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Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases - FluTrackers News and Information

Anti-NMDA receptor encephalitis and other emerging autoimmune brain diseases - FluTrackers News and Information | AntiNMDA | Scoop.it
RT @FluTrackers: Not So Rare But Rarely Diagnosed: From Demonic Possession to Anti-NMDA Receptor #Encephalitis https://t.co/37VSUDPNES h/t…
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Extreme delta brush: a unique EEG pattern in adults with anti-NMDA receptor encephalitis

Extreme delta brush: a unique EEG pattern in adults with anti-NMDA receptor encephalitis | AntiNMDA | Scoop.it
Extreme delta brush is a novel EEG finding seen in many patients with anti-NMDAR encephalitis. The presence of this pattern is associated with a more prolonged illness. Although the specificity of this pattern is unclear, its presence should raise consideration of this syndrome.
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Single-cell transcriptomics and surface epitope detection in human brain epileptic lesions identifies pro-inflammatory signaling | Nature Neuroscience

Single-cell transcriptomics and surface epitope detection in human brain epileptic lesions identifies pro-inflammatory signaling | Nature Neuroscience | AntiNMDA | Scoop.it
Single-cell analysis of immune cells from surgically resected human epileptic brain tissues showed heterogeneity and pro-inflammatory signaling in microglia and evidence for direct interaction of microglia with T cells.
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Clinical Variables, Deep Learning and Radiomics Features Help Predict the Prognosis of Adult Anti-N-methyl-D-aspartate Receptor Encephalitis Early: A Two-Center Study in Southwest China

Clinical Variables, Deep Learning and Radiomics Features Help Predict the Prognosis of Adult Anti-N-methyl-D-aspartate Receptor Encephalitis Early: A Two-Center Study in Southwest China | AntiNMDA | Scoop.it
The fusion model combining clinical variables and machine learning-based models may have early predictive value for poor outcomes associated with anti-NMDAR encephalitis.
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Little Otter Health Widening Access To Mental Health Care One Family At A Time –

Little Otter Health Widening Access To Mental Health Care One Family At A Time – | AntiNMDA | Scoop.it
Without access to mental health care, the Egger family might look very different today. As a child, Rebecca Egger routinely struggled with anxiety.This manifested as ADHD, a disorder that is sever…...
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Acute Sympotomatic Seizure Secondary to Autoimmune Encephalitis and Autoimmune-associated Epilepsy - Full Text View - ClinicalTrials.gov

Acute Sympotomatic Seizure Secondary to Autoimmune Encephalitis and Autoimmune-associated Epilepsy - Full Text View - ClinicalTrials.gov | AntiNMDA | Scoop.it
Acute Sympotomatic Seizure Secondary to Autoimmune Encephalitis and Autoimmune-associated Epilepsy - Full Text View.
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Hippocampal Neuronal Cultures to Detect and Study New Pathogenic Antibodies Involved in Autoimmune Encephalitis | Protocol

Hippocampal Neuronal Cultures to Detect and Study New Pathogenic Antibodies Involved in Autoimmune Encephalitis | Protocol | AntiNMDA | Scoop.it
Scientific Article | Autoimmune encephalitis is a new category of antibody-mediated diseases of the central nervous system. Hippocampal neurons can be used...
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Twitter Art Exhibit: An Interview with Susannah Cahalan

Twitter Art Exhibit: An Interview with Susannah Cahalan | AntiNMDA | Scoop.it
Susannah Cahalan, author of Brain on Fire, chats to Mathew Bose about the Twitter Art Exhibit and how the drawing of a clock helped a doctor diagnose her with autoimmune encephalitis...
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Local selling cheesecake raffle tickets for grandson's medical travel expenses

Local selling cheesecake raffle tickets for grandson's medical travel expenses | AntiNMDA | Scoop.it
FORT ST. JOHN, B.C. – Kimberly Sorin has created a cheesecake raffle to fund her grandson Colban’s medical travel expenses, this time to Vancouver and back. When Colban was five years old, he was diagnosed with an autoimmune disease called Anti-NMDAR Encephalitis.
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Cervical lymph nodes and ovarian teratomas as germinal centres in NMDA receptor-antibody encephalitis

Cervical lymph nodes and ovarian teratomas as germinal centres in NMDA receptor-antibody encephalitis | AntiNMDA | Scoop.it
Autoantibodies against the extracellular domain of the N-methyl-d-aspartate receptor (NMDAR) NR1 subunit cause a severe and common form of encephalitis.To better understand their generation, we aimed to characterize and identify human germinal centres actively participating in NMDAR-specific autoim...
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Lecture Series: Clinical Neuropathology of Cognitive Decline & Movement Disorders | Dementia Research

Lecture Series: Clinical Neuropathology of Cognitive Decline & Movement Disorders | Dementia Research | AntiNMDA | Scoop.it
This lecture is hosted by Prof. Gabor G. Kovacs MD PhD FRCPC. The lecture topic is subacute/chronic infections and autoimmune encephalitis associated with cognitive decline and/or movement disorders.
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Is there a place for routine cancer testing in autoimmune encephalitis?

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A video-based discussion of movement disorders in paediatric anti NMDAR encephalitis: A case series from Eastern India

A video-based discussion of movement disorders in paediatric anti NMDAR encephalitis: A case series from Eastern India | AntiNMDA | Scoop.it
MDs are a core feature of anti NMDAR encephalitis, particularly in the paediatric age group, understanding and characterization of which, is the key to early diagnosis and effective therapy.
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The Neutroimmunology-Multiple Sclerosis Unit of the Clínic-IDIBAPS seeks to collaborate with other hospitals to carry out three clinical trials in autoimmune encephalitis.

The Neutroimmunology-Multiple Sclerosis Unit of the Clínic-IDIBAPS seeks to collaborate with other hospitals to carry out three clinical trials in autoimmune encephalitis. | AntiNMDA | Scoop.it
Next Wednesday, June 22, a meeting will be held to recount the diseases and present the clinical trials.
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Refractory Anti-NMDA Receptor Encephalitis in Early Pregnancy | Neurology Neuroimmunology & Neuroinflammation

Refractory Anti-NMDA Receptor Encephalitis in Early Pregnancy | Neurology Neuroimmunology & Neuroinflammation | AntiNMDA | Scoop.it
AbstractBackground and Objectives Anti-N-methyl d-aspartate receptor (NMDAR) encephalitis classically affects women of childbearing age, producing a disproportionate number of pregnant women with anti-NMDAR encephalitis. The typical presentation includes progressive neuropsychiatric symptoms, seizures, and alterations in consciousness, all of which present potential risks to the fetus. First-line and second-line treatments similarly pose teratogenic potential; therefore, randomized studies with supportive data on pregnancy and fetal outcomes are lacking.Methods We present a case of refractory anti-NMDAR encephalitis during the first and second trimesters of pregnancy with the successful use of rituximab and cyclophosphamide and resultant healthy pregnancy.Results The patient was treated with an escalating immunotherapy regimen from 11 to 15 weeks of gestation, including steroids, plasma exchange, IV immunoglobulins, and rituximab, with no clinical response. At 16 weeks of gestation, she received cyclophosphamide with clinical improvement after 4 weeks. She subsequently gave birth to a healthy, term baby boy, who continued to do well at the follow-up.Discussion This case illustrates the effective use of cyclophosphamide in the second trimester of pregnancy for anti-NMDAR encephalitis. The use of second-line therapies remains an individualized decision because the relative risk-to-benefit ratio in pregnant women is incompletely understood.GlossaryASM=antiseizure medication; NMDAR=N-methyl d-aspartate receptorCaseA 19-year-old woman presented with status epilepticus at 11 weeks of pregnancy. Her symptoms began 4 weeks earlier with increasing psychotic behaviors and seizures. Her antiseizure medication (ASM) regimen at presentation included lacosamide, lamotrigine, levetiracetam, and clonazepam. On admission, her examination was notable for global hyperreflexia and left Babinski and with propofol wean, continuous right upper extremity twitching, and oral automatisms, during which time EEG revealed multifocal epileptiform spikes. On admission, MRI of the brain was unremarkable. CSF studies showed a lymphocytic pleocytosis with normal glucose and protein. Owing to the persistence of focal motor status despite ASM escalation, suspicion for an immune-mediated process arose leading to empiric treatment with steroids (Figure). She was subsequently treated with plasma exchange, followed by rituximab with decreasing seizure frequency on EEG, although no clinical improvement.<img width="440" class="highwire-fragment fragment-image" alt="Figure" height="243" src="https://nn.neurology.org/content/nnn/9/5/e200007/F1.medium.gif">Download figure Open in new tab Download powerpoint Figure Timeline of Clinical Events and TreatmentTimeline showing major clinical events (yellow), seizure activity (red), immunotherapy (blue), and NMDA-r antibody status (green) of the patient from symptom onset to delivery of baby. Red bar = seizure activity; purple bar = gestational age; dashed line = ongoing immunotherapy; CSF = cerebrospinal fluid; IVIG = IV immunoglobulin; IVSM = intravenous solumedrol; NMDA-r = NMDA receptor; PLEX = plasma exchange.During this time, NMDAR antibodies were detected in the CSF. Serum NMDAR antibodies were negative. Screening for an ovarian teratoma was unrevealing. Clinically she remained unchanged and thus received IV immunoglobulin and a second steroid course. CSF NMDAR antibodies remained positive. With the consent of her surrogate decision maker, she received cyclophosphamide at an estimated 16 weeks of gestation, followed by a third course of steroids.Four weeks after cyclophosphamide administration, she began to show improvement in her mental status correlating with the absence of epileptogenic activity on EEG and undetectable CSF NMDAR antibodies. Maternofetal medicine confirmed normal progression of pregnancy at 23 weeks of gestation. She transitioned to a floor bed after 24 days in the intensive care unit; by the time of discharge to inpatient rehabilitation, the patient was alert, oriented, and conversant, requiring 2-person assistance for ambulation.While at inpatient rehabilitation, her examination improved to fully ambulatory with mild cognitive deficits. She had recurrent seizures in the setting of levetiracetam wean at 29 weeks of gestation. Serum and CSF NMDAR antibodies remained undetectable; B-cell counts were suppressed. She was redosed with rituximab at this time and has continued twice yearly infusions since with infrequent, self-limited seizures. She gave birth to a healthy, term baby boy at 38 weeks and 6 days with appearance, pulse, grimace, activity, and respiration (APGAR) scores of 9 at 1 and 5 minutes. He continues to do well at the 3-year follow-up.DiscussionFetal health was favorable throughout pregnancy despite the protracted illness course and use of highly teratogenic medications (Table 1). Although the acute phase of anti-NMDAR encephalitis averages 16 weeks, this is one of the longest illnesses reported in early pregnancy. The gestational age at which disease onset occurs is of particular significance because placental transfer of maternal antibodies begins at weeks 12–13 of pregnancy; it has been postulated that fetal exposure to NMDAR antibodies may cause harm; however, recent case series have found most infants exposed to NMDAR antibodies are healthy at birth with normal development.1 Despite the duration and severity of disease in this patient, there were no adverse obstetrical outcomes; up to one-third of reported cases displays pathologic pregnancies, with spontaneous abortion and preterm birth occurring most frequently.1View inline View popup Table 1 Teratogenic Potential of Medications Commonly Used to Treat Anti-NMDAR EncephalitisThis case additionally reinforces the superior sensitivity of CSF over serum anti-NMDAR antibody testing. In refractory cases, following the CSF antibody titer may help guide therapy, with CSF titer changes more accurately reflecting relapse potential than serum titers.2 In our case, the persistence of NMDAR CSF antibodies prompted additional therapy, despite prior aggressive immunosuppression.Most notably, the use of cyclophosphamide during the second trimester for the treatment of anti-NMDAR encephalitis has yet to be reported. To date, cyclophosphamide use in pregnancy has been reported scarcely, with greatest frequency in malignancies and rheumatologic conditions. The literature presents conflicting data; many case reports reflect adverse pregnancy outcomes including preterm birth and fetal loss, whereas others mirror our experience with normal fetal development and birth.3,4 This likely reflects a myriad of factors, including underlying disease process, gestational age at exposure, and dose of or prior exposure to cyclophosphamide. Rituximab exposure in pregnancy is more frequently reported, with no significant increase in obstetric complications or fetal malformations compared with normative data.1,5 Neonatal lymphopenia may occur at birth; however, cell counts recover within 6 months, and no infectious complications are cited.6 Ultimately, the use of second-line therapies during pregnancy for the treatment of anti-NMDAR encephalitis remains an individualized decision with significant potential risk. Although the generally accepted standard of care for second-line therapy in nonpregnant individuals includes rituximab and cyclophosphamide, the relative risk-to-benefit ratio in pregnant women is incompletely understood and requires further study.Study FundingThe authors report no targeted funding.DisclosureS. Fredrich's fellowship was funded by the National Multiple Sclerosis Society. She has served as a consultant to EMD Serono. C. Wang and R. Narayan report no disclosures. L. Tardo's fellowship was funded by the National MS Society. K. Blackburn's fellowship was funded by the Siegel Rare Neuroimmune Association. S. Vernino has served as a consultant to Alterity, Genentech, Catalyst and Sage Therapeutics. He has received research support from Dysautonomia International, BioHaven, Grifols, and Quest Diagnostics (through a licensing contract). Go to Neurology.org/NN for full disclosures.Appendix Authors<img width="597" height="914" alt="Table" class="highwire-fragment fragment-image" src="https://nn.neurology.org/content/nnn/9/5/e200007/T2.medium.gif">FootnotesGo to Neurology.org/NN for full disclosures. Funding information is provided at the end of the article.Submitted and externally peer reviewed. The handling editor was Josep O. Dalmau, MD, PhD, FAAN.Received February 9, 2022.Accepted in final form April 25, 2022.Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.References1.↵Joubert B, García-Serra A, Planagumà J, et al. Pregnancy outcomes in anti-NMDA receptor encephalitis: case series. Neurol Neuroimmunol Neuroinflamm. 2020;7(3):e668.OpenUrlAbstract/FREE Full Text2.↵Gresa-Arribas N, Titulaer MJ, Torrents A, et al. Antibody titres at diagnosis and during follow-up of anti-NMDA receptor encephalitis: a retrospective study. Lancet Neurol. 2014;13(2):167-177.OpenUrlCrossRefPubMed3.↵Lannes G, Elias FR, Cunha B, et al. Successful pregnancy after cyclophosphamide therapy for lupus nephritis. Arch Gynecol Obstet. 2011;283(1):61-65.OpenUrlCrossRefPubMed4.↵Clowse ME, Magder L, Petri M. Cyclophosphamide for lupus during pregnancy. Lupus. 2005;14(8):593-597.OpenUrlCrossRefPubMed5.↵Maddow-Zimet I, Kost K. Pregnancies, Births and Abortions in the United States, 1973-2017: National and State Trends by Age. Guttmacher Institute, 2021. doi: https://doi.org/10.1363/2020.31952.6.↵Klink DT, Van Elburg RM, Schreurs MWJ, van Well GTJ. Rituximab administration in third trimester of pregnancy suppresses neonatal B-cell development. Clin Dev Immunol. 2008;2008:271363.OpenUrlCrossRefPubMed
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Seronegative Autoimmune Encephalitis: A Challenge for the Neurologist - PMC

Seronegative Autoimmune Encephalitis: A Challenge for the Neurologist - PMC | AntiNMDA | Scoop.it
Ann Indian Acad Neurol. 2022 Mar-Apr; 25(2): 280–283. Published online 2021 Oct 11. doi: 10.4103/aian.aian_362_21PMCID: PMC9175409PMID: 35693642Seronegative Autoimmune Encephalitis: A Challenge for the NeurologistLaxmi Khanna, Chandrashekar Agrawal, Mandaville Gourie-Devi, and Ankkita Sharma BhandariAuthor information Article notes Copyright and License information Disclaimer[What is new] Patients with suspected autoimmune encephalitis and negative antibody assays are a common dilemma in Neurological practice. Antibody Prevalence in Epilepsy and Encephalopathy Score [APE2] and Response to Immunotherapy in Epilepsy and Encephalopathy Scores [RITE2] enhance the value of early case detection and treatment to prevent neurological sequel.[What is old] Immunological studies in autoimmune neurological diseases may be negative despite characteristic clinical findings thus delaying diagnosis and treatment.Seronegative autoimmune encephalitis is a term coined for patients who present with the triad of cognitive disturbances, seizures, and behavioral abnormalities but continue to evade antibody detection in serum and cerebrospinal fluid. The occurrence of seronegative autoimmune encephalitis is 48% despite the availability of the latest panel of antibody assays. The dilemma arises when patients, who present with the typical clinical and imaging findings of autoimmune encephalitis persist with negative antibody results. This case illustrates the importance of suspecting and treating seronegative IgLON5 disease with typical clinical features and life-threatening complications.A 75-year-old man presented with progressive impairment of memory, mood swings, irritability, and excessive daytime sleepiness of 6-week duration. As time progressed, he experienced unsteadiness of gait, tremors, swallowing difficulty, and noisy breathing. Sleep was interrupted by snoring and rhythmic tapping of feet. Clinical examination revealed an elderly gentleman who had a loud audible inspiratory stridor. There was a dysexecutive syndrome comprising difficulty in planning, decision-making, temporal sequencing, poor attention span, and impaired abstract thinking, Glasgow coma score (GCS) was E3M6V4. He had a mask-like expression, low-toned speech, bilateral upgaze palsy, palatal involvement, cogwheel rigidity, and postural instability. Routine blood and urine examination were normal. Serum ammonia, free triiodothyronine (FT3), free thyroxine (FT4), and thyroid-stimulating hormone levels were normal. CSF was clear, cells 20 [100% lymphocytes], protein 46.80 mg/dL [normal 20–40 mg/dL], sugar 58.00 mg/dL [blood sugar 110 mg/dL]. Serology for herpes virus, and autoimmune and paraneoplastic antibodies were negative. Anti-thyroperoxidase antibody was 4.94 μm/mL [<30.00 μm/mL], and anti-thyroglobulin was 5.88 μm/mL [<30.00 μm/mL].Paired serum and CSF by indirect immunofluorescence method did not show the presence of anti-IgLON5 antibodies. Electroencephalogram (EEG) revealed periodic short interval generalized discharges [Figure 1a]. Computed tomography (CT) of the brain revealed age-related cerebral atrophy. [Figure 1b]. Fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) of the brain revealed diffuse hypermetabolism in bilateral basal ganglia, midbrain, pons, vermis, and cerebellar hemispheres favoring autoimmune encephalitis [Figure 1c]. Sleep studies revealed a severe degree of obstructive apnea with apnea-hypopnea index (AHI) 63.6 and periodic limb movements in sleep [Figure 1d] for which CPAP titration studies were done. The patient had an Antibody Prevalence in Epilepsy and Encephalopathy [APE2] score of and Response to Immunotherapy in Treatment of Epilepsy and Encephalopathy [RITE2] score of 4 and 5 each [Tables ​[Tables1a1a and ​andb]b] before starting therapy. Based on the history, clinical presentation, EEG, imaging, polysomnography, negative serology, APE2, and RITE2 scores, a diagnosis of anti-IgLON5 disease was made, and the patient received plasma exchange followed by intravenous immunoglobulin and noninvasive ventilatory support. As he presented in an advanced stage of his illness, he succumbed and expired a fortnight later.Open in a separate windowFigure 1Table 1aClinical featuresScoreNew-onset, rapidly progressive mental status changes emotional lability+1Autonomic dysfunction [presenting as labile blood pressure, labile heart rate, persistent tachycardia, postural hypotension]+1Viral prodrome [runny nose, sore throat, low-grade fever] only to be scored in the absence of underlying malignancy0Facial dyskinesias or faciobrachial dystonic movements0Seizure refractory to at least 2 antiseizure medications0CSF findings consistent with inflammation [elevated+2CSF protein level >50 mg/dL and/or lymphocytic pleocytosis >5 cells/dL, if the total number of CSF RBCs is <1000 cells/dL].Brain MRI showing signal changes consistent with limbic encephalitis [medial temporal T2/FLAIR signal changes]0Presence of underlying malignancy [excluding cutaneous squamous cell or basal cell carcinomas]0Total4Open in a separate windowTable 1bClinical featuresScoreNew-onset, rapidly progressive mental status changes that developed over 1–6 weeks or new-onset seizure activity (within 1 year of evaluation).+1Neuropsychiatric changes; agitation, aggressiveness, emotional lability.+1Autonomic dysfunction [sustained atrial tachycardia or bradycardia, orthostatic hypotension [≥ 20 mm Hg fall in systolic pressure or ≥10 mm Hg fall in diastolic pressure within 3 min of quiet standing], hyperhidrosis, persistently labile blood pressure, ventricular tachycardia, cardiac asystole, or gastrointestinal dysmotility.+1Viral prodrome [rhinorrhea, sore throat, low-grade fever] to be scored in the absence of underlying systemic malignancy within 5 years of neurological symptom onset.0Faciobrachial dystonic seizures0Facial dyskinesias, to be scored in the absence of faciobrachial dystonic seizures.0Seizure refractory to at least two antiseizure medications.0CSF findings consistent with inflammation [elevated CSF protein >50 mg/dL and/or lymphocytic pleocytosis >5 cells/mcL, if the total number of CSF RBC is <1000 cells/mcL].+2Brain MRI suggesting encephalitis [T2/FLAIR hyperintensity restricted to one or both medial temporal lobes, or multifocal in grey matter, white matter, or both compatible with demyelination or inflammation].0Systemic cancer diagnosed within 5 years of neurological symptom onset. [excluding cutaneous squamous cell carcinoma, basal cell carcinoma, brain tumor, cancer with brain metastasis].0Immunotherapy initiated within 6 months of symptom onset.0Neural plasma membrane autoantibody detected [NMDAR, GABAAR, GABABR, AMPAR, DPPX, mGluR1, mGluR2, mGluR5, LGI1, IgLON5, CASPR2 or MOG].0Total5Open in a separate windowAnti-IgLON5 disease, a new entity described in 2014, comprises a constellation of symptoms in four neurological domains which include sleep disorders, bulbar dysfunction, supranuclear gaze palsies, and cognitive decline.[1,2] Antibodies against neuronal cell adhesion proteins cause an irreversible internalization of IgLON-5 surface antigens with deposition of hyperphosphorylated tau in the hippocampus and brainstem.[1,2] Often the classical clinical picture, cerebrospinal fluid pleocytosis, and MRI findings of bilateral medial temporal hyperintensities occur in the absence of IgLON5 antibodies.[3] Seronegative autoimmune encephalitis represents patients with a well-defined neurological syndrome, cerebrospinal fluid pleocytosis, typical imaging findings, and a negative serology after excluding alternative causes.[3,4,5]Antibody negative immune-mediated encephalitis has triggered the attention of many authors, as they have been several reports of patients with chronic refractory epilepsy, encephalopathy, and cognitive dysfunction requiring immunotherapy despite antiepileptic medication if the disease progresses further.[6,7] Initiation of treatment in suspected auto-antibody negative encephalitis prevents neuronal damage and memory impairment.[6,7] Early therapy averts sequel like hippocampal sclerosis, verbal and visual memory decline, and brainstem dysfunction in clinically suspected patients.[6,7]At present, twenty serological tests are used for the detection of neural-specific autoantibodies in patients with autoimmune encephalitis [Table 1c].[7] However, despite the availability of a battery of tests, 48% of cases remain seronegative, and a consensus is achieved by including clinical and imaging findings.[6,7] The Antibody Prevalence in Epilepsy and Encephalopathy score [APE2] is a clinical scoring system used in the Mayo Clinic to estimate the probability of an autoimmune etiology in antibody-negative patients with an accurate prediction of the outcome.[6,7] [Table 1a] An APE score of 4 or more indicates the necessity for antibody testing.[6] The Response to Immunotherapy in Epilepsy and Encephalopathy score [RITE2] was derived at the Mayo Clinic from the analysis of retrospective studies to identify patients of autoimmune etiology who respond to immunotherapy.[7] A score of less than 7 is associated with refractoriness to immunotherapy [Table 1b]. The combined use of both these scales enables clinicians to apply an efficient evidence-based approach for the diagnosis of autoimmune neurological diseases in neurology clinics and busy hospitals where rapid screening is necessary.[6,7] Cognitive improvement following early immunotherapy cannot be overemphasized thus enhancing the value of these parameters in patients with negative antibody assays.[6,7]Table 1cSurface antigen associatedIntracellular antigen associatedNeural autoantibodyNeural autoantibody1. AMPAR11. AK52. LG112. Amphiphysin3. CASPAR213. GAD-654. VGKCC [P/Q or Ntype]14. ITPR15. DPPX15. ZIC46. GABAAR16. ANNA-1 [Hu]7. GABABR17. ANNA-2 [Ri]8. IgLON518. CRMP59. MGluR1 – mGluR519. PCA-1[Yo]10. NMDAR20. AmphiphysinOpen in a separate windowElderly people have a challenging time with autoimmune encephalitis due to the occurrence of age-related comorbidities that affect memory and cognition.[8] Older people have a higher chance of being antibody negative on routine testing in clinically suspected cases and 58.3% of seronegative limbic encephalitis can develop malignancy on subsequent follow-up visits.[8] In children, rapid cognitive decline, impairment of memory, refractory seizures, and dyskinesias are suggestive of an autoimmune etiology despite negative antibody results.[8,9] These scales are invaluable to enhance the yield of diagnosis in patients suspected of autoimmune encephalopathy or epilepsy irrespective of their age.[6,7]Our patient had classical clinical and PET-CT findings of anti-IgLON5–mediated encephalitis with a negative antibody report, an APE2 score, and RITE2 score of 4 and 5 each [Tables ​[Tables1a1a and ​andb].b]. Young children and the elderly are vulnerable members of our society whose susceptibility to antibody-mediated illnesses and neurocognitive outcomes depend on the speed of instituting immunotherapy and disease-modifying agents.[8,9] Use of such scores should be strongly recommended when the tempo of illness is subacute, CSF is cellular, and PET scan is suggestive.[7,10] However, a larger study in such patients [clinically suggestive of IgLON5 encephalitis but antibody negative] needs to be done to validate these scores and bring them into the standard recommendation.Declaration of patient consentThe authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.Financial support and sponsorshipNil.Conflicts of interestThere are no conflicts of interest.AcknowledgementsThe staff of the Department of Neurophysiology at Sir Ganga Ram Hospital without whose support these Case Reports could not be complied.REFERENCES1. Gaig C, Graus F, Compta Y, Högl B, Bataller L, Brüggemann N, et al. Clinical manifestations of the anti-IgLON5 disease. Neurology. 2017;88:1736–43. [PMC free article] [PubMed] [Google Scholar]2. Bhatia M, Singh Y. Anti IgLON5 disease-First case report from India. Sleep Med. 2020;67:215–6. [PubMed] [Google Scholar]3. Graus F, Escudero D, Oleaga L, Bruna J, Villarejo-Galende A, Ballabriga J, et al. Syndrome and outcome of antibody-negative limbic encephalitis. Eur J Neurol. 2018;25:1011–6. [PMC free article] [PubMed] [Google Scholar]4. Pradhan S, Das A, Das A, Mulmuley M. Antibody negative autoimmune encephalitis – Does it differ from definite one? Ann Indian Acad Neurol. 2019;22:401–8. [PMC free article] [PubMed] [Google Scholar]5. Von Rhein B, Wagner J, Widman G, Malter MP, Elger CE, Helmstaedter C. Suspected antibody negative autoimmune limbic encephalitis: Outcome of immunotherapy. Acta Neurol Scand. 2017;135:134–41. [PubMed] [Google Scholar]6. Dubey D, Alqallaf A, Hays R, Freeman M, Chen K, Ding K, et al. Neurological autoantibody prevalence in epilepsy of unknown etiology. JAMA Neurol. 2017;74:397–402. [PubMed] [Google Scholar]7. Dubey D, Kothapalli N, McKeon A, Flanagan EP, Lennon VA, Klein CJ, et al. Predictors of neural specific autoantibodies and immunotherapy response in patients with cognitive dysfunction. J Neuroimmunol. 2018;323:62–72. [PubMed] [Google Scholar]8. Chandra SR, Ray S, Isaac T, Pai AR, Krishnareddy H, Dhar D, et al. A clinical TRIAD for early suspicion of autoimmune encephalitis as a possibility in patients presenting with progressive cognitive decline. Asian J Psychiatr. 2019;41:5–12. [PubMed] [Google Scholar]9. Sahoo B, Jain MK, Mishra R, Patnaik S. Dilemmas and challenges in treating seronegative autoimmune encephalitis in Indian children. Indian J Crit Care Med. 2018;22:875–8. [PMC free article] [PubMed] [Google Scholar]10. Netravathi M. Seronegative autoimmune encephalitis-A diagnostic and therapeutic dilemma. Ann Indian Acad Neurol. 2019;22:369–70. [PMC free article] [PubMed] [Google Scholar]Articles from Annals of Indian Academy of Neurology are provided here courtesy of Wolters Kluwer -- Medknow Publications
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Anti-N-methyl-D-aspartate receptor encephalitis: characteristics and rapid diagnostic approach in the emergency department | BMC Neurology | Full Text

Anti-N-methyl-D-aspartate receptor encephalitis: characteristics and rapid diagnostic approach in the emergency department | BMC Neurology | Full Text | AntiNMDA | Scoop.it
Background Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a common type of autoimmune encephalitis. Patients with this condition are frequently very ill but are often misdiagnosed in the Emergency Department (ED). The objective of this study was to analyze the clinical characteristics of anti-NMDAR patients in the ED and to identify any associations with a diagnosis of anti-NMDAR encephalitis. Methods We performed a retrospective analysis of a prospectively obtained cohort of ED patients from May 2011 to December 2017. We identified patients diagnosed with anti-NMDAR encephalitis in this cohort and extracted key patient characteristics and clinical data, including patient gender, age, presentation, modified Rank Score (m-RS), laboratory test results, significant treatments, and mortality. Results Eighty-seven patients with anti-NMDAR encephalitis were identified. 54 (62.1%) were female, 23 (26.4%) were < 18 years old, 14 (16.1%) had teratoma, and 45 (51.7%) had an m-RS ≥ 4. Fever, altered mental status, and seizures were the most common symptoms, with a > 50% incidence of each symptom in the cohort. The sensitivity of CSF oligoclonal band (OB) testing was 78.9%. 22 (25.3%) were admitted to the ICU, 20 (23.0%) patients were intubated, but only one patient died (1.1%). 47 (54.0%) were misdiagnosed prior to ED arrival. All patients underwent immunotherapy as first-line treatment for anti-NMDAR encephalitis. Conclusions A majority of anti-NMDAR encephalitis patients presenting to the ED were female and were likely to be misdiagnosed prior to arrival. Patients with symptoms of fever, altered mental status, and seizures need a lumbar puncture, including CSF OB testing, for definitive diagnosis.
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Faciobrachial Dystonic Seizures as a Sign of Relapse in a Child with LGI-1 Encephalitis - Wajd Alotaibi, Shahid Bashir, Ali Mir, 2022

We report an interesting case of a young girl with LGI1-antibody encephalitis who presented at 7 years old with very frequent seizures and severe neurocognitive decline. She responded very well to ...
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Immune characteristics of children with autoimmune encephalitis and the correlation with a short-term prognosis | Italian Journal of Pediatrics | Full Text

Immune characteristics of children with autoimmune encephalitis and the correlation with a short-term prognosis | Italian Journal of Pediatrics | Full Text | AntiNMDA | Scoop.it
Background Autoimmune encephalitis (AE) is a type of encephalopathy mediated by an antigenic immune response in the central nervous system. Most research related to autoimmune encephalitis (AE) is focused on early diagnosis, treatment and prognosis analysis; there has been little research...
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EEG interpretation and ictal-interictal continuum

EEG interpretation and ictal-interictal continuum | AntiNMDA | Scoop.it
CONTENTS When & how long to monitor EEG: Indications for EEG monitoring in the ICU Duration of EEG monitoring Basics EEG leads  Background pattern Reactivity Paralysis to eliminate artefacts EEG in encephalopathy/coma Beta coma Alpha coma Theta coma Delta coma Spindle coma Seizures Epileptiform discharges...
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Faciobrachial Dystonic Seizures as a Sign of Relapse in a Child with LGI-1 Encephalitis - PMC

Faciobrachial Dystonic Seizures as a Sign of Relapse in a Child with LGI-1 Encephalitis - PMC | AntiNMDA | Scoop.it
We report an interesting case of a young girl with LGI1-antibody encephalitis who
presented at 7 years old with very frequent seizures and severe neurocognitive decline.
She responded very well to high dose corticosteroids and intravenous immunoglobulin ...
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Adenylate kinase 5 (AK5) autoimmune encephalitis: Clinical presentations and outcomes in three new patients

Adenylate kinase 5 (AK5) autoimmune encephalitis: Clinical presentations and outcomes in three new patients | AntiNMDA | Scoop.it
Adenylate kinase 5 (AK5) antibodies are biomarkers of a poorly responsive to immunotherapy, non-paraneoplastic, autoimmune limbic encephalitis. We detected 6 patients (all female, median age: 72 years [49-80]) with identical CSF antibody staining by indirect immunofluorescence on mouse tissues.
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Immunotherapy in autoimmune encephalitis

Immunotherapy in autoimmune encephalitis | AntiNMDA | Scoop.it
Early first-line immunotherapies, including corticosteroids and plasma exchange, improve outcomes, with emerging evidence showing second-line immunotherapies (especially rituximab) reduce relapse rates.
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McFeely Mess podcast: Bismarck lawyer, struck by rare disease, tells her tale of terror and recovery - InForum | Fargo, Moorhead and West Fargo news, weather and sports

McFeely Mess podcast: Bismarck lawyer, struck by rare disease, tells her tale of terror and recovery - InForum | Fargo, Moorhead and West Fargo news, weather and sports | AntiNMDA | Scoop.it
Jackie Stebbins was a successful young attorney with a family, driven to succeed in North Dakota's capital city. It all changed when she was struck by a rare disease, autoimmune encephalitis. She's written a book to tell her story.
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Patients with new onset psychosis should receive corticosteroids to treat autoimmune encephalitis

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Immunotherapy in autoimmune encephalitis : Current Opinion in Neurology

Immunotherapy in autoimmune encephalitis : Current Opinion in Neurology | AntiNMDA | Scoop.it
Purpose of review Autoimmune encephalitis (AE) refers to immune-mediated neurological syndromes o...
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