
January 24, 2017: Neurology
Kevin Gurcharran, Shefali Karkare
BACKGROUND: N-methyl-D-aspartate (NMDA) receptor encephalitis is an immune-mediated condition that has a broad spectrum of manifestations, including seizures, coma, psychosis, and focal neurological deficits. Although usually a diffuse process, unihemispheric involvement mimicking early stages of Rasmussen encephalitis can occur. Rasmussen's encephalitis is a unique syndrome characterized by progressive hemiplegia, drug-resistant focal epilepsy, cognitive decline, and hemispheric brain atrophy contralateral to the hemiplegia...
January 2017: Pediatric Neurology
Ilana Kahn, Guy Helman, Adeline Vanderver, Elizabeth Wells
Anti- N-methyl-d-aspartate receptor (NMDAR) encephalitis has been shown to be a treatable form of autoimmune encephalitis, but there remains no standardized approach to immunotherapy. We designed an anonymous survey sent to members of the Child Neurology Society to identify the current practices among child neurologists. A total of 151 pediatric neurologists responded to the survey. With these responses we were able to highlight areas of practice uniformity, including first-line treatment with intravenous immunoglobulin and intravenous methylprednisone and initiation of disease-modifying therapy with rituximab alone...
February 2017: Journal of Child Neurology
Chikako Kaneko, Norshalena Shakespear, Mario Tuchiya, Jin Kubo, Teiji Yamamoto, Soichi Katayama, Yukitoshi Takahashi
Five consecutive cases of anti-NMDA-receptor encephalitis that we encountered were marked by a rapidly fluctuating level of consciousness associated with psychotic and delirious mental states. Opisthotonus, catatonia, and rhythmic and non-rhythmic involuntary movements of the mouth and jaw were also characteristic features of these particular cases. Serious and potentially fatal problems included epilepsia partialis continua, partial and generalized seizures, and respiratory depression, resembling the symptoms of encephalitis lethargica...
September 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
Andrew McKeon
PURPOSE OF REVIEW: This article describes the methods of diagnosis and management of autoimmune encephalopathies and dementias. The expedited distinction of autoimmune encephalopathies and dementias from neurodegenerative disorders is important because treatment is most effective at the early stage of illness. RECENT FINDINGS: The spectrum of antibody biomarkers of treatable autoimmune encephalopathies continues to broaden and now includes antibodies targeting glutamate receptors (N-methyl-D-aspartate [NMDA] and α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid [AMPA]), γ-aminobutyric acid A and B (GABA-A and GABA-B) receptors, glycine receptors, potassium channel complexes (Kv1, which includes leucine-rich, glioma inactivated 1 [LgI1], contactin-associated proteinlike 2 [CASPR2], and unknown specificity, and Kv4...
April 2016: Continuum: Lifelong Learning in Neurology
Renu Suthar, Arushi Gahlot Saini, Naveen Sankhyan, Jitendra Kumar Sahu, Pratibha Singhi
OBJECTIVES: To study the clinical profile, and outcome of children with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. METHODS: This is a retrospective case series of children <12 y of age, diagnosed with anti-NMDAR encephalitis at a tertiary care institute during the period, May 2013 through June 2015. RESULTS: Twenty patients were tested for suspected anti-NMDAR encephalitis over this 2 y period. Of these, six children were positive for anti-NMDAR antibodies...
July 2016: Indian Journal of Pediatrics
Lídia Hau, Györgyi Csábi, Tamás Tényi
OBJECTIVE: Anti-N-methyl-D-Aspartate encephalitis is a recently diagnosed autoimmune disorder with increasing significance. During this disease antibodies are produced against the subunit of the NMDA receptor, which cause different symptoms, both psychiatric and neurological. The aim of this publication is to introduce this disease, to facilitate the diagnosis and to recommend therapeutical guideline. MATERIALS AND METHODS: In this review we summarized the relevant literature published between 2007 and 2015 giving emphasis on etiopathogenesis, diagnosis, differential diagnosis, treatment and prognosis...
2015: Psychiatria Hungarica: A Magyar Pszichiátriai Társaság Tudományos Folyóirata
Amr A Matoq, Adam S Rappoport, Yiting Yang, Jessica O'Babatunde, Rubina Bakerywala, Raj D Sheth
PURPOSE: Anti-N-methyl-d-aspartate (NMDA) receptor antibody encephalitis is an autoimmune disorder manifesting subacutely with prominent aberrant movements and psychiatric symptoms. The clinical course is one of progressive clinical deterioration that can be halted and often reversed by early diagnosis and treatment. Patterns of presentation and etiology of anti-NMDA-receptor antibody encephalitis are dependent on age and can be challenging to recognize in very young children. REPORTS: Sequential clinical case observations of anti-NMDA-receptor antibody encephalitis presenting in very young children were examined over a year at a single tertiary pediatric institution...
2015: Epilepsy & Behavior Case Reports
Hsiuying Wang
Treatments for anti-N-methyl-D-aspartate (NMDA) receptor encephalitis include immunotherapy with steroids, intravenous immunoglobulin, plasma exchange, or plasmapheresis as first-line treatments, immunotherapy with rituximab or cyclophosphamide as second-line treatments, and tumor removal. In this systematic review, we evaluated previous studies and examined the association between certain microRNAs and anti-NMDA receptor encephalitis to investigate the performance of different treatment combinations. The efficacies of different combinations of treatments classified into the following four categories were compared: (I) intravenous immunoglobulin administration, (II) plasmapheresis or plasma exchange, (III) treatment with rituximab or cyclophosphamide and (IV) tumor removal...
January 1, 2016: Frontiers in Bioscience (Landmark Edition)
Kurt-Wolfram Sühs, Florian Wegner, Thomas Skripuletz, Corinna Trebst, Said Ben Tayeb, Peter Raab, Martin Stangel
Anti-N-methyl D-aspartate (NMDA) receptor encephalitis is the most common type of encephalitis in the spectrum of autoimmune encephalitis defined by antibodies targeting neuronal surface antigens. In the present study, the clinical spectrum of this disease is presented using instructive cases in correlation with the anti-NMDA receptor antibody titers in the cerebrospinal fluid (CSF) and serum. A total of 7 female patients admitted to the hospital of Hannover Medical School (Hannover, Germany) between 2008 and 2014 were diagnosed with anti-NMDA receptor encephalitis...
October 2015: Experimental and Therapeutic Medicine
Arzu Çoban, Gökçen Gündoğdu, Mürüvvet Poyraz, Gülçin Yegen, Aslı Demirtaş-Tatlıdede, Başar Bilgiç, Hasmet A Hanagasi, Erdem Tüzün, Hakan Gürvit
PURPOSE: N-methyl-D-aspartate receptor (NMDAR) encephalitis may present as a paraneoplastic syndrome in young women and is often associated with ovarian teratoma. METHODS: We report 2 male cases of NMDAR encephalitis presenting with metastatic cancer of unknown primary origin. RESULTS: Both patients showed cognitive dysfunction as well as other neurological symptoms, slow waves on EEG, and NMDAR antibodies in sera and CSF. Symptoms were effectively treated by pulse steroid and intravenous immunoglobulin treatment...
November 11, 2016: Tumori
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