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NMDA encephalitis

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https://www.readbyqxmd.com/read/29310866/initial-clinical-presentation-of-young-children-with-n-methyl-d-aspartate-receptor-encephalitis
#1
Marion Favier, Bastien Joubert, Géraldine Picard, Véronique Rogemond, Laure Thomas, Sylvain Rheims, Marion Bailhache, Frédéric Villega, Jean-Michel Pédespan, Giulia Berzero, Dimitri Psimaras, Jean-Christophe Antoine, Virginie Desestret, Jérôme Honnorat
Autoimmune encephalitis with anti-N-methyl-d-aspartate receptor autoantibodies (NMDA-R-Abs) is a recently described disease affecting adult and pediatric patients. Symptoms of the disease are now perfectly described in the adult population but the clinical presentation is less known in young children. The aim of the present study was to describe the clinical presentation and the specificities of symptoms presented by young children with NMDA-R-Abs encephalitis to improve diagnosis of this disease, and to compare these to a series of previously published female adult patients...
December 28, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29277332/cytokine-chemokine-elevation-during-the-transition-phase-from-hsv-encephalitis-to-autoimmune-anti-nmda-receptor-encephalitis
#2
Takanori Omae, Yoshiaki Saito, Hirokazu Tsuchie, Koyo Ohno, Yoshihiro Maegaki, Hiroshi Sakuma
A 3-year-old girl suffered from anti-N-methyl-d-aspartate (anti-NMDA) receptor encephalitis after resolution of herpes simplex virus encephalitis (HSE). Methylprednisolone pulse and immunoglobulin therapies showed little effect, but the patient completely recovered after six courses of monthly cyclophosphamide pulse therapy and successive maintenance on mycophenolate mofetil for one year. Anti-NMDA receptor antibody in the cerebrospinal fluid (CSF) was minimally detected during the prodromal febrile period and then was seen to be markedly elevated at the onset of second encephalopathy phase...
December 22, 2017: Brain & Development
https://www.readbyqxmd.com/read/29258969/a-case-of-anti-nmda-receptor-encephalitis-revealed-by-insular-epilepsy
#3
Charlotte De Maeseneire, Riem El Tahry, Susana Ferrao Santos
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder of the central nervous system that typically manifests predominantly as a psychiatric disorder. However, other manifestations such as epileptic seizures, abnormal movements, and memory or language complications are not unusual. Here, we report the case of a young man who presented with a new-onset epilepsy, with ictal semiology suggestive of insular involvement; this hypothesis was supported by a PET-CT study. Anti-NMDAR antibodies were found in the CSF, confirming the diagnosis of anti-NMDAR encephalitis...
December 20, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29226117/pediatric-anti-n-methyl-d-aspartate-receptor-encephalitis-a-review-with-pooled-analysis-and-critical-care-emphasis
#4
REVIEW
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
https://www.readbyqxmd.com/read/29200116/paraneoplastic-disorders
#5
Eric Lancaster
PURPOSE OF REVIEW: Paraneoplastic neurologic syndromes target specific areas of the nervous system with pathogenic autoantibodies or T-cell responses. Each syndrome conveys a risk of particular tumors. Expanded paraneoplastic antibody testing has led to improved diagnosis but created challenges involving appropriate interpretation of test results. RECENT FINDINGS: Peripheral nervous system paraneoplastic disorders such as myasthenia gravis and Lambert-Eaton myasthenic syndrome involve pathogenic autoantibodies...
December 2017: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/29198411/epileptic-spasms-secondary-to-acute-cerebral-and-cerebellar-encephalitis
#6
Tohru Okanishi, Ayataka Fujimoto, Risa Hashimoto, Mitsuyo Nishimura, Sotaro Kanai, Miho Ogawa, Takayuki Suzuki, Hirotaka Motoi, Yukitoshi Takahashi, Hideo Enoki
BACKGROUND: Patients with infection-related acute encephalitis sometimes develop epilepsy in the chronic phase of the disease. Patients with postencephalitic epilepsy usually develop partial seizures due to the lesions generated by the encephalitis. We report a case who developed late-onset epileptic spasms after acute cerebral and cerebellar encephalitis. CASE REPORT: A 5-year-old girl showed severe tremor, gait ataxia, partial or generalized tonic-clonic seizures, hyperactivity, and panic attacks after a mild enterocolitis...
November 29, 2017: Brain & Development
https://www.readbyqxmd.com/read/29183618/encephalitis-associated-with-antibodies-against-the-nmda-receptor
#7
REVIEW
Mar Guasp, Josep Dalmau
The encephalitis associated with antibodies against the N-methyl-D-aspartate receptor (NMDAR) is characterized by the presence of antibodies against the GluN1 subunit of this receptor, resulting in symptoms that are similar to those observed in models of genetic or pharmacologic reduction of NMDARs. Patients are usually young adults, predominantly women, and children who develop, in a sequential manner, rapidly progressive symptoms including psychosis, abnormal movements, autonomic dysfunction, and coma. Epileptic seizures are variable and can occur throughout the course of the disease...
November 25, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/29160207/serial-eeg-findings-in-anti-nmda-receptor-encephalitis-correlation-between-clinical-course-and-eeg
#8
Jun Ueda, Michi Kawamoto, Ryota Hikiami, Junko Ishii, Hajime Yoshimura, Riki Matsumoto, Nobuo Kohara
Anti-NMDA receptor encephalitis is a paraneoplastic encephalitis characterised by psychiatric features, involuntary movement, and autonomic instability. Various EEG findings in patients with anti-NMDA receptor encephalitis have been reported, however, the correlation between the EEG findings and clinical course of anti-NMDA receptor encephalitis remains unclear. We describe a patient with anti-NMDA receptor encephalitis with a focus on EEG findings, which included: status epilepticus, generalised rhythmic delta activity, excess beta activity, extreme delta brush, and paroxysmal alpha activity upon arousal from sleep, which we term"arousal alpha pattern"...
November 21, 2017: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/29159205/decreased-occipital-lobe-metabolism-by-fdg-pet-ct-an-anti-nmda-receptor-encephalitis-biomarker
#9
John C Probasco, Lilja Solnes, Abhinav Nalluri, Jesse Cohen, Krystyna M Jones, Elcin Zan, Mehrbod S Javadi, Arun Venkatesan
Objective: To compare brain metabolism patterns on fluorodeoxyglucose (FDG)-PET/CT in anti-NMDA receptor and other definite autoimmune encephalitis (AE) and to assess how these patterns differ between anti-NMDA receptor neurologic disability groups. Methods: Retrospective review of clinical data and initial dedicated brain FDG-PET/CT studies for neurology inpatients with definite AE, per published consensus criteria, treated at a single academic medical center over a 10-year period...
January 2018: Neurology® Neuroimmunology & Neuroinflammation
https://www.readbyqxmd.com/read/29159196/botulinum-toxin-treatment-for-hypersalivation-in-anti-nmda-receptor-encephalitis
#10
Jin-Sun Jun, Han Gil Seo, Soon-Tae Lee, Kon Chu, Sang Kun Lee
Hypersalivation is one of the intractable symptoms of anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis. While anticholinergic medications partially improve the hypersalivation, they can aggravate the autonomic dysfunctions associated with anti-NMDAR encephalitis. Thus, we investigated the efficacy and safety of botulinum toxin type A injection on hypersalivation refractory to anticholinergics in six patients with anti-NMDAR encephalitis. Hypersalivation was well-controlled without remarkable adverse reaction over 16 weeks after botulinum toxin type A, although two patients were reinjected at 12 weeks due to reaggravation of hypersalivation...
November 2017: Annals of Clinical and Translational Neurology
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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: Journal of the Intensive Care Society
https://www.readbyqxmd.com/read/28974594/an-unusual-case-of-refractory-status-epilepticus-in-a-young-lady-anti-nmda-receptor-encephalitis
#19
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
#20
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
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