AntiNMDA
32.5K views | +0 today
Follow
 
Scooped by Nesrin Shaheen
onto AntiNMDA
Scoop.it!

Refractory NMDA-receptor encephalitis in a teenager: A novel use of Bortezomib

Refractory NMDA-receptor encephalitis in a teenager: A novel use of Bortezomib | AntiNMDA | Scoop.it
We report the case of a 14-year-old girl who was diagnosed with N-methyl-d-aspartate
(NMDA)-receptor encephalitis, with severe features and autonomic instability, requiring
intensive care unit admission.
No comment yet.
AntiNMDA
Your new post is loading...
Scooped by Nesrin Shaheen
Scoop.it!

Are We Missing Subtle Forms of Anti-N-Methyl-D-Aspartate Encephalitis With the Current Diagnostic Approach? A Case Report

Are We Missing Subtle Forms of Anti-N-Methyl-D-Aspartate Encephalitis With the Current Diagnostic Approach? A Case Report | AntiNMDA | Scoop.it
Encephalitis related to antibodies against the N-methyl-D-aspartate receptor (NMDAr) is a recently described clinical entity in which IgG autoantibodies against the NR1 subunit of the NMDAr lead to the appearance of complex neuropsychiatric symptoms.
No comment yet.
Scooped by Nesrin Shaheen
Scoop.it!

Mitochondrial diseases mimicking autoimmune diseases of the CNS and good response to steroids initially

Mitochondrial diseases mimicking autoimmune diseases of the CNS and good response to steroids initially | AntiNMDA | Scoop.it
Neuroimmunological diseases such as autoimmune encephalitis (AE) or acquired demyelinating
syndromes (ADS), can present with neurological symptoms and imaging features that
are indistinguishable from mitochondrial diseases (MD) in particular at initial presentation.
No comment yet.
Scooped by Nesrin Shaheen
Scoop.it!

Rapidly progressive dementia: limitations in Africa | Practical Neurology

Rapidly progressive dementia: limitations in Africa | Practical Neurology | AntiNMDA | Scoop.it
The term rapidly progressive dementia refers to dementia (and excludes delirium) typically developing over weeks or months, including those evolving in less than 1–2 years from onset.1 2 The fact that they are rapidly progressive underlines the need for critical clinical evaluation and detailed investigations. The case report from Zambia in Practical Neurology,3 caused by cryptococcal infection in a non-HIV setting, illustrates that rapidly progressive dementia can occur anywhere in the world and may have a treatable cause. While practising neurologists will be very familiar with the main categories of rapidly progressive dementia, the relative infrequency and diversity of causes of these conditions presents a formidable diagnostic challenge.The main categories reported worldwide are non-prion neurodegenerative disorders, mostly dementias, autoimmune encephalopathies, prion disease, infective, metabolic, nutritional and malignant disorders.1 2 Of these causes, human prion disease, Creutzfeldt-Jakob disease (CJD) has received most attention globally. CJD is classified into sporadic 85%–90%, genetic 10%–15% and acquired 1%. It is a relatively infrequent and universally fatal disease with an annual incidence of approximately 1–2 per million worldwide but is on the increase.4 Only 23 cases, mostly of sporadic CJD, have been reported from Africa,5–12 although genetic CJD has been reported historically in migrant populations coming from North Africa.13 Readers need little reminding of the dangers of transmissible prion disease, with the outbreak of bovine spongiform encephalopathy in the UK in the 1990s.14 Sporadic CJD may be suspected clinically on the basis of a rapidly progressive dementia presenting typically with myoclonus and seizures developing over weeks to months. Supportive CSF findings include a normal white cell count, elevated protein, and the finding of tau and 14-3-3. Characteristic EEG findings are periodic sharp wave complexes and an MR of the brain scan shows high signal changes in the caudate, putamen and cortical regions with cortical ribboning. While previously a definitive diagnosis relied on brain biopsy, there is now a highly sensitive (90%–97%) and specific (99%–100%) laboratory CSF test for prion diseases called the ‘real-time quaking-induced conversion” (RT-QuIC).15 This obviates the need for brain biopsy with its inherent danger of prion transmission. The occurrence of CJD is likely to be underestimated in Africa. Apparent limitations are evidenced by the relatively small number of histologically proven cases9 10 12 and just a single recent case report using the RT-QuIC test, accessed abroad.5 6 The encephalopathies are a relatively common and treatable cause of rapidly progressive dementia.2 African-Americans have a higher reported frequency at 38.3/100 000 compared with Caucasians at 13.7/100 000.16 The main causes are antibody mediated, CNS lupus, acute disseminated encephalomyelitis, Hashimoto’s disease and vasculitis, many of which require a newer sophisticated range of CSF serological tests and imaging for their diagnosis. Their increasing incidence in high-income countries is attributed to increased recognition of antibody-positive cases. There are several recent case reports from Africa, in particular associated with N-methyl-D-aspartate-receptor antibodies.17–24 In contrast to non-treatable causes, many of the infective, metabolic and nutritional causes can be diagnosed and treated in Africa. The main infective causes are viral (HIV, herpes simplex virus and progressive multifocal leukoencephalopathy), bacterial (tuberculosis, syphilis, Lyme disease), fungal (cryptococcus and aspergillosis), protozoal (malaria, trypanosomiasis and amoebiasis) and worm related (neurocysticercosis). The more common chronic CNS infections in Africa are tuberculosis and opportunistic infections in HIV.25 Neurosyphilis is distinctly uncommon in Africa26 and trypanosomiasis, despite a decreasing incidence remains a threat in endemic areas.27 Metabolic and nutritional causes include electrolyte imbalance, endocrine disorders, vitamin deficiencies, organ failure and metal toxicity. Malignant causes include metastases, primary CNS lymphoma, glioma and paraneoplastic limbic encephalitis.2 The recommended initial screening tests for suspected cases of rapidly progressive dementia involve bloods, HIV/VDRL testing, urine analysis, neuroimaging, EEG and lumbar puncture. CSF investigations include opening pressure, cells, protein, glucose, Gram, fungal and acid-fast stains, and cultures. CSF serological screening tests for infection include viral (PCR), cryptococcus antigen, tuberculosis (GeneXpert), and syphilis (VDRL/TPHA).2 These are mostly available in Africa, apart from viral PCR.28 Screening for prion proteins and autoimmune antibodies and more specialised neuroimaging is recommended only if clinically indicated and if initial rapidly progressive dementia screening tests are non-diagnostic. However, these more specialised tests are not available in Africa, except in a few highly specialised centres. 5 Thus, the laboratory limitations facing Africa become more apparent at this level.29 The case report by Chishimba et al is the first specifically on rapidly progressive dementia published from Africa. While there are only few published reports of this presentation from Africa, the field is changing, notably with increasing reports of autoimmune encephalitis within the last 5 years. Their under-recognition up until relatively recently has identified an important and treatable cause in Africa. 24 Understandable contributory reasons in Africa are the low numbers of practising neurologists, (1–2 per 10 million population), and the lack of specialised laboratory facilities. There is a clear need for sophisticated laboratory investigations to be more widely available in Africa. The case is particularly pertinent given its large population, currently almost 1.4 billion and an increasing prevalence of dementia, estimated at 2.5% in those aged over 50 years and 4% in those over 60 years.30 31 Neuroscience is a rapidly changing and exciting field globally. Africa is part of that change with increasing neuroscience publications32, the arrival of neurogenomics,33 the formation of the African Academy of Neurology and recently a brain bank for dementia/neurodegeneration in Ibadan, Nigeria, the first in sub-Saharan Africa.34 Collaboration with neuroscience institutions in high-income countries is part of this process. Meanwhile, the emphasis in Africa must remain on treatable causes of rapidly progressive dementia.Ethics statementsPatient consent for publicationNot applicable.Ethics approvalNot applicable.References↵ Paterson RW , Takada LT , Geschwind MD . Diagnosis and treatment of rapidly progressive dementias. Neurol Clin Pract 2012;2:187–200.doi:10.1212/CPJ.0b013e31826b2ae8 pmid:http://www.ncbi.nlm.nih.gov/pubmed/23634367 OpenUrlAbstract/FREE Full Text↵ Geschwind MD . Rapidly progressive dementia. Continuum 2016;22:510–37.doi:10.1212/CON.0000000000000319 pmid:http://www.ncbi.nlm.nih.gov/pubmed/27042906 OpenUrlPubMed↵ Chishimba L , Mataa MM , Yumbe-Zimba K , et al . Rapidly progressive dementia in an elderly man. Pract Neurol 2022.doi:10.1136/practneurol-2021-003272 ↵ Watson N , Brandel J-P , Green A , et al . The importance of ongoing international surveillance for Creutzfeldt-Jakob disease. Nat Rev Neurol 2021;17:362–79.doi:10.1038/s41582-021-00488-7 pmid:http://www.ncbi.nlm.nih.gov/pubmed/33972773 OpenUrlPubMed↵ Sokhi D , Yakub F , Sharma K , et al . Heidenhain variant of sporadic Creutzfeldt-Jakob disease: first reported case from East Africa. Int Med Case Rep J 2021;14:39–44.doi:10.2147/IMCRJ.S287358 pmid:http://www.ncbi.nlm.nih.gov/pubmed/33564270 OpenUrlPubMed↵ Negm M , Hashish E . Probable Creutzfeldt-Jakob disease—a case report at Suez canal university Hospital, Egypt. Egypt J Neurol Psychiatry Neurosurg 2019;55:36.doi:10.1186/s41983-019-0085-8 ↵ Hajjaj I , Kissani N . [First case of presumed sporadic Creutzfeldt-Jakob Disease in Marrakech, Morocco]. Med Trop Rev Corps Sante Colon 2011;71:289–91.OpenUrl↵ Mead S , Webb TEF , Campbell TA , et al . Inherited prion disease with 5-OPRI: phenotype modification by repeat length and codon 129. Neurology 2007;69:730–8.doi:10.1212/01.wnl.0000267642.41594.9d pmid:http://www.ncbi.nlm.nih.gov/pubmed/17709704 OpenUrlPubMed↵ Toovey S , Britz M , Hewlett RH . A case dura mater graft-associated Creutzfeldt-Jakob disease in South Africa. S Afr Med J 2008;96:592.OpenUrl↵ Adam AM , Akuku O . Creutzfeldt-Jakob disease in Kenya. Trop Med Int Health 2005;10:710–2.doi:10.1111/j.1365-3156.2005.01435.x pmid:http://www.ncbi.nlm.nih.gov/pubmed/15960711 OpenUrlPubMed↵ Mouillet-Richard S , Teil C , Lenne M , et al . Mutation at codon 210 (V210I) of the prion protein gene in a North African patient with Creutzfeldt-Jakob disease. J Neurol Sci 1999;168:141–4.doi:10.1016/S0022-510X(99)00179-3 pmid:http://www.ncbi.nlm.nih.gov/pubmed/10526198 OpenUrlCrossRefPubMed↵ Aka-Diarre E-D , Sitting T , Kouame-Aswan B . Application of the electroencephalogram (EEG) in the diagnosis of Creutzfeldt-Jakob Disease (CJD) in Africa. Description of three cases in Côte D’Ivoire. Afr J Neurol Sci 2007;26:66–72.OpenUrl↵ Chapman J , Korczyn AD . Genetic and environmental factors determining the development of Creutzfeldt-Jakob disease in Libyan Jews. Neuroepidemiology 1991;10:228–31.doi:10.1159/000110276 pmid:http://www.ncbi.nlm.nih.gov/pubmed/1798423 OpenUrlPubMed↵ Houston F , Andréoletti O . Animal prion diseases: the risks to human health. Brain Pathol 2019;29:248–62.doi:10.1111/bpa.12696 pmid:http://www.ncbi.nlm.nih.gov/pubmed/30588682 OpenUrlCrossRefPubMed↵ Figgie MP , Appleby BS . Clinical use of improved diagnostic testing for detection of prion disease. Viruses 2021;13:789.doi:10.3390/v13050789 pmid:http://www.ncbi.nlm.nih.gov/pubmed/33925126 OpenUrlPubMed↵ Dubey D , Pittock SJ , Kelly CR , et al . Autoimmune encephalitis epidemiology and a comparison to infectious encephalitis. Ann Neurol 2018;83:166–77.doi:10.1002/ana.25131 pmid:http://www.ncbi.nlm.nih.gov/pubmed/29293273 OpenUrlCrossRefPubMed↵ Rakiro J , Sokhi D . Fatal autoimmune anti-NMDA-receptor encephalitis with poor prognostication score in a young Kenyan female. Int Med Case Rep J 2021;14:343–7.doi:10.2147/IMCRJ.S311071 pmid:http://www.ncbi.nlm.nih.gov/pubmed/34079388 OpenUrlPubMed↵ Douma B , Ben Younes T , Benrhouma H , et al . Autoimmune encephalitis in Tunisia: report of a pediatric cohort. J Immunol Res 2021;2021:1–7.doi:10.1155/2021/6666117 pmid:http://www.ncbi.nlm.nih.gov/pubmed/34056010 OpenUrlPubMed↵ Sokhi DS , Bhogal OS . Autoimmune encephalitis is recognised as an important differential diagnosis in a Kenyan tertiary referral centre. BMJ Mil Health 2020;166:358.doi:10.1136/jramc-2019-001338 pmid:http://www.ncbi.nlm.nih.gov/pubmed/32015184 OpenUrlFREE Full Text↵ Thompson DC , Bailey MS , Bowley D , et al . Encephalitis on deployment in Kenya: think beyond the infections. J R Army Med Corps 2019;165:374–6.doi:10.1136/jramc-2018-001115 pmid:http://www.ncbi.nlm.nih.gov/pubmed/30992337 OpenUrlAbstract/FREE Full Text↵ Ba F , Ba EHM , Guèye MM , et al . Susac syndrome: about two cases. Pan Afr Med J 2019;33:145.doi:10.11604/pamj.2019.33.145.17954 pmid:http://www.ncbi.nlm.nih.gov/pubmed/31558942 OpenUrlPubMed↵ Toudou-Daouda M , Filali-Adib A , Slassi A , et al . Limbic encephalitis: experience of a Moroccan center. Brain Behav 2019;9:e01177.doi:10.1002/brb3.1177 pmid:http://www.ncbi.nlm.nih.gov/pubmed/30474361 OpenUrlPubMed↵ Gbadero DA , Adegbite EO , LePichon J-B , et al . Case presentation of anti-NMDA receptor encephalitis in a 4-year-old boy. J Trop Pediatr 2018;64:352–4.doi:10.1093/tropej/fmx070 pmid:http://www.ncbi.nlm.nih.gov/pubmed/29040795 OpenUrlPubMed↵ Roos I , Bhigjee AI . Autoimmune encephalitis: a missed diagnostic and therapeutic opportunity. Afr J Neurol Sci 2017;36:66–79.OpenUrl↵ Howlett WP . Neurological disorders in HIV in Africa: a review. Afr Health Sci 2019;19:1953–77.doi:10.4314/ahs.v19i2.19 pmid:http://www.ncbi.nlm.nih.gov/pubmed/31656479 OpenUrlPubMed↵ Marks M , Jarvis JN , Howlett W , et al . Neurosyphilis in Africa: a systematic review. PLoS Negl Trop Dis 2017;11:e0005880.doi:10.1371/journal.pntd.0005880 pmid:http://www.ncbi.nlm.nih.gov/pubmed/28859081 OpenUrlPubMed↵ Kennedy PGE . Update on human African trypanosomiasis (sleeping sickness). J Neurol 2019;266:2334–7.doi:10.1007/s00415-019-09425-7 pmid:http://www.ncbi.nlm.nih.gov/pubmed/31209574 OpenUrlCrossRefPubMed↵ Siddiqi OK , Ghebremichael M , Dang X , et al . Molecular diagnosis of central nervous system opportunistic infections in HIV-infected Zambian adults. Clin Infect Dis 2014;58:1771–7.doi:10.1093/cid/ciu191 pmid:http://www.ncbi.nlm.nih.gov/pubmed/24668125 OpenUrlCrossRefPubMed↵ Ondoa P , Ndlovu N , Keita M-S , et al . Preparing national tiered laboratory systems and networks to advance diagnostics in Africa and meet the continent's health agenda: insights into priority areas for improvement. Afr J Lab Med 2020;9:1103.doi:10.4102/ajlm.v9i2.1103 pmid:http://www.ncbi.nlm.nih.gov/pubmed/33102173 OpenUrlPubMed↵ Akinyemi RO , Yaria J , Ojagbemi A , et al . Dementia in Africa: current evidence, knowledge gaps, and future directions. Alzheimers Dement 2021. doi:doi:10.1002/alz.12432. [Epub ahead of print: 27 Sep 2021].pmid:http://www.ncbi.nlm.nih.gov/pubmed/34569714 ↵ Ojagbemi A , Okekunle AP , Babatunde O . Dominant and modifiable risk factors for dementia in sub-Saharan Africa: a systematic review and meta-analysis. Front Neurol 2021;12:627761.doi:10.3389/fneur.2021.627761 pmid:http://www.ncbi.nlm.nih.gov/pubmed/33841302 OpenUrlPubMed↵ Maina MB , Ahmad U , Ibrahim HA , et al . Two decades of neuroscience publication trends in Africa. Nat Commun 2021;12:3429.doi:10.1038/s41467-021-23784-8 pmid:http://www.ncbi.nlm.nih.gov/pubmed/34103514 OpenUrlPubMed↵ Akinyemi RO , Owolabi MO , Oyeniyi T , et al . Neurogenomics in Africa: perspectives, progress, possibilities and priorities. J Neurol Sci 2016;366:213–23.doi:10.1016/j.jns.2016.05.006 pmid:http://www.ncbi.nlm.nih.gov/pubmed/27288810 OpenUrlPubMed↵ Akinyemi RO , Salami A , Akinyemi J , et al . Brain banking in low and middle-income countries: Raison D'être for the Ibadan brain ageing, dementia and neurodegeneration (Ibadan) brain bank project. Brain Res Bull 2019;145:136–41.doi:10.1016/j.brainresbull.2018.08.014 pmid:http://www.ncbi.nlm.nih.gov/pubmed/30149197 OpenUrlPubMed
No comment yet.
Scooped by Nesrin Shaheen
Scoop.it!

Autoimmune and infectious neurological disorders in 20 numbers –

Autoimmune and infectious neurological disorders in 20 numbers – | AntiNMDA | Scoop.it
Below are some autoimmune and infections-related numbers we thought you might want to explore: *** 24 causes of aseptic meningitis 28 causes of chronic meningitis 13 causes of recurrent meningitis 16 causes of hypertrophic pachymeningitis 33 causes of eosinophilic meningitis *** 37 causes of rhombencephalitis...
No comment yet.
Scooped by Nesrin Shaheen
Scoop.it!

Teaching NeuroImage: Atypical Unilateral Cortical Ribboning in Anti-N-methyl-D-aspartate Receptor Encephalitis | Neurology

Teaching NeuroImage: Atypical Unilateral Cortical Ribboning in Anti-N-methyl-D-aspartate Receptor Encephalitis | Neurology | AntiNMDA | Scoop.it
Main menu Neurology.org JournalsNeurology Clinical Practice Genetics Neuroimmunology & Neuroinflammation Education Online SectionsNeurology Video Journal Club Inclusion, Diversity, Equity, Anti-racism, & Social Justice (IDEAS) Innovations in Care Delivery Practice Buzz Practice Current Residents & Fellows Without Borders CollectionsCOVID-19 Disputes & Debates Health Disparities Infographics Neurology Future Forecasting Series Null Hypothesis Patient Pages Topics A-Z Translations Podcast CME AboutAbout the Journals Contact Us Editorial Board AuthorsSubmit a Manuscript Author Center Home Latest Articles Current Issue Past Issues Residents & Fellows User menu Subscribe My Alerts Log in Search Search for this keyword Meeting abstracts only Advanced search
No comment yet.
Scooped by Nesrin Shaheen
Scoop.it!

Anti- N-Methyl-d-Aspartate Receptor Antibody Testing in First-Episode Psychosis: Universal or Targeted Testing

Anti- N-Methyl-d-Aspartate Receptor Antibody Testing in First-Episode Psychosis: Universal or Targeted Testing | AntiNMDA | Scoop.it
Anti-<i>N</i>-methyl-d-aspartate receptor (NMDAR) encephalitis is an immune-mediated disorder that typically presents with rapid development of neuropsychiatric symptoms.As a potentially reversible cause of psychosis, there have been calls internationally for routine serological screening for anti-...
No comment yet.
Scooped by Nesrin Shaheen
Scoop.it!

IDIBAPS awarded three grants by the CaixaResearch 2022 Call for Proposals in Health Research

IDIBAPS awarded three grants by the CaixaResearch 2022 Call for Proposals in Health Research | AntiNMDA | Scoop.it
The projects selected, led by Elías Campo, Patrícia Pérez Galán and Josep Dalmau, are aimed at studying how cancer cells modulate the surrounding microenvi...
No comment yet.
Scooped by Nesrin Shaheen
Scoop.it!

Brain Sciences | Free Full-Text | Coexistence of Myelin Oligodendrocyte Glycoprotein Immunoglobulin G and Neuronal or Glial Antibodies in the Central Nervous System: A Systematic Review

Brain Sciences | Free Full-Text | Coexistence of Myelin Oligodendrocyte Glycoprotein Immunoglobulin G and Neuronal or Glial Antibodies in the Central Nervous System: A Systematic Review | AntiNMDA | Scoop.it
Background: Myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG) has been considered a diagnostic marker for patients with demyelinating disease, termed &ldquo;MOG-IgG associated disorder&rdquo; (MOGAD).
No comment yet.
Scooped by Nesrin Shaheen
Scoop.it!

Cognitive and psychiatric features of anti-NMDA receptor encephalitis

Cognitive and psychiatric features of anti-NMDA receptor encephalitis | AntiNMDA | Scoop.it
Diseases that are triggered by autoantibodies against brain cell targets are a growing
area of research in neurology and psychiatry, since immunotherapy can be an effective
treatment for patients. To date, most of the autoantibodies that have been discovered that are associated with these brain...
No comment yet.
Scooped by Nesrin Shaheen
Scoop.it!

Characterisation of the post-acute stage of anti-NMDA receptor encephalitis

Characterisation of the post-acute stage of anti-NMDA receptor encephalitis | AntiNMDA | Scoop.it
The study, published in the journal The Lancet Neurology, compares the cognitive and psychiatric symptoms of patients with those of schizophrenia and concl...
No comment yet.
Scooped by Nesrin Shaheen
Scoop.it!

Autoimmune Encephalitis in the Context of Rare Neuroimmune Disorders | 2022 Pre-RNDS

No comment yet.
Scooped by Nesrin Shaheen
Scoop.it!

Author Response: Neurofilament Light Chain Levels in Anti-NMDAR Encephalitis and Primary Psychiatric Psychosis | Neurology

Author Response: Neurofilament Light Chain Levels in Anti-NMDAR Encephalitis and Primary Psychiatric Psychosis | Neurology | AntiNMDA | Scoop.it
September 13, 2022; 99 (11) Disputes & Debates: Editors' Choice Author Response: Neurofilament Light Chain Levels in Anti-NMDAR Encephalitis and Primary Psychiatric Psychosis View ORCID ProfileMar Guasp, Josep Dalmau First published September 12, 2022, DOI: https://doi.org/10.1212/WNL.0000000000201181 Full PDF Citation Permissions Make Comment See Comments Downloads0 Share Article Info & Disclosures 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. We appreciate the comments of Dr. Vale on our study,1 reminding us that psychosis is a severe ailment and that patients may benefit from CSF studies. As shown by most of our work, we agree that CSF studies should be reincorporated in the evaluation of patients with new onset psychosis.1,2 Dr. Vale also indicates that there are other types of autoimmune encephalitis (AE), and the detection of NMDA receptors (NMDAR) antibodies is not essential for AE diagnosis. We are aware of this concept.3 As indicated in our manuscript, most cases of AE can associate with symptoms of psychosis, but the presence of concurrent neurologic alterations readily excludes a primary psychiatric etiology on clinical grounds. By contrast, the initial symptoms of anti-NMDAR encephalitis can mimic to perfection a first episode of psychosis due to a primary psychiatric disorder.2,4 For this reason, we narrowed our study to this specific AE. Regardless of whether neurofilament light chain (NfL) levels can be found to be elevated in many disorders with neuronal/axonal damage, determination of NfL showed clinical utility in the context of our study.FootnotesAuthor disclosures are available upon request (journal{at}neurology.org).See Editors' NoteSee letter© 2022 American Academy of NeurologyView 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. You May Also be Interested in Back to top Related Articles Editors' Note: Neurofilament Light Chain Levels in Anti-NMDAR Encephalitis and Primary Psychiatric Psychosis Reader Response: Neurofilament Light Chain Levels in Anti-NMDAR Encephalitis and Primary Psychiatric Psychosis Alert Me Alert me when eletters are published
No comment yet.
Scooped by Nesrin Shaheen
Scoop.it!

Autoimmune encephalitis after herpes simplex encephalitis: A still undefined condition

Autoimmune encephalitis after herpes simplex encephalitis: A still undefined condition | AntiNMDA | Scoop.it
Herpes simplex encephalitis (HSE) is one of the most common sporadic viral encephalitis.Generally, HSE is characterized by a monophasic short course, although in some patients neurological relapses or worsening of deficits can develop some weeks later, when viral therapy has been discontinued and...
No comment yet.
Scooped by Nesrin Shaheen
Scoop.it!

Genetic variation in genes of inborn errors of immunity in children with unexplained encephalitis | Genes & Immunity

Pediatric encephalitis has significant morbidity and mortality, yet 50% of cases are unexplained. Host genetics plays a role in encephalitis’ development; however, the contributing variants are poorly understood.
No comment yet.
Scooped by Nesrin Shaheen
Scoop.it!

Tocilizumab in Refractory Autoimmune Encephalitis: A Series of Pediatric Cases

Tocilizumab in Refractory Autoimmune Encephalitis: A Series of Pediatric Cases | AntiNMDA | Scoop.it
These findings support the efficacy and short-term safety of tocilizumab as a third-line treatment for refractory autoimmune encephalitis in children.
No comment yet.
Scooped by Nesrin Shaheen
Scoop.it!

Teaching NeuroImage: Dura Mater Thickening and Enhancement in Anti-NMDAR Encephalitis | Neurology

Teaching NeuroImage: Dura Mater Thickening and Enhancement in Anti-NMDAR Encephalitis | Neurology | AntiNMDA | Scoop.it
A 33-year-old man presented with baryglossia, memory disturbance, and seizures for a month. The workup for infectious and rheumatic disease was negative. Serum and CSF anti-NMDAR antibody were positive. MRI showed cortical and subcortical hyperintensities with adjacent pachymeningeal thickening and enhancement (Figure, A–D). Treatment with immunoglobulin and high-dose methylprednisolone produced significant improvement in the symptoms and resolution of changes on the posttreatment MRI (Figure, E–H).<img height="227" class="highwire-fragment fragment-image" alt="Figure" src="https://n.neurology.org/content/neurology/99/14/628/F1.medium.gif"; width="440">Download figure Open in new tab Download powerpoint Figure Neuroimaging (MRI) During the Course of the DiseaseFLAIR images (A and B, arrowheads) showed multiple cortical and subcortical hyperintensities with adjacent dura mater thickening and enhancement (C and D, arrows). The FLAIR hyperintensities and dural enhancement improved after treatment (E–H). FLAIR = fluid-attenuated inversion recovery.The frequently reported abnormalities on MRI in anti-NMDAR encephalitis are leptomeningeal enhancement and T2/FLAIR cortical and subcortical hyperintensities in the temporal lobe, followed by the frontal lobe, periventricular region, and cerebellum, rarely involving the dura mater.1,2Study FundingNo targeted funding reported.DisclosureThe authors report no relevant disclosures. Go to Neurology.org/N for full disclosures.Appendix Authors<img alt="Table" width="599" class="highwire-fragment fragment-image" src="https://n.neurology.org/content/neurology/99/14/628/T1.medium.gif"; height="341">FootnotesGo 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.Submitted and externally peer reviewed. The handling editor was Roy Strowd III, MD, Med, MS.Teaching slides links.lww.com/WNL/C217Received March 19, 2022.Accepted in final form June 17, 2022.© 2022 American Academy of NeurologyReferences1.↵Bacchi S, Franke K, Wewegama D, et al. Magnetic resonance imaging and positron emission tomography in anti-NMDA receptor encephalitis: a systematic review. J Clin Neurosci. 2018;52(1):54-59.OpenUrlCrossRefPubMed2.↵Suzuki H, Kitada M, Ueno S, et al. Anti-NMDAR encephalitis preceded by dura mater lesions. Neurol Sci. 2013;34(6):1021-1022.OpenUrlCrossRefPubMed
No comment yet.
Scooped by Nesrin Shaheen
Scoop.it!

Editor's Choice: The Frequency of Autoimmune N-Methyl-D-Aspartate Receptor Encephalitis Surpasses That of Individual Viral Etiologies in Young Individuals Enrolled in the California Encephalitis Pr...

Editor's Choice: The Frequency of Autoimmune N-Methyl-D-Aspartate Receptor Encephalitis Surpasses That of Individual Viral Etiologies in Young Individuals Enrolled in the California Encephalitis Pr... | AntiNMDA | Scoop.it
Background. In 2007, the California Encephalitis Project (CEP), which was established to study the epidemiology of encephalitis, began identifying cases of anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis.
No comment yet.
Scooped by Nesrin Shaheen
Scoop.it!

A Metabolic Brain Pattern Associated With Anti-N-Methyl-D-Aspartate Receptor Encephalitis

A Metabolic Brain Pattern Associated With Anti-N-Methyl-D-Aspartate Receptor Encephalitis | AntiNMDA | Scoop.it
Our findings suggest that 18F-FDG-PET should be included as an imaging tool when assessing affected patients in the clinical workup to rule out anti-NMDA encephalitis and help determine the most effective treatment.
No comment yet.
Scooped by Nesrin Shaheen
Scoop.it!

Autoimmune brain diseases: what you need to know

Autoimmune brain diseases: what you need to know | AntiNMDA | Scoop.it
In recent decades, neurology has evolved from localisation-based eponymous diagnoses
to molecular characterisation and classification. Neuroimmunology has received particular attention as many diagnostic mysteries have become answerable using an increasing array of IgG antibody diagnostic tests,...
No comment yet.
Scooped by Nesrin Shaheen
Scoop.it!

Autoimmune-associated epilepsy: Dr. Claude Steriade // International League Against Epilepsy

Autoimmune-associated epilepsy: Dr. Claude Steriade // International League Against Epilepsy | AntiNMDA | Scoop.it
Epigraph Vol. 24 Issue 3, Summer 2022 Autoimmune-associated epilepsy: Dr. Claude Steriade Reported by Dr. Maryam Nabavi-Nouri | Edited and produced by Nancy Volkers Dr. Claude Steriade talks with Dr. Maryam Nabavi-Nouri about distinguishing acute seizures with autoimmune cause from autoimmune-associated epilepsy—and why it can be difficult to tell the difference. She discusses the biological, clinical, and therapeutic differences between these two disease states, and what is known about effective diagnosis and treatment. Listen below or download the episode. Find Sharp Waves episodes on Spotify, Apple Podcasts, Google Podcasts, Amazon Music, iHeart Radio, and Stitcher. Podcast Transcript I’m Maryam Nabavi-Nouri; I’m a pediatric neurologist and epileptologist. I practice at the University of Western Ontario, in London, Ontario, Canada. Part of my research and clinical focus revolves around autoimmune mediated epilepsy. I’m interviewing Dr. Claude Steriade, from the New York University School of Medicine, about autoimmune mediated epilepsy and autoimmune encephalitis. Thank you for joining us, Claude, for a friendly chat and interview on this episode of Sharp Waves. I wanted to start by asking you how you became interested in this field, and what inspired you to focus on autoimmunity and epilepsy. Dr. Steriade: Sure. I think my interest in autoimmunity and neurology probably started in residency. I had a patient with autoimmune encephalitis, and I was a PGY2 (second-year resident) on the inpatient ward, and you know, that can sometimes be a little bit draining. You see a lot of patients who are very sick and not all of them get better. I had this patient who was very ill and responded beautifully to immunotherapy and it was like a breath of fresh air. This was a disease for which the pathophysiology was fascinating, and it also was extremely rewarding clinically in terms of therapeutic response. That’s probably where my clinical interest in that started. As I went through residency, I was really drawn to epilepsy for various reasons—probably also in part the really drastic response to treatment that many patients have – you can really turn people’s lives around, you know, in epilepsy as well. So when I went into fellowship in epilepsy, I decided to try to marry those two interests. In addition to my epilepsy fellowship, I joined in some of the autoimmune neurology clinics, at the Cleveland Clinic, and tried to gain some clinical experience there. And I noticed clinically that there were quite a few situations where there was relevant overlap between the two fields. A lot of patients with autoimmune encephalitis develop seizures, so whenever I was on the cEEG rotation, reading critically ill patients’ EEGs, there were patients in whom that overlap was relevant, so I started wondering if that relevance was there in other settings as well. That’s where my interest in autoimmune associated epilepsy came about, and I developed an interest in trying to hone down the phenotypic signatures of patients who may have an autoimmune mechanism for their epilepsy. When I came to NYU to start my first attending job, my only attending job, that’s what I continued doing. It’s a field that sort of has it all for me. Clinically it’s such an interesting area, these patients are fascinating to listen to, and you listen to their seizure history, their seizure semiology, when you listen to the other neurologic symptoms they have – they’re interesting to treat, they’re rewarding to treat for the most part, though obviously a lot of patients with autoimmune associated epilepsies can be therapy resistant and they can be very sick, but it can be a very rewarding process. I also continue to be fascinated by their biology, which is why I continue to focus my research effort on that patient population. Dr. Nabavi-Nouri: Can you very broadly tell us about our current knowledge of immune-mediated epilepsies in the field? Dr. Steriade: Yeah, and I think we’ve known for a long time that epilepsy in and of itself is a neuroinflammatory disease. There’s a broad literature on neuroinflammation and seizures and the bidirectional relationships between both. In patients whose epilepsy is structural in nature, for example in focal cortical dysplasias, or in any epilepsies, what’s been more, uh, new is that over the past decade or so there’s this entity of epilepsies in which neuroinflammation isn’t just necessarily an epiphenomenon which may feed back into furthering seizures, but it’s the originating or causative factor that is initiating the epilepsy in the first place. So here I’m not talking about patients who have structural lesions and they have seizures and there’s neuroinflammation. I’m talking about patients who are healthy, have no reason to develop seizures and all of the sudden develop hard to treat, drug-resistant seizures, often temporal lobe in onset, with associated psychiatric and cognitive comorbidities, in whom the original causative etiological problem is autoimmune in nature. And the reason we’ve been able to identify this is the discovery of various antibodies that target neuronal proteins, some of which target intracellular antigens like GAD65 and some of the onconeural antibodies, and some of which target cell surface antigens like LGI-1, CASPR-2, NMDA, GABA-B, and they each present in different ways, as we know, and can respond to immunotherapy, particularly the cell surface antibodies, much better than they might respond to conventional epilepsy treatments like antiseizure medications, epilepsy surgery and so on. And so that’s where really the advance has been and now there’s an interest in finding out well, are there patients out there who are walking, talking, with seizure disorders, showing up to our epilepsy clinics, some of them may be not responding to antiseizure medications or epilepsy surgery, who may have an autoimmune cause for their seizures that we’re not identifying because we’re not looking for it in some of these patients. The question is how many patients are there—not with phenotypically clear autoimmune encephalitis who are intubated in the ICU – how many patients are there in our epilepsy clinics with focal epilepsy of unknown cause who actually have an autoimmune problem? There’s been a number of studies in this area, they’re rigorous studies that tried to exclude patients who phenotypically very clearly have encephalitis. The studies that look at this question – how many patients with focal epilepsy have an autoimmune cause for their seizures, defined as the presence of an autoantibody, it’s about 5%. Which is pretty significant if you think about the number of patients who walk into clinics with focal epilepsy of unknown cause – 1 in 20 people have an autoimmune cause for their seizures and can potentially be diagnosed and treated with immunotherapy and potentially respond to it. Dr. Nabavi-Nouri: I would say it was a very revealing moment when that paper came out from the ILAE task force, and I would say you have contributed significantly to our understanding of this field. It’s definitely something that most physicians were struggling with, and once the distinction and the definitions came out, for a lot of us working in epilepsy it was like a breath of fresh air because you could coin it differently. What led to the definitions and the task force and the work that you guys published as a result? Dr. Steriade: Sure. So the history behind that paper was that this task force was created, probably while I was finishing fellowship, so 2018, by the ILAE, to try to improve the diagnosis and treatment of autoimmune-related seizure disorders. That task force is kind of unique because it’s a mix of epileptologists and immunologists. I was lucky enough to be selected – I was very junior when I was selected. I was in this room with the bigs of this field – Andrew McKeon, Jeff Britton, Sarosh Irani, and all these big names. And one of the main themes that came about in some of these initial meetings was well, what are we even talking about when we say autoimmune epilepsy? That term is all over the place in our clinics, in our electronic health records, the term autoimmune epilepsy comes up a lot, in the literature, the term autoimmune epilepsy if you do a PubMed search, comes up a lot. But what is it that we actually talking about when we say autoimmune epilepsy and if we asked different people in the room, we were getting different answers, and that’s a problem, if the experts don’t agree on that. In the end, the experts did agree that we’re talking about two problems and lumping them into one, and those two problems are biologically different, they behave differently clinically, and they should be tackled therapeutically differently. And they’re really sort of diseases that need to be addressed with a different mindset in terms of research. So they can’t be lumped in together. Those two entities should be distinguished. There’s the entity of acute symptomatic seizure secondary to autoimmune encephalitis. Those are just like acute symptomatic seizures from a traumatic brain injury – they are seizures that happen acutely because of an acute brain injury – in this case it’s an inflammatory brain injury – related to autoimmune encephalitis. For example, your patient with NMDA receptor encephalitis who’s admitted – she’s got psychiatric changes, catatonia, and then she has a seizure on the psych ward and gets transferred to neurology. Does that patient have epilepsy? No, she has acute symptomatic seizures from an acute brain problem that needs to be treated, and when you treat it with immunotherapy, almost all the time, not always but almost all the time, the seizures will resolve. Epilepsy is not that. Epilepsy is an enduring predisposition to recurrent unprovoked seizures. It’s a chronic condition, that’s why it’s so disabling. The other condition we outlined in the paper was autoimmune-associated epilepsy. That’s the patient showing up to your clinic with five-year history of bitemporal drug resistant epilepsy who’s also got a lot of memory impairment, and you know has comorbid depression, anxiety, she’s also got comorbid type 1 diabetes, very frequent seizures whenever you do an EEG, and then you do a GAD65 and she’s got titers through the roof. MRI shows bitemporal flare changes. That patient has GAD65 autoantibody associated epilepsy. The term “associated” was chosen very carefully. There was a thought okay, this is actual epilepsy, this is autoimmune epilepsy but then we paused and said well, autoimmune epilepsy implies this is a pure autoimmune problem, and therefore as you treat the autoimmune problem the epilepsy should go away. But is that the case? Not really, so that patient with five years history of bitemporal epilepsy and her MRI shows signal changes in the temporal lobes, that patient very often doesn’t respond to immunotherapy very well. Our GAD65 patients are very refractory. Is the issue that we’re not good at choosing the type of immunotherapy? Maybe, but maybe the other issue is that it’s not purely an autoimmune problem. In many of these patients there’s a structural substrate for their epilepsy. Many have findings consistent with hippocampal sclerosis on MRI, if you end up doing epilepsy surgery, many of them do have hippocampal sclerosis on histopathology. So it’s associated with an autoimmune problem, and in some patients the autoimmune part of their epilepsy may be the predominant part and if you treat that well, they may respond really well. But in some other patients, maybe it started out as mostly an autoimmune problem, and as the years have gone on it’s become mostly a structural problem. That’s why if you give them steroids, IVIG, Cytoxan and all the rest, they’re not going to get better because the hippocampus is fried. So that term “associated” was chosen carefully. Actually, Jackie French was the one who suggested that. I came to her and said you know there’s a problem, I feel like we’re going to mislead people with that term and she’s the one who suggested associated and it stuck. And once you sort of put that on paper, and I’m glad it was well received, I think most people were like okay, clearly there’s two different categories of patients. Now operationally, I feel like there are patients who will go from one category to another, like I have LGI-1 patients, they’ve had LGI-1 for weeks to months, I treat them and not all of them respond. I used to think it was me who didn’t know what I was doing and then the Mayo group came out with their long-term data and even Mayo has some patients at 2 years who still seize – about 20%. So how does this LGI-1 patient who had encephalitis, you’ve been treating him with immunotherapy and they’re continuing to have seizures and it’s been 2 years – does that patient now have epilepsy? Do they still have encephalitis? I think it comes down, the issue of distinguishing in some of these patients who are transitioning from the acute phase to the chronic phase, the issue of how we’re going to define them as acute symptomatic versus autoimmune-associated epilepsy comes down to a lack of really good biomarkers for active autoimmunity and active inflammation. Antibody titers aren’t great. In NMDA, antibody titers tend to follow clinical course, but not always. There are patients that have NMDA antibodies when they’re doing really well after years and years. Same with LGI-1. We don’t have great ways of trying to track whether we’re morphing from an inflammatory problem to a structural one. That means it’s a problem therapeutically in terms of clinical decision making. Why does it matter to have two different categories? What’s the impact of that on your clinical decision making, and on your counseling of patients? Well the impact of that is A, patients with acute symptomatic seizures secondary to autoimmune encephalitis tend to do better, have a better prognosis, than patients with autoimmune associated epilepsy, so it helps in your counseling patients. And B, it helps you in terms of how hard to push immunotherapy. I generally always do a trial of immunotherapy when I make a new diagnosis of, whether it’s autoimmune encephalitis or autoimmune associated epilepsy, I mean you’re going to treat both but your expectations for response are going to be different. Not every autoimmune-associated epilepsy patient behaves the same, and not every autoimmune encephalitis patient behaves the same. There are a number of factors that come into play – the type of antibody, how long they’ve been sick, presence of an underlying malignancy and so on. Having these two categories are the starting point for expectations in counseling, expected response to immunotherapy and decisions on escalation of immunotherapy. You’re for sure going to escalate to second-line therapy in an autoimmune encephalitis case that’s not responding to first line. In a patient that has autoimmune-associated epilepsy and has zero response to first-line immunotherapy, we don’t know what to do. These patients are usually very sick, and I do tend to try many different therapies, being very open with them that there might not be a response. But we don’t know that that’s the right thing to do, to give someone with GAD65 antibody associated epilepsy Cytoxan when we have clear hippocampal sclerosis on the MRI if they haven’t responded to steroids/IVIG – there are many physicians who would not do that, and they would not be wrong. I think trying to operationalize the distinction will be important to sort of make it easier for clinicians to do these things that I just talked about – accurately prognosticate and make clinical decisions. I think it’s going to come down to having better biomarkers of active neuroinflammation, active brain autoimmunity in these patients, to inform us whether there’s untreated brain autoimmunity that would benefit from aggressive immunotherapy. Dr. Nabavi-Nouri: And to that point, we don’t have any set time points. Of course, these are patients that are pretty complex so there’s never, I don’t think there’s been a consensus in terms of whether we use a timeline to decide patients fall on this side of the spectrum versus the other. Dr. Steriade: That’s a really important comment. There was a discussion about that in the task force – do we say that if you’re still having seizures at one year, do you then have epilepsy? And the decision was made to not have that timeline because it was felt that A, there’s so much heterogeneity in the disease between patients and B, there are some patients who come to us late. They haven’t been diagnosed. I’ve seen an LGI-1 patient who came to me a year after she first got diagnosed and she responded beautifully to steroids, thankfully. Just because she hadn’t been identified as having LGI-1 does that mean she didn’t have encephalitis anymore at the magic 365-day mark? No! Because of that we decided to not have a timeline and instead say that whether someone falls into one or the other category comes down to the whole clinical picture, whether there’s ancillary evidence of an active autoimmune encephalitis, whether they have active CSF, an MRI showing signs encephalitis and so on, uh, to make that decision, that distinction, but you know it’s not perfect. Dr. Nabavi-Nouri: In what subset of patients with drug-resistant epilepsy do you consider an autoimmune cause that you would go the extra mile of investigating them and how far? How extensive do you investigate them? Dr. Steriade: So there’s a couple of groups that have tried to answer this. There’s the Dutch group, Martin Titulaer’s group, developed a score called the ACES score, the antibodies contributing to epilepsy and seizures score, in which they enrolled patients who did not have a clear encephalitis clinically and tested them for neural autoantibodies – those are all patients with focal epilepsy of unknown cause – and then developed a score which indicates whether you’re likely to find an antibody or not. There were various elements in there which were kind of reminiscent of encephalitis – behavioral changes, autonomic changes and so on. The Oxford group, Sarosh Irani’s group, also came up with a score, which they didn’t give a catchy name to, with various other clinical features – they also included piloerection semiology, retention scores, absence of epilepsy risk factors. Their score wasn’t validated in an independent cohort. But there are tools out there. Are those tools perfect? No. they’re a starting point. We’re trying to develop a score that would hopefully be a little bit easier to implement operationally speaking, because what does it mean, “cognitive changes” and “behavioral changes” in an epilepsy patient? That can be kind of hard to define, and different epileptologists might have different interpretations. So there are ways we can improve on the ACES score. We’re doing a multicenter study looking at patients with not only focal epilepsy of unknown cause but also disease control cohorts and really systematically phenotyping them and testing them for antibodies and whether they’re antibody positive in serum, testing CSF as well, and in both cohorts doing serum and CSF in hopes of defining the phenotype better. I think a core issue in some of the prior studies is that the basic assumption is that positive antibody equals antibody-associated epilepsy, and no antibody equals no antibody-associated epilepsy. But if you examine these statements there are potential issues, right, so. The way that “positive antibody” is defined actually is different between reputable places. Some places will count a positive anti-glycine as a definite positive. Other places will say well, that assay is problematic and there can be false positives. Different places will count low-titer CASPR-2 differently. The other aspect of the assumption, that if you don’t have an antibody you definitely don’t have autoimmune associated epilepsy, also is potentially problematic. There may be antibodies out there that we haven’t defined. And also maybe not every autoimmune associated epilepsy is antibody mediated. So we all have these patients that we could have sworn that their panels would come back positive. They walked and talked and smelled autoimmune, you know? They had late-onset bitemporal epilepsy that just exploded, they had an abnormal MRI with bitemporal changes, they had very rapid cognitive decline – they had to be autoimmune, and their panel comes back negative. So what we’re trying to do in our current study is have a more clean control cohort because in all prior studies, they only looked at focal epilepsy of unknown cause and they divided antibody positive versus negative, but it makes you think like, both of those groups may have some noise in them. So by having a disease control group, which means a patient with a clear cause for their epilepsy, like they have a malformation of cortical development or some other clear cause that’s not autoimmune, then you know for sure that when you’re trying to do your analysis and compare your autoimmune to your non-autoimmune group that your non-autoimmune group is definitely non-autoimmune. Dr. Nabavi-Nouri: Glad you brought this up. I was actually going to ask you about this influx of multiple different scoring systems that clinically apply to different categories, different disease/epilepsy categories, in particular the APE/APE2 score and the ACES score. What would you see as the next step in unifying our approach and better implementing these various scores in clinical practice? Dr. Steriade: Yeah. So I have no hard data, like I didn’t do a randomized clinical trial to prove that one score is better than the other. This is just my opinion, but we wrote a systematic review, an opinion piece in JAMA Neurology last year, and what we ended up recommending in that paper was that if you think the patient has acute symptomatic seizures from autoimmune encephalitis, then use the APE2 score. Because really the APE2 score uses many elements that are autoimmune encephalitis related. And if you think your patient may have an autoimmune associated epilepsy, then use the ACES score, because that was developed explicitly in an epilepsy group. What the workup should be – definitely serum and CSF in all suspected autoimmune encephalitis – I think that’s really standard. The question is that if you have a patient with suspected autoimmune-associated epilepsy, does every patient need to be tapped? I tend to tap most. There’s no clear evidence right now that that’s necessary – most of the antibodies you’re going to find are antibodies that have pretty good sensitivity in serum like CASPR-2, GAD-65. I’ve seen some chronic LGI-1s that show up in clinic. The antibodies that have high sensitivity in CSF like NMDA, GABA-B, those don’t tend to show up in an epilepsy clinic, so it may not be the right thing to do to tap everybody. In the JAMA Neurology paper we sort of said “consider CSF” to leave it up to people what they do in the autoimmune epilepsy suspected cases. Dr. Nabavi-Nouri: One of the questions that lies heavy with me is how long you treat your patients for who have a diagnosis or who you’re convinced have a diagnosis of autoimmune-associated epilepsy. Dr. Steriade: I think the issue of how long to treat is an important one and a difficult one. We don’t have great guidelines. There is now a consensus publication on long-term management of autoimmune encephalitis, that was published in JNNP (the Journal of Neurology, Neurosurgery & Psychiatry) this year or last. And even then, it’s not – different experts will treat for different lengths of time after an autoimmune encephalitis to prevent relapses. And then the autoimmune associated epilepsy groups, it’s even less sort of clear what to do. I think generally, if someone responds, I keep them on for at least a year before thinking about tapering off. Sometimes I do trials of response, and 6 to 12 weeks is usually a good duration of treatment to see if someone’s steroid responsive or immunotherapy responsive to decide what to do next. I think it’s really important in these patients to have a clear idea in your mind, like before you do an immunotherapy trial – like, what are going to be my parameters in terms of deciding if I was successful or not? Because you know, many patients feel better on steroids, or some patients feel worse after their IVIG. There needs to be some objective marker of response, whether that’s a seizure diary, cognitive testing, having objective markers of response and in a very clear sort of pre-treatment plan, of what you’re going to consider a success or a failure, can be helpful because it’s kind of easy in these patients to end up in this sort of loop of endless treatments and then realize well, am I making this person better, or am I exposing them to really toxic therapies? Dr. Nouri: I wanted to conclude by talking about your, personally about your successful career as a young clinician investigator and what advice you would have to our listeners, especially as it pertains to forming networks and securing grants? Dr. Steriade: I think the most important thing is to choose to do something that you love. Because it’s a lot of hard work, and if there’s a grant deadline coming up and there are late nights, and if you don’t have joy for what you’re writing about, it’s going to feel like very, very hard work. If there’s joy, then it’s not going to feel like work. So I think like for me, finding something that I felt passionate about and still feel passionate about is really why I think I’ve been successful, is because I still feel excited even when the circumstances are objectively a little bit difficult and the hours are long, you know. Finding something that you love, sticking with people who support you and believe in you. I’ve been so lucky – every institution I’ve been in, I’ve had incredible mentors. And maybe I’m biased because I’m a young woman, but they’ve been women who get it and who have been through the same sort of challenges that let’s face it, sometimes young women are faced with different types of challenges than our male counterparts. I’m not saying our male counterparts don’t have challenges but they’re different. But when I was in fellowship my mentor there, Lara Jehi, was just incredible – she is the reason I really believed in myself and ended up really pursuing this seriously. She looked at me and she was like, “You are going to end up being an autoimmune epilepsy expert.” Having somebody who believes in you – it’s true when you’re a kid and it’s true when you’re an adult too – someone needs to believe in you and give you a belief in yourself. And then I came here and I had Jackie French, who’s like my work mom and she believed in me, and she makes me believe in myself. And obviously having mentors who have the intellectual ability to help you, the network ability to help you, has been huge. I wouldn’t be where I am now if it hadn’t been for those two and many more people who were able to read my grants and read my papers and link me with the people who would help me do the work. Dr. Nouri: Having trained with you from early days, I truly admire what you’ve accomplished and your energy and the knowledge you brought to the field. Because of the love you have for the field you’ve been able to contribute so much to it. Dr. Steriade: Thanks Maryam. It’s always so much fun talking about this stuff with you. Related publications Discerning the Role of Autoimmunity and Autoantibodies in Epilepsy: A Review Acute symptomatic seizures secondary to autoimmune encephalitis and autoimmune-associated epilepsy: Conceptual definitions About Dr. Steriade Claude Steriade, MD, CM is originally from Quebec City, Canada, and completed medical school at McGill University in Montreal, then an adult neurology residency at the University of Toronto. She then completed a two-year epilepsy fellowship at the Cleveland Clinic before joining the Epilepsy Center at NYU.
No comment yet.
Scooped by Nesrin Shaheen
Scoop.it!

Neuropsychological functioning in children and adolescents with anti-NMDA receptor encephalitis (anti-NMDARE)

Neuropsychological functioning in children and adolescents with anti-NMDA receptor encephalitis (anti-NMDARE) | AntiNMDA | Scoop.it
The objective of this study was to describe neuropsychological functioning and associated medical features in pediatric patients with anti-NMDA receptor encephalitis (anti-NMDARE). Retrospective data were collected from electronic medical records and neuropsychological reports of 15 children and...
No comment yet.
Scooped by Nesrin Shaheen
Scoop.it!

Neuroleptic intolerance in the context of anti-N-methyl-D-aspartate receptor encephalitis: A systematic review and synthesis of global case reports

Neuroleptic intolerance in the context of anti-N-methyl-D-aspartate receptor encephalitis: A systematic review and synthesis of global case reports | AntiNMDA | Scoop.it
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis presents commonly with psychiatric symptoms. One cohort of these patients reported that antipsychotic administration led to neuroleptic intolerance (NI) in 19% of them, which was preventable by a prompt encephalitis diagnosis.
No comment yet.
Scooped by Nesrin Shaheen
Scoop.it!

Anti–NMDA-Receptor Encephalitis

Anti–NMDA-Receptor Encephalitis | AntiNMDA | Scoop.it
PreviousNext Cases from the Cooky JarFree AccessAnti–NMDA-Receptor EncephalitisMichail E. Klontzas , Ciléin Kearns, Sara Sheikhbahaei, Patricia CornejoAuthor AffiliationsPublished Online:Sep 16 2022https://doi.org/10.1148/rg.220173Anti–N-methyl-d-aspartate (NMDA)–receptor encephalitis (ANMDARE) is a rare condition characterized by the presence of autoantibodies against the NR1 and NR2 subunits of the NMDA receptor (Fig 1). Patients typically present with psychiatric symptoms (eg, anxiety, hallucinations, psychosis) and may experience progression to sleep disorders, seizures, dyskinesias, and autonomic instability. The imaging features of ANMDARE can vary but usually include brain atrophy with temporal lobe predilection (1,2). Asymmetric temporal lobe or cerebellar cortical hyperintensity on fluid-attenuated inversion-recovery (FLAIR) MR images can also be a feature of the disease and may be accompanied by restricted diffusion (1,3) (Fig 2).ANMDARE can present as a paraneoplastic manifestation of tumors expressing the same receptors (NR1, NR2), most commonly ovarian teratomas, which may contain mature and immature neurons expressing subunits of the NMDA receptor (2) (Figs 3, 4). Diagnosis is based on a combination of imaging and laboratory findings, which include serum and cerebrospinal fluid (CSF) autoantibodies, CSF pleocytosis, and/or high CSF protein concentration (1,2). Immunosuppressive therapy (steroids and intravenous immunoglobulin) and tumor resection, if tumor is present, lead to complete remission in most cases (4).Disclosures of conflicts of interest.—C.K. Editorial board member of RadioGraphics.C.K. has provided disclosures (see end of article); all other authors have disclosed no relevant relationships.References1. Dalmau J, Tüzün E, Wu HY, et al. Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol 2007;61(1):25–36. Crossref, Medline, Google Scholar2. Bassal FC, Harwood M, Oh A, et al. Anti-NMDA receptor encephalitis and brain atrophy in children and adults: A quantitative study. Clin Imaging 2021;78:296–300. Crossref, Medline, Google Scholar3. Magudia K, Menias CO, Bhalla S, Katabathina VS, Craig JW, Hammer MM. Unusual imaging findings associated with germ cell tumors. RadioGraphics 2019;39(4):1019–1035. Link, Google Scholar4. Dalmau J, Rosenfeld MR. Paraneoplastic syndromes of the CNS. Lancet Neurol 2008;7(4):327–340. Crossref, Medline, Google ScholarArticle HistoryReceived: July 14 2022Accepted: July 15 2022Published online: Sept 16 2022 FiguresReferencesRelatedDetails Recently Published Metrics PDF download
Scooped by Nesrin Shaheen
Scoop.it!

Parents Battle For Cure For Toddler Plagued By Rare Disease

Parents Battle For Cure For Toddler Plagued By Rare Disease | AntiNMDA | Scoop.it
The parents of an adorable toddler who suffers from a rare syndrome for which there are no known treatments are fighting to find a cure for...
No comment yet.
Scooped by Nesrin Shaheen
Scoop.it!

Editors' Note: Neurofilament Light Chain Levels in Anti-NMDAR Encephalitis and Primary Psychiatric Psychosis | Neurology

Editors' Note: Neurofilament Light Chain Levels in Anti-NMDAR Encephalitis and Primary Psychiatric Psychosis | Neurology | AntiNMDA | Scoop.it
September 13, 2022; 99 (11) Disputes & Debates: Editors' Choice Editors' Note: Neurofilament Light Chain Levels in Anti-NMDAR Encephalitis and Primary Psychiatric Psychosis View ORCID ProfileAravind Ganesh, View ORCID ProfileSteven Galetta First published September 12, 2022, DOI: https://doi.org/10.1212/WNL.0000000000201179 Full PDF Citation Permissions Make Comment See Comments Downloads0 Share Article Info & Disclosures 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. Dr. Guasp et al. assessed the performance of serum neurofilament light chain (NfL) testing in differentiating anti-NMDA receptor encephalitis (NMDARe) from psychiatric causes of a first episode of psychosis (pFEP) in an observational study of 118 patients with NMDARe (33 of whom had isolated psychosis at presentation), comparing them with 45 patients with pFEP, 36 patients with herpes simplex encephalitis (HSV), and 36 healthy controls. They found that young patients with first episode psychosis and serum NfL ≥15 pg/mL had a 120 times higher chance of having NMDARe than pFEP, with this cutoff correctly classifying 96% of patients with pFEP and 85% of patients with NMDARe and isolated psychosis. Notably, all patients with HSV also had serum NFL≥15 pg/mL. The authors concluded that patients with first episodes of psychosis of unclear etiology and serum NfL ≥15 pg/mL should undergo testing for NMDAR antibodies in the CSF. In response, Dr. Vale notes that the presence of NMDAR antibodies is not essential for diagnosing autoimmune encephalitis, given that other autoantibodies can cause psychotic presentations, and that elevations of NfL may reflect comorbidities causing central or peripheral neuroaxonal damage. Dr. Vale argues that given the severity of first episodes of psychosis, all such patients should be considered for CSF examinations, as was the historical practice for concerns of neurosyphilis. Responding to these comments, the authors agree that CSF studies should be reincorporated in the evaluation of patients with new onset psychosis, but note that they restricted their study to the identification of NMDARe, given that this condition can almost perfectly mimic psychiatric causes of first episodes of psychosis, in contrast to other autoimmune encephalitides, which typically have other concurrent neurologic features. They argue that the usefulness of serum NfL testing seen in their study is a relevant finding, regardless of any other underlying contributors to NfL elevation. This exchange highlights the importance of ongoing research into noninvasive markers of neurologic causes of psychosis. It is the editors' opinion that in practice, routine CSF testing for all patients with first episodes of psychosis is unlikely to be feasible in all psychiatric facilities, and as such, screening using serum markers will likely complement clinical examination-guided autoantibody testing in the future.Dr. Guasp et al. assessed the performance of serum neurofilament light chain (NfL) testing in differentiating anti-NMDA receptor encephalitis (NMDARe) from psychiatric causes of a first episode of psychosis (pFEP) in an observational study of 118 patients with NMDARe (33 of whom had isolated psychosis at presentation), comparing them with 45 patients with pFEP, 36 patients with herpes simplex encephalitis (HSV), and 36 healthy controls. They found that young patients with first episode psychosis and serum NfL ≥15 pg/mL had a 120 times higher chance of having NMDARe than pFEP, with this cutoff correctly classifying 96% of patients with pFEP and 85% of patients with NMDARe and isolated psychosis. Notably, all patients with HSV also had serum NFL≥15 pg/mL. The authors concluded that patients with first episodes of psychosis of unclear etiology and serum NfL ≥15 pg/mL should undergo testing for NMDAR antibodies in the CSF. In response, Dr. Vale notes that the presence of NMDAR antibodies is not essential for diagnosing autoimmune encephalitis, given that other autoantibodies can cause psychotic presentations, and that elevations of NfL may reflect comorbidities causing central or peripheral neuroaxonal damage. Dr. Vale argues that given the severity of first episodes of psychosis, all such patients should be considered for CSF examinations, as was the historical practice for concerns of neurosyphilis. Responding to these comments, the authors agree that CSF studies should be reincorporated in the evaluation of patients with new onset psychosis, but note that they restricted their study to the identification of NMDARe, given that this condition can almost perfectly mimic psychiatric causes of first episodes of psychosis, in contrast to other autoimmune encephalitides, which typically have other concurrent neurologic features. They argue that the usefulness of serum NfL testing seen in their study is a relevant finding, regardless of any other underlying contributors to NfL elevation. This exchange highlights the importance of ongoing research into noninvasive markers of neurologic causes of psychosis. It is the editors' opinion that in practice, routine CSF testing for all patients with first episodes of psychosis is unlikely to be feasible in all psychiatric facilities, and as such, screening using serum markers will likely complement clinical examination-guided autoantibody testing in the future.FootnotesAuthor disclosures are available upon request (journal{at}neurology.org).See letterSee responseReceived July 15, 2022.Accepted in final form July 15, 2022.© 2022 American Academy of NeurologyView 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. You May Also be Interested in Back to top Related Articles Reader Response: Neurofilament Light Chain Levels in Anti-NMDAR Encephalitis and Primary Psychiatric Psychosis Author Response: Neurofilament Light Chain Levels in Anti-NMDAR Encephalitis and Primary Psychiatric Psychosis Alert Me Alert me when eletters are published
No comment yet.