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Update on Autoimmune Encephalitis

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Count Down to WED Conference Saturday, 20 February 2021: Meet Dr. Tom Pollak

Count Down to WED Conference Saturday, 20 February 2021: Meet Dr. Tom Pollak | AntiNMDA | Scoop.it
We are delighted to spotlight Dr. Tom Pollak from the UK. Thanks to his engagement and research, many more are now getting the help they ...Read More...
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Editors' Note: Clinical Significance of Anti-NMDAR Concurrent With Glial or Neuronal Surface Antibodies | Neurology

Editors' Note: Clinical Significance of Anti-NMDAR Concurrent With Glial or Neuronal Surface Antibodies | Neurology | AntiNMDA | Scoop.it
SHARE January 26, 2021; 96 (4) DISPUTES & DEBATES: EDITORS' CHOICE Editors' Note: Clinical Significance of Anti-NMDAR Concurrent With Glial or Neuronal Surface Antibodies Ariane Lewis, Steven Galetta First published January 25, 2021, DOI: https://doi.org/10.1212/WNL.0000000000011358 FULL PDF CITATION PERMISSIONS MAKE COMMENT SEE COMMENTS Downloads0 This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased. In “Clinical Significance of Anti-NMDAR Concurrent With Glial or Neuronal Surface Antibodies,” Martinez-Hernandez et al. reported that between 4% and 7.5% of patients with anti-NMDAR encephalitis have concurrent glial or neuronal surface antibodies (glial-Ab or NS-Ab). Although they found that the presence of myelin oligodendrocyte glycoprotein (MOG) or aquaporin 4 antibodies was associated with demyelinating disorders and NS-Ab was associated with medial temporal or subcortical MRI findings, a positive glial fibrillary acidic protein (GFAP) antibody was nonspecific. McKeon et al. suggested the coexistence of GFAP antibodies and their significance may have been underreported in this study as both serum and CSF testing can be imperfect and that a phenotypic clinical-radiographic presentation associated with this antibody—a steroid-responsive meningoencephalitis with a predilection for the midbrain, cerebellar white matter, hippocampus, and cortex—may have been missed. Martinez-Hernandez et al. responded that McKeon et al. (1) misunderstood the frequency of NMDAR-Ab and GFAP-Ab co-occurrence in their cohort and (2) mischaracterized the specificity of GFAP-Ab. Lancaster reinforced Martinez-Hernandez et al.’s findings, noting that clinicians must be aware that patients with anti-NMDAR encephalitis can have multiple antibodies, which may impact presentation and prognosis. Dalmau and Martinez-Hernandez further commented that patients with NMDAR-Ab who have an atypical presentation of anti-NMDAR encephalitis should prompt consideration of the possibility that the presence of NMDAR-Ab was a false positive or that there could be concurrent antibodies but that the conclusion that any particular clinical or radiologic findings are associated with any particular antibody can only be made after careful evaluation for disease specificity. In “Clinical Significance of Anti-NMDAR Concurrent With Glial or Neuronal Surface Antibodies,” Martinez-Hernandez et al. reported that between 4% and 7.5% of patients with anti-NMDAR encephalitis have concurrent glial or neuronal surface antibodies (glial-Ab or NS-Ab). Although they found that the presence of myelin oligodendrocyte glycoprotein (MOG) or aquaporin 4 antibodies was associated with demyelinating disorders and NS-Ab was associated with medial temporal or subcortical MRI findings, a positive glial fibrillary acidic protein (GFAP) antibody was nonspecific. McKeon et al. suggested the coexistence of GFAP antibodies and their significance may have been underreported in this study as both serum and CSF testing can be imperfect and that a phenotypic clinical-radiographic presentation associated with this antibody—a steroid-responsive meningoencephalitis with a predilection for the midbrain, cerebellar white matter, hippocampus, and cortex—may have been missed. Martinez-Hernandez et al. responded that McKeon et al. (1) misunderstood the frequency of NMDAR-Ab and GFAP-Ab co-occurrence in their cohort and (2) mischaracterized the specificity of GFAP-Ab. Lancaster reinforced Martinez-Hernandez et al.’s findings, noting that clinicians must be aware that patients with anti-NMDAR encephalitis can have multiple antibodies, which may impact presentation and prognosis. Dalmau and Martinez-Hernandez further commented that patients with NMDAR-Ab who have an atypical presentation of anti-NMDAR encephalitis should prompt consideration of the possibility that the presence of NMDAR-Ab was a false positive or that there could be concurrent antibodies but that the conclusion that any particular clinical or radiologic findings are associated with any particular antibody can only be made after careful evaluation for disease specificity. Footnotes Author disclosures are available upon request (journal{at}neurology.org). See letter See letter See response © 2021 American Academy of Neurology View Full Text AAN Members We have changed the login procedure to improve access between AAN.com and the Neurology journals. If you are experiencing issues, please log out of AAN.com and clear history and cookies. (For instructions by browser, please click the instruction pages below). After clearing, choose preferred Journal and select login for AAN Members. You will be redirected to a login page where you can log in with your AAN ID number and password. When you are returned to the Journal, your name should appear at the top right of the page. Google Safari Microsoft Edge Firefox CLICK HERE TO LOGIN AAN Non-Member Subscribers CLICK HERE TO LOGIN Purchase access For assistance, please contact: AAN Members (800) 879-1960 or (612) 928-6000 (International) Non-AAN Member subscribers (800) 638-3030 or (301) 223-2300 option 3, select 1 (international) Sign Up Information on how to subscribe to Neurology and Neurology: Clinical Practice can be found here  Purchase Individual access to articles is available through the Add to Cart option on the article page.  Access for 1 day (from the computer you are currently using) is US$ 39.00.  Pay-per-view content is for the use of the payee only, and content may not be further distributed by print or electronic means.  The payee may view, download, and/or print the article for his/her personal, scholarly, research, and educational use.  Distributing copies (electronic or otherwise) of the article is not allowed. 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Anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma in Korea

Anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma in Korea | AntiNMDA | Scoop.it
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe autoimmune paraneoplastic syndrome associated with ovarian teratomas.Most patients develop neurologic symptoms, including psychosis, memory deficits, seizures, or abnormal movements, and experience abdominal pain related to ovarian...
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Neurological webinar series –

Neurological webinar series – | AntiNMDA | Scoop.it
By EUROIMMUN academy First Webinar: Autoimmune Encephalitis - Adult and Paediatric cases When: January 28thTime: 11-12.30PM EST Click here to register.
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Paediatric multiple sclerosis and antibody-associated demyelination: clinical, imaging, and biological considerations for diagnosis and care

Paediatric multiple sclerosis and antibody-associated demyelination: clinical, imaging, and biological considerations for diagnosis and care | AntiNMDA | Scoop.it
The field of acquired CNS neuroimmune demyelination in children is transforming. Progress in assay development, refinement of diagnostic criteria, increased biological insights provided by advanced neuroimaging techniques, and high-level evidence for the therapeutic efficacy of biological agents...
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Antineuronal antibodies and epilepsy: treat the patient, not the lab | Journal of Neurology, Neurosurgery & Psychiatry

The study results implicate that starting immunosppressive treatment in new-onset epilepsy should be guided by clinics, not simply antibody presence Epilepsy affects about 70–80 million people worldwide; about one-third of patients cannot become seizure free. New diagnostic and therapeutic avenues to improve this situation are welcome. The impact of autoimmune phenomena on pathogenesis of some epilepsies increasingly gained attention as these mechanisms open the door for alternative medical treatments beyond antiseizure medications, that is, immunosuppressants. Thus, ‘autoimmune’ has become one of the six aetiologic categories of the new Intenational League Against Epilepsy classification of seizures and epilepsies. Numerous neuronal surface autoantibodies (NSAbs) identified in the past years cause autoimmune encephalitis (AE),1 often associated with severe seizures and status epilepticus. Additionally, the prevalence of NSAbs in patients with chronic epilepsies of unknown aetiology yielded a prevalence between 3% and 21%, but the question whether all epilepsy patients with NSAbs or only those with pharmacoresistant epilepsy (PRE) and/or additional signs of AE warrant immunosuppressants remained unresolved yet. A study looking at the presence of NSAbs in PRE found that 62% of patients responded to immunotherapy, and 34% even became seizure free, indicating that a trial may be justfied.2 But how does this result relate to patients with new-onset focal epilepsy (NFE)? The paper by Mc Ginty et al,3 tackles this issue by prospectively looking at those patients with NFE and a test positive for at least one NSAbs. The authors established an NSAbs prediction score based on clinical and paraclinical information and evaluated the value of immunotherapy in patients with NFE. About 10% of their cohort was NSAbs positive and 40% of them were diagnosed with AE. They identified six features which in combination were highly predictive for the presence of NSAbs, that is, age >54 years, ictal piloerection, self-reported lowered mood, MRI changes in the limbic system, the absence of ‘conventional’ epilepsy risk factors and intact attention. This ‘NSAbs-detecting’ Score compared better with the recently published ‘antibody prevalence in epilepsy and encephalopathy’ (APE2) Score4 in terms of forecasting AE, but worse in predicting presence of NSAbs. According to the present study (with an admittedly small sample of patients), immunotherapy could be omitted in those patients with NSAbs-positive new-onset epilepsy without signs or symptoms of AE. Conversely, the study also indicates that immunosuppressants are warranted in patients with even subtle AE. This is in line with another study where patients with AE even without NSAbs benefitted from immunosuppressants.5 The authors conclude that the administration of immunotherapy in NSAbs-positive patients should be guided by clinical signs for (subtle or obvious) AE and not only by NSAbs positivity per se. The study did not rely on cerebrospinal fluid data, probably leading to some missed cases of NSAbs positivity and AE. It is also interesting that 5/16 NSAbs positive, but AE-negative patients had mRS >0 and, thus, likely were pharmacoresistant, although this information was not exactly verifiable without follow-up phone interview. Statistically, this would exactly fit the one-third of patients basically becoming pharmacoresistant in chronic epilepsy of various aetiologies. Thus, future trials should test whether immunotherapy given to these patients would prevent pharmacoresistancy despite the fact that outcome without such treatment was mostly promising in this study.
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Autoimmune encephalitis and epilepsy: evolving definition and clinical spectrum

Autoimmune encephalitis and epilepsy: evolving definition and clinical spectrum | AntiNMDA | Scoop.it
Advances in autoimmune encephalitis studies in the past 10 years have led to the identification of new syndromes and biomarkers that have transformed the diagnostic approach to the disorder.The disorder or syndrome has been linked to a wide variety of pathologic processes associated with the neuron...
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Correction to: Refractory anti‑NMDAR encephalitis successfully treated with bortezomib and associated movements disorders controlled with tramadol: a case report with literature review | SpringerLink

Correction to: Refractory anti‑NMDAR encephalitis successfully treated with bortezomib and associated movements disorders controlled with tramadol: a case report with literature review | SpringerLink | AntiNMDA | Scoop.it
The authors would like to correct some errors in the publication of the original article. The corrected and updated details are given below for your reading.
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Ca V α2δ Autoimmune Encephalitis: A Novel Antibody and its Characteristics

Ca V α2δ Autoimmune Encephalitis: A Novel Antibody and its Characteristics | AntiNMDA | Scoop.it
Here, we found a novel autoimmune encephalitis associated with anti-Ca<sub>V</sub> α2δ antibody. Further analysis of the antibody in autoimmune encephalitis might promote the early diagnosis and treatment.
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Clinical features and management of coexisting anti-N-methyl-D-aspartate receptor encephalitis and myelin oligodendrocyte glycoprotein antibody-associated encephalomyelitis: a case report and revie...

Clinical features and management of coexisting anti-N-methyl-D-aspartate receptor encephalitis and myelin oligodendrocyte glycoprotein antibody-associated encephalomyelitis: a case report and revie... | AntiNMDA | Scoop.it
The rates of coexisting anti-NMDA receptor encephalitis and MOG antibody-associated encephalomyelitis may be underestimated.Clinical symptoms such as seizures and cognitive decline accompanied by atypical central nervous system demyelination serve as warning signs of possible coexisting anti-NMDA...
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The Antibodies Contributing to focal Epilepsy Signs and symptoms (ACES) score - Bruijn - - Annals of Neurology

The Antibodies Contributing to focal Epilepsy Signs and symptoms (ACES) score - Bruijn - - Annals of Neurology | AntiNMDA | Scoop.it
Objective Diagnosing autoimmune encephalitis (AIE) is difficult in patients with less fulminant diseases, like epilepsy. However, recognition is important, as patients require immunotherapy. This s...
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Can You Identify Autoimmune Psychosis? | Psychiatric News

Can You Identify Autoimmune Psychosis? | Psychiatric News | AntiNMDA | Scoop.it
Patients with a rare neurological condition known as anti-NMDA receptor encephalitis often present first with psychosis.
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Good Article! It used to be that relapse rates were between 15-25%. In this report, Dalmau refers to a 10% relapse rate. 

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Infiltration of plasma cells in colorectal adenocarcinoma associated with autoimmune encephalitis

Infiltration of plasma cells in colorectal adenocarcinoma associated with autoimmune encephalitis | AntiNMDA | Scoop.it
Paraneoplastic autoimmune encephalitis (PAE) represents a group of rare neurological syndromes associated with neoplastic diseases.Here, we report a case that multiple anti-neuronal antibodies were present in a patient with PAE who developed both small cell lung cancer and colorectal adenocarcinoma...
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Reader Response: Clinical Significance of Anti-NMDAR Concurrent With Glial or Neuronal Surface Antibodies

Reader Response: Clinical Significance of Anti-NMDAR Concurrent With Glial or Neuronal Surface Antibodies | AntiNMDA | Scoop.it
We welcome the study by Martinez-Hernandez et al.1 reporting antibody coexistence in anti-n-methyl-d-aspartate receptor (NMDA-R) encephalitis. The authors confirmed coexistence of aquaporin-4-IgG or myelin oligodendrocyte glycoprotein (MOG)-IgG as predictors of co-occurrence of anti-NMDA-R...
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How to Report with Accuracy and Sensitivity on Contested Illnesses

How to Report with Accuracy and Sensitivity on Contested Illnesses | AntiNMDA | Scoop.it
Contested illnesses pose extraordinary challenges for science journalists, because the scientific consensus we aim to present simply doesn’t exist for most questions. Science journalists thus have a heavy lift figuring out what the science truly says and making sure they neither elevate fringe...
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From brain to behaviour

From brain to behaviour | AntiNMDA | Scoop.it
Each month Nature Communications’ editors highlight new Articles they see as particularly interesting or important in the area of neuroscience research.
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Frontiers | Clinical and Radiological Features of Adult Onset Bilateral Medial Frontal Cerebral Cortical Encephalitis With Anti-myelin Oligodendrocyte Glycoprotein Antibody | Neurology

Frontiers | Clinical and Radiological Features of Adult Onset Bilateral Medial Frontal Cerebral Cortical Encephalitis With Anti-myelin Oligodendrocyte Glycoprotein Antibody | Neurology | AntiNMDA | Scoop.it
Objective: To clarify the clinical and radiological features of adult onset anti-myelin oligodendrocyte glycoprotein (MOG) antibody-associated bilateral medial frontal cerebral cortical encephalitis (BFCCE).Methods: We systematically reviewed the literature for patients with anti-MOG...
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Seizures in autoimmune encephalitis—A systematic review and quantitative synthesis - Yeshokumar - - Epilepsia

Seizures in autoimmune encephalitis—A systematic review and quantitative synthesis - Yeshokumar - - Epilepsia | AntiNMDA | Scoop.it
Abstract Objective This study aimed to evaluate the proportion of patients with seizures and electroencephalography (EEG) abnormalities in autoimmune encephalitis (AE) and its most common subtypes....
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High serum creatinine is associated with reduction of psychiatric symptoms in female patients with anti-NMDAR encephalitis - ScienceDirect

High serum creatinine is associated with reduction of psychiatric symptoms in female patients with anti-NMDAR encephalitis - ScienceDirect | AntiNMDA | Scoop.it
Serum creatinine (SCR) has been shown to be associated with many neurodegenerative diseases.In this study, we investigated the relationship between S…...
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Count Down to WED Conference 2021: Meet Dr. Jérôme Honnorat

Count Down to WED Conference 2021: Meet Dr. Jérôme Honnorat | AntiNMDA | Scoop.it
Dr. Honnorat has more than 25 years of experience in the field of paraneoplastic neurological syndromes (PNS) and autoimmune encephalitis (AE). The topic of his presentations will be the role of genetics in autoimmune encephalitis (AE). He is head of the Department of Neuro-Oncology at the neurological hospital, Hospices Civils de Lyon and a team leader of the “Synaptopathies and Autoantibodies (SynatAc)” at the Institute NeuroMyoGene (UMR INSERM U1217, CNRS 5310). He is also chair of the French rare disease reference center “paraneoplastic neurological syndrome and autoimmune encephalitis” since 2007, which collects samples from all over France and provides care and follow-up for people with rare diseases. He has been working on PNS and AE for more than 25 years. His main work combines Basic and Clinical Research at multiple levels (patient’s cohorts; biological collections; cellular and animal models; biomarkers...) to explore the pathophysiologies of both brain tumors and neuro-inflammatory diseases and to detect specific autoantibodies as biomarkers of these diseases and as tools to understand synaptic functions.  We invite you to register today for what promises to be just one of the fascinating presentations of the conference.
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Normally Sharp, He Was Chronically Confused. What Was Going On? - The New York Times

Normally Sharp, He Was Chronically Confused. What Was Going On? - The New York Times | AntiNMDA | Scoop.it
He was getting lost driving home from work. And he was having strange spasms. What was the cause?
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Leveraging molecular biomarkers to make the common diagnosis in the uncommon patient - ScienceDirect

Leveraging molecular biomarkers to make the common diagnosis in the uncommon patient - ScienceDirect | AntiNMDA | Scoop.it
The factors that predispose to relapse in patients recovering with autoimmune encephalitis (AE) are largely unknown, complicating efforts to distingui…...
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Flortaucipir (tau) PET in LGI1 antibody encephalitis - Day - - Annals of Clinical and Translational Neurology

Flortaucipir (tau) PET in LGI1 antibody encephalitis - Day - - Annals of Clinical and Translational Neurology | AntiNMDA | Scoop.it
Abstract The contributors to persistent cognitive impairment and hippocampal atrophy in leucine‐rich glioma‐inactivated 1 antibody encephalitis (LGI1) patients are unknown. We evaluated whether tau...
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Collection of Biological Samples From Patients With Rare Neurological...

The aim of this project is to improve biological collections of patients presenting rare neurological disorders with known or suspected autoimmune origin...
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Individuals with psychosis more likely to have certain antibodies

Individuals with psychosis more likely to have certain antibodies | AntiNMDA | Scoop.it
Individuals with psychosis were more likely to have a higher prevalence of a particular antibody vs. controls, according to results of a meta-analysis published in The Lancet Psychiatry.&ldquo;The discovery of an encephalitic syndrome driven by antibodies targeting the N-methyl-D aspartate receptor...
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