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NMDA encephalitis

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https://www.readbyqxmd.com/read/29141829/cerebral-cortical-encephalitis-followed-by-recurrent-cns-demyelination-in-a-patient-with-concomitant-anti-mog-and-anti-nmda-receptor-antibodies
#1
Lei Zhou, Jingzi ZhangBao, Haiqing Li, Xiaoyang Li, Yongheng Huang, Min Wang, Chongbo Zhao, Jiahong Lu, Chuanzhen Lu, Yuxin Li, Chao Quan
We report the case of a patient who initially presented with fever, headache and seizure. MRI revealed a fluid attenuation inversion recovery (FLAIR) high-intensity lesion involving the right temporal, parietal and occipital cortex. Afterwards, the patient developed three recurrent episodes, manifested as brainstem encephalitis, optic neuritis and ADEM-like illness successively, indicating demyelination. Both of his serum anti-MOG and CSF anti-NMDAR antibodies were proved positive by transfected cell based assays...
November 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/29103424/autoantibody-associated-movement-disorders-in-children-proven-and-proposed
#2
Harvey S Singer
Movement disorders secondary to autoantibodies in children represent a rapidly expanding group of conditions. Once considered to be limited to poststreptococcal Sydenham's chorea or rare cases of childhood systemic lupus erythematosus, a variety of antibody-related movement abnormalities are now seen as part of noninfectious autoimmune encephalitis or within an expanding list of postinfectious disorders. In this article, several proposed autoantibody-mediated movement disorders in children are reviewed. In each one, there is a hypothesized antibody biomarker that is believed to be pathogenic and cause the clinical symptoms...
August 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/29101253/superficial-white-matter-damage-in-anti-nmda-receptor-encephalitis
#3
Owen Robert Phillips, Shantanu H Joshi, Katherine L Narr, David W Shattuck, Manpreet Singh, Margherita Di Paola, Christoph J Ploner, Harald Prüss, Paul Friedemann, Carsten Finke
BACKGROUND: Clinical brain MRI is normal in the majority of patients with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. However, extensive deep white matter damage wasrecently identifiedin these patients using diffusion weighted imaging. Here, our aim was to study a particularly vulnerable brain compartment, the late myelinating superficial white matter. METHODS: Forty-six patients with anti-NMDAR encephalitis were included. Ten out of these were considered neurologically recovered (modified Rankin scale of zero), while 36 patients were non-recovered...
November 3, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/29077387/anti-nmda-receptor-encephalitis-from-bench-to-clinic
#4
Arun Venkatesan, Krishma Adatia
NMDAR encephalitis is a common cause of autoimmune encephalitis, predominantly affecting young adults. Current data supports the idea that autoantibodies targeting NMDARs are responsible for disease pathogenesis. While these autoantibodies occur in the setting of underlying malignancy in approximately half of all patients, initiating factors for the autoimmune response in the remainder of patients are unclear. While there is increasing evidence supporting viral triggers such as herpes simplex encephalitis, this association and the mechanism of action have not yet been fully described...
November 7, 2017: ACS Chemical Neuroscience
https://www.readbyqxmd.com/read/29075658/improving-the-antibody-based-evaluation-of-autoimmune-encephalitis
#5
Lindsey McCracken, Junxian Zhang, Maxwell Greene, Anne Crivaro, Joyce Gonzalez, Malek Kamoun, Eric Lancaster
OBJECTIVE: We tested whether antibody screening samples of patients with suspected autoimmune encephalitis with additional research assays would improve the detection of autoimmune encephalitis compared with standard clinical testing alone. METHODS: We examined 731 samples (333 CSF, 182 sera, and 108 pairs) from a cohort of 623 patients who were tested for CNS autoantibodies by the University of Pennsylvania clinical laboratory over a 24-month period with cell-based assays (CBAs) on commercially obtained slides of fixed cells for antibodies to NMDA receptor (NMDAR), α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), γ-aminobutyric acid-B receptor (GABABR), leucine-rich glioma-inactivated 1 (LGI1), contactin-associated protein-like 2 (Caspr2), and glutamic acid decarboxylase (GAD65)...
November 2017: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/29050531/anti-nmda-receptor-encephalitis-still-unknown-and-underdiagnosed-by-physicians-and-especially-by-psychiatrists
#6
Tine Hermans, Patrick Santens, Celine Matton, Kristine Oostra, Gunter Heylens, Sarah Herremans, Gilbert M D Lemmens
Anti-NMDA receptor encephalitis is an autoimmune disorder confirmed by the presence of antibodies against the NMDA-receptor in serum or CSF. This case report describes a young woman with anti-NMDA receptor encephalitis, who presented with prominent psychiatric symptoms. There was a crucial delay in diagnosis and necessary treatment due to the fact that the clinical presentation was diagnosed and treated as a first psychotic episode. Physicians and especially psychiatrists, should consider the possibility of an autoimmune encephalitis in their differential diagnosis in every new onset psychotic episode with rapid progression, the presence of pathognomonic orofacial dyskinesia, the lack of psychiatric history, and the non-responding to psychopharmacological treatment...
October 19, 2017: Acta Clinica Belgica
https://www.readbyqxmd.com/read/29040795/case-presentation-of-anti-nmda-receptor-encephalitis-in-a-4-year-old-boy
#7
Daniel A Gbadero, Emmanuel O Adegbite, Jean-Baptist LePichon, Tina M Slusher
We report a case of a 4-year-old boy from Oyo, Nigeria, presenting with prolonged seizures and coma with the subsequent development of oro-lingual-facial dyskinesia with frequent tongue thrusting, dysconjugate gaze and choreoathetoid movements of the limbs because of autoimmune encephalitis consistent with anti-N-methyl-D-aspartate (anti-NMDAR) encephalitis.
October 6, 2017: Journal of Tropical Pediatrics
https://www.readbyqxmd.com/read/28979520/status-epilepticus-and-anti-nmda-receptor-encephalitis-after-resection-of-an-ovarian-teratoma
#8
Amritpal S Jandu, Peter M Odor, Steven D Vidgeon
Anti-N-methyl-D-aspartate receptor encephalitis is a recently recognised autoimmune, paraneoplastic syndrome that typically presents with psychiatric disturbance, reduced conscious level and seizures. The disorder has been previously associated with ovarian teratomas. We present the case of a 35-year-old female, with a previous surgical history for resection of an ovarian teratoma, who later developed status epilepticus and anti-N-methyl-D-aspartate receptor encephalitis requiring intensive care management...
November 2016: J Intensive Care Soc
https://www.readbyqxmd.com/read/28974594/an-unusual-case-of-refractory-status-epilepticus-in-a-young-lady-anti-nmda-receptor-encephalitis
#9
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
https://www.readbyqxmd.com/read/28959704/cryptogenic-norse-its-distinctive-clinical-features-and-response-to-immunotherapy
#10
Takahiro Iizuka, Naomi Kanazawa, Juntaro Kaneko, Naomi Tominaga, Yutaka Nonoda, Atsuko Hara, Yuya Onozawa, Hiroki Asari, Takashi Hata, Junya Kaneko, Kenji Yoshida, Yoshihiro Sugiura, Yoshikazu Ugawa, Masashi Watanabe, Hitomi Tomita, Arifumi Kosakai, Atsushi Kaneko, Daisuke Ishima, Eiji Kitamura, Kazutoshi Nishiyama
OBJECTIVE: To report the distinctive clinical features of cryptogenic new-onset refractory status epilepticus (C-NORSE) and the C-NORSE score based on initial clinical assessments. METHODS: A retrospective study was conducted for 136 patients with clinically suspected autoimmune encephalitis who underwent testing for autoantibodies to neuronal surface antigens between January 1, 2007, and August 31, 2016. Eleven patients with C-NORSE were identified. Their clinical features were compared with those of 32 patients with anti-NMDA receptor encephalitis (NMDARE)...
November 2017: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/28948076/peripheral-neuropathy-in-limbic-encephalitis-with-anti-glutamate-receptor-antibodies-case-report-and-systematic-literature-review
#11
Yi-Chia Wei, Chin-Chang Huang, Chi-Hung Liu, Hung-Chou Kuo, Jainn-Jim Lin
INTRODUCTION: Autoantibodies to the alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor and N-methyl-d-aspartate (NMDA) receptor are known to be the causes of autoimmune encephalitis particularly limbic encephalitis. The involvement of the peripheral nervous system is rarely reported. METHODS: We analyzed the serial nerve conduction studies of a previously reported case of anti-AMPA receptor encephalitis, who was presented with conscious disturbance and quadriplegia...
September 2017: Brain and Behavior
https://www.readbyqxmd.com/read/28932896/-update-on-anti-n-methyl-d-aspartate-receptor-encephalitis
#12
S Kovac, J Alferink, D Ahmetspahic, V Arolt, N Melzer
Autoimmune encephalitis is a group of autoimmune inflammatory disorders affecting both grey and white matter of the central nervous system. Encephalitis with autoantibodies against the N‑methyl-D-aspartate receptor (NMDA-R) is the most frequent autoimmune encephalitis syndrome presenting with a characteristic sequence of psychiatric and neurological symptoms. Treatment necessitates a close interdisciplinary cooperation. This article provides an update on the current knowledge on diagnostic standards, pathogenesis, and treatment strategies for anti-NMDA-R encephalitis from psychiatric and neurological perspectives...
September 20, 2017: Der Nervenarzt
https://www.readbyqxmd.com/read/28930529/a-protocol-for-investigating-the-association-of-vaccination-and-anti-nmda-receptor-encephalitis
#13
Hsiuying Wang
Anti-N-methyl-D-aspartate (Anti-NMDA) receptor encephalitis is an acute autoimmune neurological disorder that can be triggered by virus, H1N1/tetanus/diphtheria/pertussis and polio vaccines or by presence of a tumor. The association between anti-NMDA receptor encephalitis and Japanese encephalitis vaccination was examined and a general protocol of phylogenetic method which details the steps and code and an example of its utility is provided. The approach used here is potentially useful for analyzing the relationship between vaccines and diseases...
January 1, 2018: Frontiers in Bioscience (Scholar Edition)
https://www.readbyqxmd.com/read/28905135/a-case-of-reversible-anti-nmda-receptor-encephalitis-neuropsychological-and-neuroradiological-features
#14
Carlotta Mutti, Federica Barocco, Lucia Zinno, Anna Negrotti, Marco Spallazzi, Giovanni Pavesi, Simona Gardini, Paolo Caffarra
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is an autoimmune encephalitis mainly affecting young women. We report a case of a mild paraneoplastic anti-NMDAR encephalitis in a 31-year-old female with an ovarian immature teratoma. The patient exhibited a severe short-term episodic memory impairment and psychiatric symptoms. A detailed diagnostic work-up including complete clinical and laboratory examinations, neuropsychological assessments, and neuroradiological investigations has been done at the onset and during follow-up...
September 13, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28899640/status-epilepticus-in-pregnancy-etiology-management-and-clinical-outcomes
#15
Keni Ravish Rajiv, Ashalatha Radhakrishnan
BACKGROUND: Status epilepticus (SE) in pregnancy carries significant risk to both mother and fetus. There is limited literature available on SE occurring in pregnancy world-over, with majority being from obstetric centers. METHODS: All women who developed SE related to pregnancy (gestation, labor, or puerperium) between January 2000 and December 2016 were included in the study. Data were collected from our SE registry, maintained, and archived in the institute. The variables influencing the maternal and fetal outcome were compared using Student's t-test for continuous variables and Fisher's exact test for discrete variables...
September 9, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28886955/successful-combined-targeting-of-b-and-plasma-cells-in-treatment-refractory-anti-nmdar-encephalitis
#16
Olafur Sveinsson, Mathias Granqvist, Yngve Forslin, Kaj Blennow, Henrik Zetterberg, Fredrik Piehl
We describe an extremely severe case of therapy refractory NMDA receptor encephalitis (NMDAe) in a 26-year-old woman. After rituximab, bilateral oophorectomy, repeated cycles of high dose methylprednisolone and plasma exchange, she received repeated cyclophosphamide, tocilizumab (interleukin-6 inhibitor) and finally bortezomib (plasma cell depleting drug) leading to remission after 204days in intensive care. Two years after disease onset her cognitive functions are still affected, but slowly improving and the cerebral atrophy has been partly reversed...
November 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28882707/functional-connectivity-of-large-scale-brain-networks-in-patients-with-anti-nmda-receptor-encephalitis-an-observational-study
#17
Michael Peer, Harald Prüss, Inbal Ben-Dayan, Friedemann Paul, Shahar Arzy, Carsten Finke
BACKGROUND: In anti-NMDA receptor (NMDAR) encephalitis, antibody-mediated dysfunction of NMDARs causes severe neuropsychiatric symptoms, including psychosis, memory deficits, and movement disorders. However, it remains elusive how antibody-mediated NMDAR dysfunction leads to these symptoms, and whether the symptoms arise from impairment in specific brain regions and the interactions between impaired regions. METHODS: In this observational study, we recruited 43 patients with anti-NMDAR encephalitis from a tertiary university hospital and 43 age-matched and sex-matched healthy controls without a history of neurological or psychiatric disorders, who were recruited from the general population of Berlin...
October 2017: Lancet Psychiatry
https://www.readbyqxmd.com/read/28853520/-anti-n-methyl-d-aspartate-nmda-encephalitis-the-israeli-pediatric-multi-center-experience
#18
Keren Politi, Dafna Marom, Shay Ashkenazi, Gilat Livni, Hadassa Goldberg, Avinoam Shuper, Tami Steinberg, Eli Lahat, Eli Haiman, Rachel Shtrausberg
Anti-N-methyl-D-aspartate (NMDA) encephalitis is a disorder characterized by acute neuro-psychiatric symptoms, appearing mostly after a recent febrile disease, with a gradual progressive course, associated with laboratory or radiologic evidence of active inflammation. Many of the patients will present with a continuous neuro-cognitive disorder which could lead to major morbidity and even mortality. It was recently reported that this disorder can present at childhood as a primary disease or as a secondary complication of herpes simplex infection...
August 2017: Harefuah
https://www.readbyqxmd.com/read/28841275/neuropsychiatric-manifestations-of-pediatric-nmda-receptor-autoimmune-encephalitis-a-case-series-from-a-tertiary-care-center-in-india
#19
Salah Basheer, Madhu Nagappa, Anita Mahadevan, Parayil Sankaran Bindu, Arun B Taly, Satish Chandra Girimaji
Objective: Although psychiatric manifestations are one of the most common presentations of pediatric N-methyl-D-aspartate receptor (NMDAR) encephalitis, there is a lack of studies that characterize psychiatric aspects of this disorder. This study was designed to address this gap. Methods: Initial clinical presentations including psychiatric symptoms, treatment details, and outcome with respect to psychiatric symptoms were collected from medical case records of children aged less than 18 years with seropositive NMDAR encephalitis from a single tertiary care center (May 2010-November 2016)...
August 17, 2017: Primary Care Companion to CNS Disorders
https://www.readbyqxmd.com/read/28829986/neurobiology-of-autoimmune-encephalitis
#20
REVIEW
Masaki Fukata, Norihiko Yokoi, Yuko Fukata
Autoimmune encephalitis presenting with amnesia, seizures, and psychosis is highly topical in basic and clinical neuroscience. Recent studies have identified numerous associated autoantibodies, targeting cell-surface synaptic proteins including neurotransmitter receptors (e.g. NMDA receptors (NMDARs)) and a secreted protein, LGI1. In vitro and in vivo analyses of the influence of the autoantibodies have begun to clarify their causal roles. Of particular interest is the generation of recombinant monoclonal antibodies from patients' B cells with anti-NMDAR encephalitis...
August 19, 2017: Current Opinion in Neurobiology
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