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Mycophenolate mofetil in paediatric autoimmune or immune‐mediated diseases of the central nervous system: clinical experience and recommendations - Nosadini - - Developmental Medicine & Child N...

Aim To gather data on mycophenolate mofetil (MMF) in paediatric autoimmune/immune‐mediated central nervous system (CNS) conditions, focusing on safety and factors that may affect MMF efficacy. Method Retrospective, multicentre study based on four paediatric neurology centres. Results Forty‐four children were included (30 females, 14 males): 19 had proven/suspected autoimmune encephalitis, 14 had inflammatory demyelinating CNS diseases, and 11 had other autoimmune/immune‐mediated CNS conditions. Before MMF, all received first‐line immune therapies, and 17 had second‐line rituximab and/or cyclophosphamide. MMF was started at a median of 9.5 months from disease onset (range 1–127mo) (median age 9y 4mo, range 1y 5mo–16y 5mo), and was used for median 18 months (range 0.3–73mo). On MMF, 31 patients were relapse‐free, whereas eight relapsed (excluding patients with chronic–progressive course). Relapses on MMF were associated with medication weaning/cessation, or with suboptimal MMF dosage/duration. Adverse events of MMF occurred in eight patients: six moderate (gastrointestinal, movement disorder, dermatological) and two severe (infectious). Interpretation MMF use in paediatric neuroimmunology is heterogeneous, although relatively safe. We have identified factors that may affect MMF efficacy and provide recommendations on MMF usage. What this paper adds Mycophenolate mofetil (MMF) use was heterogeneous with relatively common adverse events, although mostly not severe. MMF treatment reduced median annualized relapse rate, although 20% of patients relapsed on MMF. A high relapse rate pre‐MMF and late MMF start were associated with higher probability of relapsing on MMF. Most relapses were associated with suboptimal MMF dosage, short MMF duration, or concurrent medication weaning/discontinuation.
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First 'brain on fire' case identified in Cape Breton | SaltWire

First 'brain on fire' case identified in Cape Breton | SaltWire | AntiNMDA | Scoop.it
Sydney woman's rare disease left family, doctors thinking it was mental illness not physical
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Pediatric Autoimmune Encephalitis and Its Relationship With Infection | Read by QxMD

Pediatric Autoimmune Encephalitis and Its Relationship With Infection | Read by QxMD | AntiNMDA | Scoop.it
Create a free QxMD account to take advantage of the features offered by Read like saving your papers and creating collections. Get Started JOURNAL ARTICLEREVIEW Pediatric Autoimmune Encephalitis and Its Relationship With Infection Qinrui Li, Na Fu, Ying Han, Jiong Qin Pediatric Neurology 2021 April 7, 120: 27-32 Autoimmune encephalitis (AE) is an increasingly recognized inflammatory disorder of the central nervous system and is most often characterized by antibodies against intracellular and neuronal surface antigens. AE is a devastating disease that may result in developmental delay or regression in children. However, the pathogenesis of AE is not clear, and immune system disorders after infection likely play an important role in AE. Many studies have reported that patients with herpes simplex virus encephalitis develop anti-N-methyl-d-aspartate receptor encephalitis after antiviral treatment. It is critical to recognize pediatric AE early and to distinguish it from infectious forms because AE is treatable and responsive to immunotherapies. In this review, we discuss the clinical features of pediatric AE and focus on the relationship between AE and postinfection status. In addition, we review the probable mechanisms underlying infection-triggered AE, which include molecular mimicry, bystander activation, epitope spreading, immune system disorder, and genetic susceptibility. Full Text Links We have located links that may give you full text access. Full Text Web Additional links Discussion You are not logged in. Sign Up or Log In to join the discussion. Trending Papers US Case Reports of Cerebral Venous Sinus Thrombosis With Thrombocytopenia After Ad26.COV2.S Vaccination, March 2 to April 21, 2021. Isaac See, John R Su, Allison Lale, Emily Jane Woo, Alice Y Guh, Tom T Shimabukuro, Michael B Streiff, Agam K Rao, Allison P Wheeler, Suzanne F Beavers, Anna P Durbin, Kathryn Edwards, Elaine Miller, Theresa A Harrington, Adamma Mba-Jonas, Narayan Nair, Duong T Nguyen, Kawsar R Talaat, Victor C Urrutia, Shannon C Walker, C Buddy Creech, Thomas A Clark, Frank DeStefano, Karen R Broder JAMA 2021 April 30 Preliminary Findings of mRNA Covid-19 Vaccine Safety in Pregnant Persons. Tom T Shimabukuro, Shin Y Kim, Tanya R Myers, Pedro L Moro, Titilope Oduyebo, Lakshmi Panagiotakopoulos, Paige L Marquez, Christine K Olson, Ruiling Liu, Karen T Chang, Sascha R Ellington, Veronica K Burkel, Ashley N Smoots, Caitlin J Green, Charles Licata, Bicheng C Zhang, Meghna Alimchandani, Adamma Mba-Jonas, Stacey W Martin, Julianne M Gee, Dana M Meaney-Delman New England Journal of Medicine 2021 April 21 Advances in Clinical Cardiology 2020: A Summary of Key Clinical Trials. Aileen Kearney, Katie Linden, Patrick Savage, Ian B A Menown Advances in Therapy 2021 April 12 Palpitations in the Clinic. Jacob L Ransom, Ka C Wong Circulation 2021 April 20, 143 (16): 1631-1634 Neurologic Assessment of the Neurocritical Care Patient. Shane Musick, Anthony Alberico Frontiers in Neurology 2021, 12: 588989 The Role of Procalcitonin in Antibiotic Decision-Making in Covid-19 Infection. Valeria Fabre, Sara Karaba, Joe Amoah, Matthew Robinson, George Jones, Kathryn Dzintars, Morgan Katz, B Mark Landrum, Sarojini Qasba, Pooja Gupta, Eili Klein, Sara E Cosgrove Infection Control and Hospital Epidemiology 2021 April 19, : 1-24 Coronary Microvascular Dysfunction. Shigeo Godo, Akira Suda, Jun Takahashi, Satoshi Yasuda, Hiroaki Shimokawa Arteriosclerosis, Thrombosis, and Vascular Biology 2021 March 25, : ATVBAHA121316025 Perioperative hypotension 2021: a contrarian view. Teus Kappen, William Scott Beattie British Journal of Anaesthesia 2021 April 23
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Autoimmune Encephalitis Related to Cancer Treatment With Immune Checkpoint Inhibitors: Systematic Review

Autoimmune Encephalitis Related to Cancer Treatment With Immune Checkpoint Inhibitors: Systematic Review | AntiNMDA | Scoop.it
ICPI-AIE consists of a heterogenous group of conditions. Neurologists will likely encounter ICPI-AIE more often in the future, but important unresolved questions include the exact pathophysiological mechanisms, the epidemiology and the best treatment approaches associated with ICPI-AIE.
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Overlapping syndrome of anti-N-methyl-D-aspartate receptor encephalitis and anti-myelin oligodendrocyte glycoprotein inflammatory demyelinating diseases: A distinct clinical entity? - ScienceDirect

Overlapping syndrome of anti-N-methyl-D-aspartate receptor encephalitis and anti-myelin oligodendrocyte glycoprotein inflammatory demyelinating diseases: A distinct clinical entity? - ScienceDirect | AntiNMDA | Scoop.it
The co-existence of anti-N-methyl-D-aspartate receptor encephalitis (NMDARe) and anti-myelin oligodendrocyte glycoprotein (MOG) antibody disease has s…...
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Anti N‐Methyl‐D‐Aspartate (NMDA) receptor encephalitis: from psychosis to cognitive impairment

Anti N‐Methyl‐D‐Aspartate (NMDA) receptor encephalitis: from psychosis to cognitive impairment | AntiNMDA | Scoop.it
In this study, while presenting a clinical case with early psychiatric manifestations, we emphasized the need to pay attention to neurological diagnoses such as Anti N‐Methyl‐D‐Aspartate Receptor (NMDAR) encephalitis at the time ...
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“Antibody of Unknown Significance” (AUS): The Issue of Interpreting Antibody Test Results - Balint - - Movement Disorders - Wiley Online Library

“Antibody of Unknown Significance” (AUS): The Issue of Interpreting Antibody Test Results - Balint - - Movement Disorders - Wiley Online Library | AntiNMDA | Scoop.it
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Autoimmune encephalitis in children

Autoimmune encephalitis in children | AntiNMDA | Scoop.it
The causes of encephalitis are numerous, and extensive investigations for infectious agents and other etiologies are often negative. The discovery that many of these encephalitis are immune mediated has changed the approach to the diagnosis and treatment of these disorders.
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SARS-CoV-2-related encephalitis with prominent parkinsonism: clinical and FDG-PET correlates in two patients | SpringerLink

SARS-CoV-2-related encephalitis with prominent parkinsonism: clinical and FDG-PET correlates in two patients | SpringerLink | AntiNMDA | Scoop.it
Considering the similarities with other pandemics due to respiratory virus infections and subsequent development of neurological disorders (e.g. encephalitis lethargica after the 1918 influenza), there is growing concern about a possible new wave of neurological complications following the...
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The challenge of diagnosing and successfully treating anti-NMDA receptor encephalitis in a toddler

The challenge of diagnosing and successfully treating anti-NMDA receptor encephalitis in a toddler | AntiNMDA | Scoop.it
Anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis is an immune-mediated syndrome that is still under-recognised, with grave consequences if not treated early.A multidisciplinary team approach is required in the process of diagnosis and management of this potentially treatable and reversib...
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Evaluation of the Diagnostic Criteria for Anti-NMDA Receptor Encephalitis in Japanese Children | Neurology

Evaluation of the Diagnostic Criteria for Anti-NMDA Receptor Encephalitis in Japanese Children | Neurology | AntiNMDA | Scoop.it
Abstract Objective To evaluate the validity of the 2016 clinical diagnostic criteria proposed for probable anti-NMDA receptor (NMDAR) encephalitis in children, we tested the criteria in a Japanese pediatric cohort. Methods We retrospectively reviewed clinical information of patients with neurologic symptoms whose CSF was analyzed for NMDAR antibodies (NMDAR-Abs) in our laboratory from January 1, 2015, to March 31, 2019. Results Overall, 137 cases were included. Of the 41 cases diagnosed as probable anti-NMDAR encephalitis (criteria-positive) according to the 2016 criteria, 13 were positive and 28 were negative for anti–NMDAR-Abs. Of the 96 criteria-negative cases, 3 were positive and 93 were negative for anti–NMDAR-Abs. The sensitivity of the criteria was 81.2%, specificity was 76.9%, positive predictive value (PPV) was 31.7%, and negative predictive value was 96.9%. Compared with the true-positive group, the false-positive group contained more male than female patients (male:female, 4:9 in the true-positive vs 19:9 in the false-positive group, p = 0.0425). The majority of the cases with false-positive diagnoses were associated with neurologic autoimmunity. Conclusion The clinical diagnostic criteria are reliable for deciding to start immunomodulatory therapy in the criteria-positive cases. Low PPV may be caused by a lower prevalence of NMDAR encephalitis or lower level of suspicion for encephalitis in the pediatric population. Physicians should therefore continue differential diagnosis, focusing especially on other forms of encephalitis. Classification of Evidence This study provides Class IV evidence that the proposed diagnostic criteria for anti-NMDAR encephalitis in children has a sensitivity of 81.2% and a specificity of 76.9%. Glossary AMPAR=α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor; CASPR2=contactin-associated protein-like 2; CI=confidence interval; GABABR=γ-aminobutyric acid B receptor; GFP=green fluorescence protein; HSV=herpes simplex virus; IgG=immunoglobulin G; LGI1=leucine-rich glioma-inactivated 1; MOG=myelin oligodendrocyte glycoprotein; NMDAR=NMDA receptor; NMDAR-Abs=NMDA receptor antibodies; PPV=positive predictive value Footnotes Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article. Class of Evidence: NPub.org/coe CME Course: NPub.org/cmelist Received June 12, 2020. Accepted in final form January 25, 2021. © 2021 American Academy of Neurology
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Call for Society Prize | CNSF Congress

Call for Society Prize | CNSF Congress | AntiNMDA | Scoop.it
Call for Society Prizes | 2021 Congress SOCIETY PRIZES CNSF members have the opportunity to win valuable society prizes by submitting 'Abstracts' to the Congress as well as an 'Expanded Abstracts' to the society prize competitions. There are multiple first place prizes available to Junior Members or an Active Member within two years of receiving their certificate. Each valued at approximately $2500. Winners have the privilege of presenting their work at the Grand Plenary, alongside our Distinguished Guest Lecturers, during the Congress. Prize winners’ will be announced in the Neuro|News newsletter, in the Canadian Journal of Neurological Sciences and on the CNSF website. $500 second place prizes and additional subsidiary prizes may be awarded. Rules governing all Society Prize Submissions Authors are invited to submit an expanded abstract, on or before April 30, 2021 Contestants must be a member in good standing with any one of the five Societies of the CNSF; a Junior member or an Active member within two years of receiving their certificate. (Become a Member | CNSF) The same person may submit on different topics for the same prize The same expanded abstract may not be submitted to more than one Society Submissions must be provided in PDF format Those submitting an expanded abstract for a Society Prize must also submit their basic abstract to the CNSF Congress, on the official online abstract submission site. The Congress Abstract submission process is independent from the Society Prize submission process. Submission Details Prize submissions accepted by email ONLY Clearly indicate name of Society Prize you are submitting for Submissions must be submitted as three separate PDF files label: last name_ 'Letter' from their program chair 'Biography' 'Expanded Abstract' (include abstract number) CNSF will send a receipt of confirmation within 5 business days. If you do not receive a receipt of confirmation after 6 business days please contact the CNSF at nicole-rozak@cnsf.org The Canadian Neurological Society (CNS) Francis McNaughton Memorial Prize André Barbeau Memorial Prize Anti-NMDA Receptor Encephalitis Foundation Prize The Canadian Neurosurgical Society (CNSS) K.G. McKenzie Memorial Prize for Basic Neuroscience Research Prize K.G. McKenzie Memorial Prize for Clinical Neuroscience Research Prize The Canadian Society of Clinical Neurophysiologists (CSCN) Herbert Jasper Prize The Canadian Association of Child Neurology (CACN) President’s Prize The Canadian Society of Neuroradiology (CSNR) CSNR Society Prize
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Recurrent anti-NMDAR encephalitis during pregnancy combined with two antibodies positive

Recurrent anti-NMDAR encephalitis during pregnancy combined with two antibodies positive | AntiNMDA | Scoop.it
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune synaptic encephalitis likely mediated by neuronal surface antibody. Clinically, it is characterized by a variety of neurological and psychiatric symptoms, predominantly affecting young women.
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Cells | Free Full-Text | Neurodegeneration Induced by Anti-IgLON5 Antibodies Studied in Induced Pluripotent Stem Cell-Derived Human Neurons

Cells | Free Full-Text | Neurodegeneration Induced by Anti-IgLON5 Antibodies Studied in Induced Pluripotent Stem Cell-Derived Human Neurons | AntiNMDA | Scoop.it
Anti-IgLON5 disease is a progressive neurological disorder associated with autoantibodies against a neuronal cell adhesion molecule, IgLON5. In human postmortem brain tissue, the neurodegeneration and accumulation of hyperphosphorylated tau (p-tau) are found.
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Residual Fatigue and Cognitive Deficits in Patients After Leucine-Rich Glioma-Inactivated 1 Antibody Encephalitis | Allergy and Clinical Immunology | JAMA Neurology | JAMA Network

Residual Fatigue and Cognitive Deficits in Patients After Leucine-Rich Glioma-Inactivated 1 Antibody Encephalitis | Allergy and Clinical Immunology | JAMA Neurology | JAMA Network | AntiNMDA | Scoop.it
This study evaluates residual fatigue and cognitive defects in patients after leucine-rich glioma-inactivated 1 antibody encephalitis.
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Autoimmune Encephalitis Presenting with Malignant Catatonia in a 40-Year-Old Male Patient with Covid-19 - Abstract - Europe PMC

Autoimmune Encephalitis Presenting with Malignant Catatonia in a 40-Year-Old Male Patient with Covid-19 - Abstract - Europe PMC | AntiNMDA | Scoop.it
Europe PMC is an archive of life sciences journal literature.
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Encephalitis with autoantibodies against the glutamate kainate receptors GluK2 - Landa - - Annals of Neurology

Encephalitis with autoantibodies against the glutamate kainate receptors GluK2 - Landa - - Annals of Neurology | AntiNMDA | Scoop.it
Objective To report the identification of antibodies against the glutamate kainate receptor subunit 2 (GluK2‐abs) in patients with autoimmune encephalitis, and describe the clinical‐immunological f...
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Paroxysmal Sympathetic Hyperactivity in Severe Anti-N-Methyl-d-Aspartate Receptor Encephalitis: A Single Center Retrospective Observational Study

Paroxysmal Sympathetic Hyperactivity in Severe Anti-N-Methyl-d-Aspartate Receptor Encephalitis: A Single Center Retrospective Observational Study | AntiNMDA | Scoop.it
The incidence of PSH in severe anti-NMDARE patients was as high as 50%. Patients with PSH demonstrated prolonged NICU stay, hospital stay and increased duration of mechanical ventilation, while no effect on hospital mortality and functional outcome.
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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 ...
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Shared Decision‐making in Autoimmune Neurology | Neurology Clinical Practice

Shared Decision‐making in Autoimmune Neurology | Neurology Clinical Practice | AntiNMDA | Scoop.it
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Therapeutic plasma exchange in paediatric neurology: a critical review and proposed treatment algorithm - Eyre - 2018 - Developmental Medicine & Child Neurology

Therapeutic plasma exchange in paediatric neurology: a critical review and proposed treatment algorithm - Eyre - 2018 - Developmental Medicine & Child Neurology | AntiNMDA | Scoop.it
Therapeutic plasma exchange (TPE) has been a key immunotherapeutic strategy in numerous neurological syndromes, predominantly during the acute phase of illness. This paper reviews the indications, ...
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Characteristics and Outcomes of Autonomic Dysfunction in Anti-NMDAR Encephalitis | Research Square

Characteristics and Outcomes of Autonomic Dysfunction in Anti-NMDAR Encephalitis | Research Square | AntiNMDA | Scoop.it
Background To explore the characteristics and prognosis of autonomic dysfunction and paroxysmal sympathetic hyperactivity (PSH), and evaluate the efficacy of drugs used to suppress PSH episode in anti-NMDAR encephalitis patients.MethodsPat...
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Clinical Features and Outcomes of Anti-N-Methyl-d-Aspartate Receptor Encephalitis in Infants and Toddlers

Clinical Features and Outcomes of Anti-N-Methyl-d-Aspartate Receptor Encephalitis in Infants and Toddlers | AntiNMDA | Scoop.it
Anti-NMDAR encephalitis in infants and toddlers clinically presents with movement disorders, developmental regression, and abnormal behaviors. Interestingly, this group had a higher proportion of patients after viral encephalitis, which is regarded as the only risk factor for poor outcomes.
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Pediatric autoimmune encephalitis and its relationship with infection - ScienceDirect

Pediatric autoimmune encephalitis and its relationship with infection - ScienceDirect | AntiNMDA | Scoop.it
Autoimmune encephalitis (AE) is an increasingly recognized inflammatory disorder of the central nervous system and is most often characterized by anti…...
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Co-occurrence of Anti-N-Methyl-D-Aspartate Receptor Encephalitis and Anti-myelin Oligodendrocyte Glycoprotein Inflammatory Demyelinating Diseases: A Clinical Phenomenon to Be Taken Seriously

Co-occurrence of Anti-N-Methyl-D-Aspartate Receptor Encephalitis and Anti-myelin Oligodendrocyte Glycoprotein Inflammatory Demyelinating Diseases: A Clinical Phenomenon to Be Taken Seriously | AntiNMDA | Scoop.it
Background: Anti-N-methyl-D-aspartate receptor (NMDAR) immunoglobulin G antibodies which exist on myelin sheaths, composed of oligodendrocytes, especially target GluN1 subunits and are highly characteristic of anti-NMDAR encephalitis which is a newly recognized autoimmune encephalitis (AE) ch
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Pediatric anti-NMDA receptor encephalitis associated with COVID-19

Pediatric anti-NMDA receptor encephalitis associated with COVID-19 | AntiNMDA | Scoop.it
Anti-N-methyl-D-aspartate receptor encephalitis is a clinical condition characterized by acute behavioral and mood changes, abnormal movements, autonomic instability, seizures, and encephalopathy.We describe a 7-year-old boy diagnosed with autoimmune encephalitis due to NMDAR antibody in associatio...
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