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Anti-NMDAR encephalitis

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https://www.readbyqxmd.com/read/27899309/repeated-misdiagnosis-of-a-relapsed-atypical-anti-nmda-receptor-encephalitis-without-an-associated-ovarian-teratoma
#1
Weihe Zhang, Li Yan, Jinsong Jiao
We present an atypical case of relapsed anti-NMDAR encephalitis in a young female patient without an associated ovarian teratoma. She presented with recurrent seizure attacks with muscle weakness, psychosis, dyskinesia, autonomic failure and insomnia. She was first misdiagnosed as mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) then Hashimoto's encephalopathy due to diffuse cerebral lesions, elevated serum lactic acid concentration, increased amount of thyroid peroxidase and thyroglobulin antibodies in serum and diffuse lesions of the thyroid gland...
November 26, 2016: Neuroscience Letters
https://www.readbyqxmd.com/read/27872179/anti-n-methyl-d-aspartate-nmda-receptor-encephalitis-a-survey-of-treatment-progress-and-prospects-from-pediatric-neurologists
#2
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...
November 21, 2016: Journal of Child Neurology
https://www.readbyqxmd.com/read/27854088/long-term-and-strong-immunotherapy-to-treat-anti-n-methyl-d-aspartate-receptor-encephalitis-with-refractory-status-epilepticus
#3
Lan-Hsin Lee, Chien-Jung Lu
BACKGROUND: Anti-N-Methyl-D-Aspartate receptor (anti-NMDAR) encephalitis is responsive to immunotherapy and removal of tumor, but there is no consensus in the treatment of severe anti- NMDAR encephalitis with prolonged refractory status epilepticus (SE). CASE REPORT: A 17-year-old girl presented as acute psychosis, refractory seizures, hyperkinesia, autonomic instability, and soon progressed to a dissociative state of coma. Anti-NMDAR antibodies were positive in serum and CSF...
September 15, 2016: Acta Neurologica Taiwanica
https://www.readbyqxmd.com/read/27844165/myelin-oligodendrocyte-glycoprotein-mog-autoantibodies-as-potential-markers-of-severe-optic-neuritis-and-subclinical-retinal-axonal-degeneration
#4
Joachim Havla, T Kümpfel, R Schinner, M Spadaro, E Schuh, E Meinl, R Hohlfeld, O Outteryck
Antibodies against conformation-dependent epitopes of myelin-oligodendrocyte-glycoprotein (MOG-abs) are present in subgroups of neuromyelitis optica spectrum disorder (NMOSD), recurrent optic neuritis (rON), multiple sclerosis (MS), and anti-NMDAR encephalitis. Using optical coherence tomography (OCT) we assessed whether MOG-abs might serve as potential marker of retinal axonal degeneration. We investigated a clinically heterogeneous cohort of 13 MOG-abs-positive patients (4 MOG-abs-positive rON, 4 MOG-abs-positive adult MS, 3 MOG-abs-positive relapsing encephalomyelitis, 2 MOG-abs-positive aquaporin-4-abs-negative NMOSD)...
November 14, 2016: Journal of Neurology
https://www.readbyqxmd.com/read/27836631/low-serum-vitamin-d-levels-and-anti-n-methyl-d-aspartate-receptor-encephalitis-a-case-control-study
#5
Yaqing Shu, Qingmei Su, Siyuan Liao, Tingting Lu, Rui Li, Xiaobo Sun, Wei Qiu, Yu Yang, Xueqiang Hu, Zhengqi Lu
BACKGROUND: Low vitamin D levels are associated with autoimmunity, but the relationship with anti-N-Methyl-d-aspartate receptor (anti-NMDAR) encephalitis is unknown. METHODS: 25(OH) D levels and clinical and cerebrospinal fluid parameters were evaluated in 30 patients with anti-NMDAR encephalitis and compared with 90 age-, sex-, and season-matched healthy controls. RESULTS: 25(OH)D levels were lower in patients with anti-NMDAR encephalitis compared to controls (43...
November 8, 2016: Neurochemistry International
https://www.readbyqxmd.com/read/27823947/coexisting-neuronal-autoantibodies-among-children-with-demyelinating-syndromes
#6
Hikmet Kıztanır, Gonca Bektaş, Edibe Pembegül Yıldız, Tuğçe Aksu Uzunhan, Burak Tatlı, Nur Aydınlı, Mine Çalışkan, Meral Özmen
OBJECTIVES: To determine the incidence and clinical relevance of neuronal autoantibodies in children with demyelinating syndromes. METHODS: We conducted a prospective study including 31 consecutive children with demyelinating syndromes. Four patients with N-Methyl-D-aspartate receptor (NMDAR) encephalitis, 32 patients with Guillain-Barre syndrome, 13 children with benign childhood epilepsy, and 28 healthy children were used as controls. Prior to initiating immunomodulatory therapy, serum samples were tested for antibodies against NMDAR, α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) 1, AMPAR2, leucine-rich glioma-activated protein 1, contactin-associated protein 2, gamma-aminobutyric acid B receptors, paraneoplastic ma antigen 2 (PNMA2/Ta), Yo, Ri, Hu, CV2, amphiphysin, and aquaporin-4 by indirect immunofluorescence assays...
November 4, 2016: Brain & Development
https://www.readbyqxmd.com/read/27821481/serial-eeg-monitoring-in-a-patient-with-anti-nmda-receptor-encephalitis
#7
Yanfang Shi
Electroencephalogram (EEG) abnormalities are very common in anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis. Extreme delta brush (EDB) is a distinctive EEG pattern that is can be suggestive of the diagnosis; however, the etiology of the EDB remains unclear. Furthermore, there is question with regard to its ictal or interictal nature. We report a 20-year-old woman with anti-NMDAR encephalitis whose serial video-EEG monitoring was obtained at 2, 2.5, 4, and 6 months after admission. There was a long-standing EDB lasting up to several hours, with no evolution in frequency, amplitude, or morphology, and without clear association her frequent orofacial dyskinesia...
November 7, 2016: Clinical EEG and Neuroscience: Official Journal of the EEG and Clinical Neuroscience Society (ENCS)
https://www.readbyqxmd.com/read/27778112/anti-n-methyl-d-aspartate-receptor-encephalitis-associated-with-intracranial-angiostrongylus-cantonensis-infection-a-case-report
#8
Yu Peng, Xiaojia Liu, Suyue Pan, Zuoshan Xie, Honghao Wang
Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a recently described paraneoplastic syndrome with prominent neuropsychiatric symptoms. Many of these cases are associated with neoplasma especially teratoma. In addition, a few of cases with anti-NMDAR antibodies triggered by viral infection have been reported, but never by parasitic infection. Here, we report a novel case of NMDA receptor encephalitis in a 51-year-old male related to the development of anti-NMDAR antibodies triggered by Angiostrongylus cantonensis infection...
October 24, 2016: Neurological Sciences
https://www.readbyqxmd.com/read/27762163/spontaneous-recovery-of-memory-functions-in-an-untreated-case-of-anti-nmdar-encephalitis-a-reason-to-maintain-hope
#9
Katherine McIvor, Perry Moore
OBJECTIVE: Anti N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is an autoimmune disorder that was only fully discovered recently and neuropsychological outcome data remains sparse. We present the case of BA, a 19-year-old male, which illustrates the cognitive outcome in an untreated case over a time period of over 2½ years. METHOD: We conducted three cognitive assessments, including tests of memory and executive functioning, over this time period and considered the evidence for reliable change in memory function using the Wechsler Advanced Clinical Solutions (ACS) serial assessment package...
October 20, 2016: Clinical Neuropsychologist
https://www.readbyqxmd.com/read/27667486/-anti-nmda-receptor-antibody-related-encephalitis
#10
Shigemi Nagayama, Keiko Tanaka
Recently, the search for diagnostic antibody markers has drawn considerable attention in relation to autoimmune encephalitis. Among the antibody markers, the most frequently detected is the anti-N-methyl-D-aspartate receptor (NMDAR)antibody. Patients with this antibody develop characteristic clinical features. This disease tends to affect young women, and starts with psychiatric symptoms followed by seizures, involuntary movements, autonomic failure, and respiratory failure. Nearly half of these female patients have ovarian teratoma...
September 2016: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/27652003/long-term-poor-rapport-lack-of-spontaneity-and-passive-social-withdrawal-related-to-acute-post-infectious-encephalitis-a-case-report
#11
Atsurou Yamada, Nobuhiro Miyachi, Toshiyasu Miura, Masako Suzuki, Norio Watanabe, Tatsuo Akechi
INTRODUCTION: Post-infectious encephalitis/encephalopathy is a neurological syndrome that sometimes develops following common viral or bacterial infections. The most common form is acute disseminated encephalomyelitis (ADEM). ADEM is a demyelinating disease of the central nervous system that typically presents as a monophasic disorder associated with multifocal neurologic symptoms and encephalitis. Anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis is another type of severe autoimmune disorder, characterized by seizures, movement disorders and psychiatric symptoms...
2016: SpringerPlus
https://www.readbyqxmd.com/read/27632180/clinical-characteristics-and-outcomes-between-children-and-adults-with-anti-n-methyl-d-aspartate-receptor-encephalitis
#12
Qi Huang, Yuan Wu, Rongfa Qin, Xing Wei, Meigang Ma
Anti-N-Methyl-D-Aspartate receptor (NMDAR) encephalitis is an acute neurological disorder affecting children and adults. We aimed to compare the clinical characteristics, treatments, and outcomes between children and adults with anti-NMDAR encephalitis and to assess the probable risk factors. In this observational study, patients who tested positive for anti-NMDAR antibody in the cerebrospinal fluid were enrolled. The patients were divided into children and adults group on the basis of age (whether <16 or not)...
December 2016: Journal of Neurology
https://www.readbyqxmd.com/read/27631202/variations-of-movement-disorders-in-anti-n-methyl-d-aspartate-receptor-encephalitis-a-nationwide-study-in-taiwan
#13
Bi-Chun Duan, Wen-Chin Weng, Kuang-Lin Lin, Lee Chin Wong, Sung-Tse Li, Mei-Hsin Hsu, Jainn-Jim Lin, Pi-Chaun Fan, Ming-I Lin, Nan-Chang Chiu, Yu-Ching Lin, Huei-Shyong Wang, Kun-Long Hung, Wang-Tso Lee
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is one of the most common autoimmune encephalitis that presents with a wide variety of movement disorders. The purpose of our study is to review the manifestations and duration of movement disorders in different ages with NMDAR encephalitis.A retrospective cohort of 28 patients (20 females and 8 males) with positive cerebrospinal fluid (CSF) anti-NMDAR antibody in a 5-year period from major hospitals in Taiwan was enrolled. They were categorized into 3 age groups: 7 patients were ≤10 years, 14 patients were 10 to 18 years, and 7 patients were >18 years...
September 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27620725/anti-n-methyl-d-aspartate-receptor-encephalitis-a-common-cause-of-encephalitis-in-the-intensive-care-unit
#14
Xueping Chen, Jin-Mei Li, Fan Liu, Qiong Wang, Dong Zhou, Xiaohui Lai
Anti-N-methyl-D-aspartate receptor encephalitis (anti-NMDAR encephalitis) is the most common type of immune-mediated encephalitis. This study aimed to assess the incidence and mortality of anti-NMDAR encephalitis in intensive care unit (ICU) to evaluate the clinical manifestations, laboratory findings, managements and outcomes, and to compare these characteristics with patients with non-anti-NMDAR encephalitis admitted to ICU. Patients admitted to the neurological ICU with suspected encephalitis were included between January 1, 2012 and July 31, 2015...
December 2016: Neurological Sciences
https://www.readbyqxmd.com/read/27609625/prevalence-of-serum-anti-neuronal-autoantibodies-in-patients-admitted-to-acute-psychiatric-care
#15
M Schou, S G Sæther, K Borowski, B Teegen, D Kondziella, W Stoecker, A Vaaler, S K Reitan
BACKGROUND: Autoimmune encephalitis associated with anti-neuronal antibodies may be challenging to distinguish from primary psychiatric disorders. The significance of anti-neuronal antibodies in psychiatric patients without clear evidence of autoimmune encephalitis is unknown. We investigated the serum prevalence of six anti-neuronal autoantibodies in a cohort of unselected patients admitted to acute psychiatric care. METHOD: Serum was drawn from 925 patients admitted to acute psychiatric in-patient care...
December 2016: Psychological Medicine
https://www.readbyqxmd.com/read/27609293/frequent-rhabdomyolysis-in-anti-nmda-receptor-encephalitis
#16
Jung-Ah Lim, Soon-Tae Lee, Tae-Joon Kim, Jangsup Moon, Jun-Sang Sunwoo, Jung-Ick Byun, Keun-Hwa Jung, Ki-Young Jung, Kon Chu, Sang Kun Lee
The aim of this study was to analyze the clinical presentation and provocation factors of rhabdomyolysis in anti-NMDAR encephalitis. Among the 16 patients with anti-NMDAR encephalitis in our institutional cohort, nine patients had elevated CK enzyme levels and clinical evidence of rhabdomyolysis. Rhabdomyolysis was more frequent after immunotherapy. The use of dopamine receptor blocker (DRB) increased the risk of rhabdomyolysis. None of the patients without rhabdomyolysis received DRBs. Rhabdomyolysis is a frequent complication in anti-NMDAR encephalitis and more common after immunotherapy and the use of DRBs increases the risk...
September 15, 2016: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/27606355/neuroleptic-intolerance-in-patients-with-anti-nmdar-encephalitis
#17
Florian Lejuste, Laure Thomas, Géraldine Picard, Virginie Desestret, François Ducray, Veronique Rogemond, Dimitri Psimaras, Jean-Christophe Antoine, Jean-Yves Delattre, Laurent Groc, Marion Leboyer, Jerome Honnorat
OBJECTIVE: To precisely describe the initial psychiatric presentation of patients with anti-NMDA receptor (NMDAR) antibodies encephalitis (anti-NMDAR encephalitis) to identify potential clues enhancing its early diagnosis. METHODS: We retrospectively studied the French Reference Centre medical records of every adult patient with anti-NMDAR encephalitis to specify the patients' initial psychiatric symptoms leading to hospitalization in a psychiatric department and the reasons underlying the diagnosis of anti-NMDAR encephalitis...
October 2016: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/27575749/utility-of-csf-cytokine-chemokines-as-markers-of-active-intrathecal-inflammation-comparison-of-demyelinating-anti-nmdar-and-enteroviral-encephalitis
#18
Kavitha Kothur, Louise Wienholt, Shekeeb S Mohammad, Esther M Tantsis, Sekhar Pillai, Philip N Britton, Cheryl A Jones, Rajeshwar R Angiti, Elizabeth H Barnes, Timothy Schlub, Sushil Bandodkar, Fabienne Brilot, Russell C Dale
BACKGROUND: Despite the discovery of CSF and serum diagnostic autoantibodies in autoimmune encephalitis, there are still very limited CSF biomarkers for diagnostic and monitoring purposes in children with inflammatory or autoimmune brain disease. The cause of encephalitis is unknown in up to a third of encephalitis cohorts, and it is important to differentiate infective from autoimmune encephalitis given the therapeutic implications. AIM: To study CSF cytokines and chemokines as diagnostic biomarkers of active neuroinflammation, and assess their role in differentiating demyelinating, autoimmune, and viral encephalitis...
2016: PloS One
https://www.readbyqxmd.com/read/27574610/electroconvulsive-therapy-and-or-plasmapheresis-in-autoimmune-encephalitis
#19
Jessica L Gough, Jan Coebergh, Brunda Chandra, Ramin Nilforooshan
Autoimmune encephalitis is a poorly understood condition that can present with a combination of neurological and psychiatric symptoms, either of which may predominate. There are many autoantibodies associated with a variety of clinical syndromes - anti-N-Methyl-D-Aspartate receptor (NMDAR) is the commonest. Currently, the most widely used therapy is prompt plasmapheresis and steroid treatment (and tumour resection if indicated), followed by second line immunosuppression if this fails. Given the growing awareness of autoimmune encephalitis as an entity, it is increasingly important that we consider it as a potential diagnosis in order to provide timely, effective treatment...
August 16, 2016: World Journal of Clinical Cases
https://www.readbyqxmd.com/read/27552490/anti-n-methyl-d-aspartate-receptor-encephalitis-in-adult-patients-requiring-intensive-care
#20
Étienne de Montmollin, Sophie Demeret, Noëlle Brulé, Marie Conrad, Frédéric Dailler, Nicolas Lerolle, Jean-Christophe Navellou, Carole Schwebel, Mikaël Alves, Martin Cour, Nicolas Engrand, Jean-Marie Tonnelier, Eric Maury, Stéphane Ruckly, Géraldine Picard, Véronique Rogemond, Éric Magalhaes, Tarek Sharshar, Jean-François Timsit, Jérôme Honnorat, Romain Sonneville
RATIONALE: Encephalitis due to anti-N-methyl-D-aspartate receptor (NMDAR) antibodies is the leading cause of immune-mediated encephalitis. There is limited data on intensive care unit (ICU) management of these patients. OBJECTIVES: To identify prognostic factors of good neurologic outcome in patients admitted to an ICU with anti-NMDAR encephalitis. METHODS: Observational multicenter study of all consecutive adult patients diagnosed with anti-NMDAR encephalitis, admitted to an ICU between 2008 and 2014...
August 23, 2016: American Journal of Respiratory and Critical Care Medicine
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