The Anti-NMDA Receptor Encephalitis Foundation Newsletter

To evaluate the frequency of anti-NMDAR encephalitis in a secondary mental health service and investigate the challenges of its diagnosis in routine clinical practice. Patients whose electronic health records registered an indication for NMDAR-IgG assessment were selected and seropositive patients were reviewed. In 1661 patients assessed for NMDAR-IgG over 12 years, the positivity rate was 3.79% (95% confidence interval [CI]: 2.87%-4.70%). The working diagnosis at assessment was new onset psychosis in 38.7% and a chronic psychotic syndrome in 34.0%. Among seropositive patients, 30 (47.6%, 95%CI: 35.8% – 59.7%) had a final alternative diagnosis different from encephalitis after a median period of 49 months from onset. Patients with a final diagnosis of encephalitis were more frequently female (27/35 vs 13/30, p= 0.011) than other seropositive patients and had more frequently an acute (34/35 vs 11/30, p < 0.001), fluctuating (21/23 vs 4/27, p < 0.001) or agitated (32/32 vs 10/26, p < 0.001) presentation. Nine encephalitic patients received specialized follow-up for chronic neuropsychiatric problems including learning disabilities, organic personality disorder, anxiety, fatigue, obsessive-compulsive and autism-like disorder. In a psychiatric setting, NMDAR-IgG seropositivity rates were low with a positive predictive value for encephalitis around 50% when screened patients had chronic presentations and absence of other diagnostic criteria for encephalitis or psychosis of autoimmune origin. Chronic neuropsychiatric problems in anti-NMDAR encephalitis are not uncommon, so better diagnostic and treatment strategies are still needed. Copyright © 2021. Published by Elsevier Inc. For latest news and updates By signing up, you will receive emails about Physician’s Weekly products and you agree to our Terms & Conditions and Privacy Policy.

 







Autoimmune anti-NMDAr encephalitis is an antibody-mediated disorder characterized by psychiatric symptoms followed by decreased consciousness, dysauto……

 




Anti-N-methyl-d-aspartate receptor encephalitis (ANMDARE) is a neurologic disorder that is becoming more recognized in children and adolescents. Early studies suggested that patients typically reco…

 




The results of our prospective study show that autoimmune encephalitis occurred in
27% of patients with herpes simplex encephalitis. It was associated with development of neuronal antibodies and usually presented within 2 months after treatment of herpes simplex encephalitis; the symptoms were…

 




A dysfunction of the glutamatergic transmission, especially of the NMDA receptor (NMDAR), constitutes one of the main biological substrate of psychotic disorders, such as schizophrenia. The NMDAR signaling hypofunction, through genetic and/or environmental insults, would cause a neurodevelopmental…

 




Abstract Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disease of the central nervous system. Gasdermin D (GSDMD) is associated with autoimmune disorders and neuroinflamm…

 




Background Autoimmune encephalitis (AE) is a noninfectious emergency with severe clinical attacks. It is difficult for the earlier diagnosis of acute AE due to the lack of antibody detection resour…

 




Introduction A new scale, named the Clinical Assessment Scale for Autoimmune Encephalitis (CASE), has recently been developed for rating the severity of autoimmune encephalitis (AE) with a high level of clinimetric properties.

 




Abstract Accumulating data on patients with autoimmune encephalitis have shed light on specificities concerning clinical presentation and outcomes, which are dependent on the antigen targeted by th…

 




Case report Persistent seropositivity in oophorectomy-resistant anti-NMDA receptor encephalitis http://orcid.org/0000-0001-7594-0855Susmit Tripathi1,2, Nara M Michaelson1,2 and Alan Segal1 Neurology, NewYork-Presbyterian Hospital/Weill Cornell Medical Center, New York, New York, USA Neurology, Memorial Sloan Kettering Cancer Center, New York, New York, USA Correspondence to Dr Susmit Tripathi; sut9038{at}nyp.org Abstract To discuss (1) the significance of seropositivity in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis and (2) clinical decision making in oophorectomy resistant disease. Patient A (a 35-year-old woman) had high CSF and serum anti-NMDA antibody titres, a complicated hospital course, little improvement with first and second-line therapies, and remained with high CSF and serum antibody titres despite unilateral oophorectomy, requiring a nearly 13-month long hospitalisation. Conversely, patient B (a 29-year-old woman) had low CSF titres, seronegative disease and quickly recovered to her baseline with first line therapies and oophorectomy. Anti-NMDAR antibodies are themselves pathological, causing signalling dysfunction and internalisation of the NMDAR. Seropositivity with anti-NMDAR antibodies likely reflects leakage from the blood–brain barrier, with high serum titres being a downstream effect of high CSF titres. Empiric bilateral oophorectomies is controversial but appropriate on a case-by-case basis in extremely treatment-resistant NMDAR encephalitis given the possibility of antigenic microteratomas, which may not be detected on imaging or even bilateral ovarian biopsies. Statistics from Altmetric.com View Full Text Footnotes Twitter @nystrokedoc ST and NMM contributed equally. Contributors ST conceptualised and created all figures. ST, NMM and AS drafted the original manuscript. ST and NMM are cofirst authors. All authors were directly involved in the patient’s care and management, provided intellectual content for the manuscript and edited the manuscript. Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors. Competing interests None declared. Provenance and peer review Not commissioned; externally peer reviewed. Request Permissions If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways. Copyright information: © BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ. Read the full text or download the PDF: Subscribe Log in

 




Dr. Luca Bartolini, director of the pediatric epilepsy program at Hasbro Children’s Hospital, was recently named editor of “Neurology: Clinical Practice.” His 10-year term begins next month. Bartolini is the second editor of the journal since it was founded in 2011.

 




The Swiss-Ped-IBrainD is a national patient registry that collects information on diagnosis, symptoms, treatment, and follow-up of pediatric patients with…

 




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