The Anti-NMDA Receptor Encephalitis Foundation Newsletter




Our meta-analysis showed that rituximab is an effective second line agent for AE with an acceptable toxicity profile.

 




Objective:

We present a patient with severe aphasic and apraxic syndromes as her clinical manifestation of anti-NMDAR encephalitis. Nineteen months..

 




Brain, Behavior, and Immunity Available online 23 May 2020 Anti-NMDA receptor encephalitis in a psychiatric Covid-19 patient: A case report Author links open overlay panel Show more Keywords COVID-19Autoimmune encephalitisPsychiatric disorder View Abstract Published by Elsevier Inc.

 




Antibodies to glutamic acid decarboxylase (GAD) are associated with several neurological syndromes but their importance and role are controversial. In this Review, Graus et al. propose criteria for assessing the association between GAD antibodies and neurological syndromes and discuss the…

 




N-methyl-d-aspartate receptor antibody (NMDAR-Ab) encephalitis consensus criteria has recently been defined.We aimed to examine the prevalence of NMD……

 




Abstract Objective To determine the frequency and significance of concurrent glial (glial-Ab) or neuronal-surface (NS-Ab) antibodies in patients with anti–NMDA receptor (NMDAR) encephalitis. Methods Patients were identified during initial routine screening of a cohort (C1) of 646 patients consecutively diagnosed with anti-NMDAR encephalitis and another cohort (C2) of 200 patients systematically rescreened. Antibodies were determined with rat brain immunostaining and cell-based assays. Results Concurrent antibodies were identified in 42 patients (4% from C1 and 7.5% from C2): 30 (71%) with glial-Ab and 12 (29%) with NS-Ab. Glial-Ab included myelin oligodendrocyte glycoprotein (MOG) (57%), glial fibrillary acidic protein (GFAP) (33%), and aquaporin 4 (AQP4) (10%). NS-Ab included AMPA receptor (AMPAR) (50%), GABAa receptor (GABAaR) (42%), and GABAb receptor (8%). In 39 (95%) of 41 patients, concurrent antibodies were detected in CSF, and in 17 (41%), concurrent antibodies were undetectable in serum. On routine clinical-immunologic studies, the presence of MOG-Ab and AQP4-Ab was suggested by previous episodes of encephalitis or demyelinating disorders (8, 27%), current clinical-radiologic features (e.g., optic neuritis, white matter changes), or standard rat brain immunohistochemistry (e.g., AQP4 reactivity). GFAP-Ab did not associate with distinct clinical-radiologic features. NS-Ab were suggested by MRI findings (e.g., medial temporal lobe changes [AMPAR-Ab], or multifocal cortico-subcortical abnormalities [GABAaR-Ab]), uncommon comorbid conditions (e.g., recent herpesvirus encephalitis), atypical tumors (e.g., breast cancer, neuroblastoma), or rat brain immunostaining. Patients with NS-Ab were less likely to have substantial recovery than those with glial-Ab (5 of 10 [50%] vs 17 of 19 [89%], p = 0.03). Conclusions Between 4% and 7.5% of patients with anti-NMDAR encephalitis have concurrent glial-Ab or NS-Ab. Some of these antibodies (MOG-Ab, AQP4-Ab, NS-Ab) confer additional clinical-radiologic features and may influence prognosis. Glossary AMPAR=AMPA receptor; AQP4=aquaporin 4; CBA=cell-based assay; FLAIR=fluid-attenuated inversion recovery; GABAaR=GABAa receptor; GABAbR=GABAb receptor; GFAP=glial fibrillary acidic protein; glial-ab=antibodies against glial proteins; GlyR=glycine receptor; IQR=interquartile range; MOG=myelin oligodendrocyte glycoprotein; mRS=modified Rankin Scale; NMDAR=NMDA receptor; NS-Ab=antibodies against neuronal cell surface proteins Footnotes Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article. Received August 30, 2019. Accepted in final form December 3, 2019. © 2020 American Academy of Neurology

 




High specificity of the proposed criteria, seen in this study, suggests that cases which are positive can be considered for expedited commencement of treatment. However, if clinical suspicion is high despite criteria being negative, it is essential to test CSF for anti-NMDA antibody.

 




Neurological rarities Autoimmune encephalitis associated with Ma2 antibodies and immune checkpoint inhibitor therapy http://orcid.org/0000-0001-8381-7531Shane Lyons1, Ronan Joyce1, Patrick Moynagh1, Luke O’Donnell1, Silive Blazkova2, Timothy J Counihan1,3 Neurology Department, University Hospital Galway, Galway, Ireland Oncology Department, University Hospital Galway, Galway, Ireland National University of Ireland Galway, Galway, Ireland Correspondence to Dr Shane Lyons, University Hospital Galway, Galway H91 YR71, Ireland; shane.lyons{at}ndcn.ox.ac.uk Abstract Immune checkpoint inhibitors have transformed the treatment of advanced malignancy, while increasing the risk of immune-related adverse events. A 56-year-old woman who had received nivolumab for stage 4 renal cell carcinoma subsequently developed altered behaviour, memory deficits and worsening of previously stable epilepsy. MR scan of the brain showed bilateral FLAIR (fluid-attenuated inversion recovery) hyperintensity of the mesial temporal lobes, and there were anti-Ma2 antibodies in both serum and cerebrospinal fluid. She was treated with corticosteroids but developed further clinical relapses requiring immunoglobulin and rituximab. The immune-related adverse events relating to immune checkpoint inhibitors are an emerging challenge for the neurologist. Some cases are refractory and require serial immunosuppression. View Full Text Statistics from Altmetric.com View Full Text Footnotes SL and RJ contributed equally. Contributors SL and RJ drafted the manuscript. PM and LOD reviewed and edited the manuscript. SB and TJC reviewed, revised 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. Patient consent for publication Next of kin consent obtained. Provenance and peer review Not commissioned. Externally peer reviewed by Sarosh Irani, Oxford, UK. 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: © Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ. Read the full text or download the PDF: Subscribe Log in

 




Anti- Leucine Glioma Inactivated (LGI)1 autoimmune encephalitis (AE) is a rare neuroinflammatory brain condition.Individuals afflicted with this cond……

 




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