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Anti-Hu limbic encephalitis preceding the appearance of mediastinal germinoma by 9 years | Neurology Neuroimmunology & Neuroinflammation

Anti-Hu limbic encephalitis preceding the appearance of mediastinal germinoma by 9 years | Neurology Neuroimmunology & Neuroinflammation | AntiNMDA | Scoop.it
Autoimmune limbic encephalitis is characterized by the subacute onset of seizures, short-term memory loss, and confusion and can have a paraneoplastic or nonparaneoplastic etiology.1 Anti-Hu limbic encephalitis is typically paraneoplastic, most frequently associated with small-cell lung cancer.2 We report the case of a 28-year-old man diagnosed with a mediastinal germ cell tumor 9 years after presenting with anti-Hu-positive limbic encephalitis. Case report A 19-year-old man presented after a generalized tonic-clonic seizure after several days of headaches and confusion. He had no medical history and did not take regular medications. There was no history of drug use, and he was a nonsmoker. There was no focal neurologic deficit on examination. Electroencephalography demonstrated right temporal spike and slow wave activity. Brain MRI showed hyperintensity in the right medial temporal lobe on T2 FLAIR sequence, without gadolinium enhancement (figure, A). CSF showed 3 monocytes, and the protein level was slightly elevated (0.48 g/L, NR 0.15–0.45 g/L). He was diagnosed with viral encephalitis. Figure Imaging investigations (A) Axial T2 fluid attenuated inversion recovery (FLAIR) MRI of the brain showing hyperintensity in the right medial temporal lobe at the age of 22 years. (B) Large anterior mediastinal mass diagnosed at the age of 28 years as demonstrated on CT chest. (C) Large anterior mediastinal mass (age 28) demonstrating high glucose avidity on PET-CT. (D) Left: Markedly increased metabolism in the left anteromesial temporal lobe and hippocampus. Right: Resolution of those changes 11 months after resection of the mediastinal tumor. At the age of 21 years, he re-presented with seizures, confusion, emotional lability, night sweats, and short-term memory loss. MRI demonstrated ongoing T2 FLAIR hyperintensity in the right medial temporal lobe. Further workup revealed serum anti-Hu antibodies by immunofluorescence (Inova Diagnostics, CA), confirmed by line immunoblot (Euroimmun Lubeck, Germany). Other antibodies, including amphiphysin, CRMP5, Ma2, Yo, Ri, titin, recoverin, SOX-1, and VGKC, were negative. He was diagnosed with paraneoplastic limbic encephalitis as per the guidelines by Graus.1 The CT scan of chest, abdomen, and pelvis was negative for malignancy, and whole-body PET was normal. Ultrasound revealed microcalcifications in the right testis. Alfa-fetoprotein and β-human chorionic gonadotropin (β-hCG) were negative. He was managed with IV cyclophosphamide 0.75 g/m2 monthly for 6 months as per the National Institute of Health (NIH) protocol, IV methylprednisolone 1000 mg daily for 5 days, and IV immunoglobulin (IVIG) 0.4 g/kg/d for 5 days. This was followed by oral prednisone 50 mg daily, weaned to 10 mg daily after several weeks, azathioprine 50 mg twice daily (1.33 mg/kg/d), IVIG 0.4 g/kg 1 day per month, and antiepileptic therapy. His condition followed a fluctuating course, and repeated malignancy screening remained negative. Right orchiectomy was performed at the age of 24 years; however, findings were nonspecific and his condition remained unchanged. At the age of 26 years, a single course of rituximab 1000 mg was given in response to radiologic relapse, after which neuropsychometric parameters and MRI mildly improved. Azathioprine and IVIG continued throughout; prednisone was ceased after a slow wean over 3 years. At the age of 28 years, he re-presented with pleuritic chest pain. CT scan demonstrated a large anterior mediastinal soft-tissue lesion measuring 107 mm, which was highly glucose avid on PET (figure, B and C). Biopsy revealed a nonseminomatous germ cell tumor. Serum alfa-fetoprotein was 954 kIU/L (NR 0–6), and serum β-hCG 19 IU/L (NR 0–5). Serum anti-Hu remained positive, and all other antibodies remained negative. Immunotherapy was ceased, and he received bleomycin, etoposide, and cisplatin (BEP) 2 months before surgical resection. Pathologic assessment showed a mixed germ cell tumor with low grade sarcomatous components. Immunohistochemistry was performed on permeabilized formalin-fixed paraffin-embedded tissue with HuD antibody at a dilution of 1:100 on an automated Leica Bond III. Tumor tissue was compared with normal brain as a positive control; however, it did not stain positive. He has been neurologically stable for 3 years postoperatively and has now been able to undertake a vocational training course. He is no longer receiving immunotherapy. Lung recurrences 6 months and 2 years postoperatively required repeat resection and chemoradiotherapy. Neurologic PET, 11 months postoperatively, demonstrated resolution of previously persistent temporal lobe changes (figure, D). Discussion Our patient presented with paraneoplastic limbic encephalitis, presumably since the age of 19 years. However, it was not until 9 years later that malignancy was detected. Paraneoplastic limbic encephalitis frequently precedes the diagnosis of an underlying malignancy;2 however, the current criteria for diagnosis include demonstration of malignancy within 42 or 51 years. Anti-Hu antibodies are associated with malignancy in more than 95% of cases, so the eventual detection of malignancy as in our case should not be surprising. It is more typical that Hu antibodies are associated with small-cell lung cancer,3 although a mediastinal germinoma has been reported.4 It is possible that testicular microcalcifications were evidence of earlier immunologic activity, especially given the development of a germ cell tumor. This very long delay between Hu antibody positivity and the diagnosis of malignancy is atypical. Similar cases include detection of small-cell lung cancer 5.5 years after anti-Hu-associated subacute sensory neuronopathy,5 lung carcinoid detected 8 years after initial diagnosis of anti-Ri antibodies,6 and 2 patients in whom malignancy developed 4.5 and 10 years, respectively, after detection of anti-Yo antibody-associated cerebellar degeneration.7 Although our patient's tumor did not stain positive for anti-Hu, it was likely responsible for his syndrome, as evidenced by his improvement after resection. Negative staining might be explained by preanalytical fixation of the tissue influencing the binding capacity of the antibody or a lack of specificity to particular epitopes on the tumor itself. The long delay between the detection of anti-Hu antibodies and the diagnosis of malignancy in this case highlights the importance of ongoing surveillance for malignancy in the presence of onconeuronal antibodies. Study Funding No targeted funding reported. Disclosure The authors report no disclosures. Go to Neurology.org/NN for full disclosures. Appendix Authors Footnotes Go to Neurology.org/NN for full disclosures. Funding information is provided at the end of the article. The Article Processing Charge was funded by Concord Repatriation General Hospital. Received November 5, 2019. Accepted in final form January 23, 2020. Copyright © 2020 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. References 1.↵Graus F, Delattre JY, Antoine JC, et al. Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatr 2004;75:1135–1140. 2.↵Gultekin SH, Rosenfeld MR, Voltz R, Eichen J, Posner JB, Dalmau J. Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Brain 2000;123:1481–1494.OpenUrlCrossRefPubMed 3.↵Graus F, Titulaer MJ, Balu R, et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol 2016;15:391–404.OpenUrlCrossRefPubMed 4.↵Nanavati A, Zeck J, Philips G, Bade NA. A unique case of pronounced neurologic deficit from paraneoplastic syndrome that precedes appearance of mediastinal germinoma by two years. Case Rep Clin Pathol 2016;3:6–9.OpenUrl 5.↵Gaillard N, Charif M, Carlander B, Pujol JL, Touchon J. Chemotherapy treatment for anti-Hu paraneoplastic syndrome without active malignancy [in French]. Rev Neurol (Paris) 2006;162:862–865.OpenUrlPubMed 6.↵Harloff A, Hummel S, Kleinschmidt M, Rauer S. Anti-Ri antibodies and limbic encephalitis in a patient with carcinoid tumour of the lung. J Neurol 2005;252:1404–1405.OpenUrlCrossRefPubMed 7.↵Rojas-Marcos I, Rousseau A, Keime-Guibert F, et al. Spectrum of paraneoplastic neurologic disorders in women with breast and gynecologic cancer. Medicine (Baltimore) 2003;82:216–223.OpenUrlCrossRefPubMed
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Neural Antibody Testing for Autoimmune Encephalitis: A Canadian Single-Centre Experience | Canadian Journal of Neurological Sciences

Neural Antibody Testing for Autoimmune Encephalitis: A Canadian Single-Centre Experience - Volume 48 Issue 6
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Dysfunction of the Autonomic Nervous System and its Role in the Pathogenesis of Septic Critical Illness (Review)

Dysfunction of the Autonomic Nervous System and its Role in the Pathogenesis of Septic Critical Illness (Review) | AntiNMDA | Scoop.it
Dysfunction of the autonomic nervous system (ANS) of the brain in sepsis can cause severe systemic inflammation and even death. Numerous data confirmed the role of ANS dysfunction in the occurrence, course, and outcome of systemic sepsis.
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Frontiers | Risk Factors and Brain Metabolic Mechanism of Sleep Disorders in Autoimmune Encephalitis | Immunology

Frontiers | Risk Factors and Brain Metabolic Mechanism of Sleep Disorders in Autoimmune Encephalitis | Immunology | AntiNMDA | Scoop.it
BackgroundSleep disorders (SDs) in autoimmune encephalitis (AE) have received little attention and are poorly understood. We investigated the clinical characteristics, risk factors, and cerebral metabolic mechanism of SD in AE.MethodsClinical, laboratory, and imaging data were retrospectively...
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A peculiar case of psychosis: anti-NMDAr encephalitis | International Journal of Emergency Medicine | Full Text

A peculiar case of psychosis: anti-NMDAr encephalitis | International Journal of Emergency Medicine | Full Text | AntiNMDA | Scoop.it
Background Psychosis in pregnancy is rare and could be life-threatening. It requires prompt evaluation and proper management accordingly. Anti-N-methyl-d-aspartate receptor (anti-NMDAr) encephalitis following herpes simplex virus (HSV) infection is a rare cause of psychosis during pregnancy.
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Acute Psychosis Due to Anti-N-Methyl D-Aspartate Receptor Encephalitis Following COVID-19 Vaccination: A Case Report - PMC

Acute Psychosis Due to Anti-N-Methyl D-Aspartate Receptor Encephalitis Following COVID-19 Vaccination: A Case Report - PMC | AntiNMDA | Scoop.it
Anti-N-methyl D-aspartate (NMDA) receptor (anti-NMDAR) encephalitis has been reported after SARS-CoV-2 infection, but not after SARS-CoV-2 vaccination. We report the first known case of anti-NMDAR encephalitis after SARS-CoV-2 immunization in a young ...
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Anti-NMDAR Autoantibodies Disrupt Ionotropic Receptor Signaling –

Anti-NMDAR Autoantibodies Disrupt Ionotropic Receptor Signaling – | AntiNMDA | Scoop.it
Vignesh Subramanian '24 Figure 1: The N-methyl-D-aspartate receptor (NMDAR) functions as an ion channel. N-methyl-D-aspartate receptors (NMDARs) are ligand-gated ion channels whose signaling enables higher-order functions, such as learning and memory, throughout the brain.
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A Brain on Fire: Laura's Battle with Autoimmune Encephalitis

A Brain on Fire: Laura's Battle with Autoimmune Encephalitis | AntiNMDA | Scoop.it
Laura Martin, a strong student and standout goalie at Transylvania University, hit a sudden wall as things turned worse. Diagnosis: Autoimmune Encephalitis.
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Frontiers | Case Report: Prominent Brainstem Involvement in Two Patients With Anti-CASPR2 Antibody-Associated Autoimmune Encephalitis | Immunology

Frontiers | Case Report: Prominent Brainstem Involvement in Two Patients With Anti-CASPR2 Antibody-Associated Autoimmune Encephalitis | Immunology | AntiNMDA | Scoop.it
Anti-contactin-associated protein-like 2 (CASPR2) antibody-associated autoimmune encephalitis is commonly characterized by limbic encephalitis with clinical symptoms of mental and behavior disorders, cognitive impairment, deterioration of memory, and epilepsy.
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Frontiers | Acute Psychosis Due to Anti-N-Methyl D-Aspartate Receptor Encephalitis Following COVID-19 Vaccination: A Case Report | Neurology

Frontiers | Acute Psychosis Due to Anti-N-Methyl D-Aspartate Receptor Encephalitis Following COVID-19 Vaccination: A Case Report | Neurology | AntiNMDA | Scoop.it
Anti-N-methyl D-aspartate (NMDA) receptor (anti-NMDAR) encephalitis has been reported after SARS-CoV-2 infection, but not after SARS-CoV-2 vaccination. We report the first known case of anti-NMDAR encephalitis after SARS-CoV-2 immunization in a young female presenting with acute psychosis,...
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Lauren's Healing Story (Autoimmune Encephalitis) - Phoenix Helix

Lauren's Healing Story (Autoimmune Encephalitis) - Phoenix Helix | AntiNMDA | Scoop.it
When you have a disease only recently discovered and most doctors don't know it exists, it takes strong self-advocacy to get the help you need.
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CSF Findings in Acute NMDAR and LGI1 Antibody–Associated Autoimmune Encephalitis

CSF Findings in Acute NMDAR and LGI1 Antibody–Associated Autoimmune Encephalitis | AntiNMDA | Scoop.it
CSF in antibody-defined autoimmune encephalitis (AE) subtypes shows subtype-dependent degrees of inflammation ranging from rare and often mild to frequent and often robust. AEs with NMDA receptor antibodies (NMDAR-E) and leucine-rich glioma-inactivated ...
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Physical Therapy Interventions and Outcome Measures for a Patient Diagnosed with Anti-NMDA Receptor Encephalitis

Physical Therapy Interventions and Outcome Measures for a Patient Diagnosed with Anti-NMDA Receptor Encephalitis | AntiNMDA | Scoop.it
Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is the most common cause of
autoimmune encephalitis after acute demyelinating encephalitis. Patients usually present
with acute behavioral changes, psychosis, and abnormal limb movements and can also
...
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Celebrating Excellence IU School of Medicine student solves her own mystery illness

Celebrating Excellence IU School of Medicine student solves her own mystery illness | AntiNMDA | Scoop.it
In 2019, Dana Mitchell, a fourth-year medical student at IU School of Medicine, was diagnosed with autoimmune encephalitis—a brain inflammation that wreaks neurological havoc. In search of a therapy that would provide lasting relief of her debilitating symptoms, Mitchell reviewed 40...
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Autoimmune encephalitis with coexistent LGI1 and GABABR1 antibodies: case report | BMC Neurology | Full Text

Autoimmune encephalitis with coexistent LGI1 and GABABR1 antibodies: case report | BMC Neurology | Full Text | AntiNMDA | Scoop.it
Background Autoimmune encephalitis (AE) with multiple auto-antibodies is of great clinical significance because its complex clinical manifestations and atypical imaging increase the difficulty of diagnosis, differential diagnosis and treatment, which may aggravate the disease, increase the recurrence rate and mortality. The coexistence of anti-Leucinie-rich Glioma Inactivated 1 (LGI1) and anti-γ-aminobutyric acid-beta-receptor 1 (GABABR1) has not been published before. Case presentation We herein present the case of a 60-year-old man with slow response, behavioral changes, psychosis and sleep disorders. Laboratory test included serum hyponatremia, positive serum LGI1 and GABABR1 antibodies using transfected cell-based assays. Electroencephalogram exhibited moderate diffusion abnormality. The patient responded well to steroid impulse treatment and sodium supplement therapy, and did not recur during the follow-up. Conclusions Here we report the first AE characterized by positive LGI1 and GABABR1 antibodies, as well as summarizing AE with multiple auto-antibodies reported so far, hopefully to provide experience for clinical practice.
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EEG Contribution to the Diagnosis of Antibody-Negative Autoimmune Encephalitis: A Case Report - FullText - Case Reports in Neurology 2021, Vol. 13, No. 3 - Karger Publishers

Autoimmune encephalitis (AE) is a group of inflammatory brain diseases that are characterized by prominent neuropsychiatric symptoms. Early therapeutic intervention is important for AE.Therefore, wit...
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Author Response: Clinical, Neuroimmunologic, and CSF Investigations in First Episode Psychosis | Neurology

Author Response: Clinical, Neuroimmunologic, and CSF Investigations in First Episode Psychosis | Neurology | AntiNMDA | Scoop.it
We appreciate the interest in our research.1 According to Pollak and colleagues,2 criteria of possible autoimmune psychosis (AP) are fulfilled if a patient has abrupt onset psychotic symptoms with at least one of the following: the presence of a tumor, movement disorder (dyskinesias, catatonia), adverse response to antipsychotics, “severe or disproportionate” cognitive dysfunction, decreased level of consciousness, unexplained seizures, and significant autonomic dysfunction.2 Fulfilment of these criteria should lead to additional tests such as EEG, MRI, and serum or CSF investigations. In our series of 105 patients with first episode of psychosis (FEP), 20% fulfilled these criteria but never developed AP.1 We confirm that 2 of 3 patients with anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis presenting with FEP did not fulfill any of these criteria, including catatonia, which is a complex syndrome with its own set of 12 criteria that include echolalia.3 Thus, catatonia and echolalia should not be used as interchangeable terms.
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Autoantibody-associated psychiatric symptoms and syndromes in adults: A narrative review and proposed diagnostic approach

Autoantibody-associated psychiatric symptoms and syndromes in adults: A narrative review and proposed diagnostic approach | AntiNMDA | Scoop.it
Autoimmune-mediated encephalitis is a disease that often encompasses psychiatric symptoms as its first clinical manifestation’s predominant and isolated characteristic. Novel guidelines even distinguish autoimmune psychosis from autoimmune encephalitis.
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Vennada's Story: Recovery from Anti-NMDA Receptor Encephalitis

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The grey zone between autoimmune encephalitis and autoimmune‐associated epilepsy - Morano - - Epilepsia Open

The grey zone between autoimmune encephalitis and autoimmune‐associated epilepsy - Morano - - Epilepsia Open | AntiNMDA | Scoop.it
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The role of dendritic cells and their interactions in the pathogenesis of antibody-associated autoimmune encephalitis | Journal of Neuroinflammation | Full Text

The role of dendritic cells and their interactions in the pathogenesis of antibody-associated autoimmune encephalitis | Journal of Neuroinflammation | Full Text | AntiNMDA | Scoop.it
Autoimmune encephalitis (AE) is an inflammatory brain disease which is frequently associated with antibodies (Abs) against cell-surface, synaptic or intracellular neuronal proteins. There is increasing evidence that dendritic cells (DCs) are implicated as key modulators in keeping the balance...
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Unraveling the enigma of new-onset refractory status epilepticus: a systematic review of aetiologies | Read by QxMD

Unraveling the enigma of new-onset refractory status epilepticus: a systematic review of aetiologies | Read by QxMD | AntiNMDA | Scoop.it
RESULTS: Four hundred and fifty records were initially identified, of which 197 were included in the review. The selected studies were retrospective case-control (n=11), case series (n=83), and case reports (n=103) and overall described 1334 patients both of paediatric and adult age. Aetiology remains unexplained in about half of the cases, representing the so-called "cryptogenic NORSE". Among adult patients without cryptogenic NORSE, the most often identified cause is autoimmune encephalitis, either non-paraneoplastic or paraneoplastic. Infections are the prevalent aetiology of paediatric non-cryptogenic NORSE. Genetic and congenital disorders can have a causative role in NORSE, and toxic, vascular, and degenerative conditions have been also described.
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Screening for pathogenic neuronal autoantibodies in serum and CSF of patients with first-episode psychosis

Screening for pathogenic neuronal autoantibodies in serum and CSF of patients with first-episode psychosis | AntiNMDA | Scoop.it
Patients with autoimmune encephalitides, especially those with antibodies to the N-methyl-d-aspartate receptor (NMDAR), often present with prominent psychosis and respond well to immunotherapies. Although most patients progress to develop various neurological symptoms, it has been hypothesised...
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Rituximab Treatment and Long-term Outcome of Patients With Autoimmune Encephalitis | Neurology Neuroimmunology & Neuroinflammation

Rituximab Treatment and Long-term Outcome of Patients With Autoimmune Encephalitis | Neurology Neuroimmunology & Neuroinflammation | AntiNMDA | Scoop.it
Immunotherapeutic strategies for GAD65-AE remain highly controversial.27 Most patients are considered to require immunotherapy, and early immunotherapy has been found to be associated with a better outcome.10,28 However, the different neurologic manifestations of SPS, CA, and LE appear to respond...
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Anti-NMDAR Encephalitis: Multidisciplinary Development of a Clinical Practice Guideline | American Academy of Pediatrics

Anti-NMDAR Encephalitis: Multidisciplinary Development of a Clinical Practice Guideline | American Academy of Pediatrics | AntiNMDA | Scoop.it
Knowledge about the diagnosis of autoimmune encephalitis (AE) is rapidly expanding. In the last 15 years, multiple new antibodies have been described. Anti-N-methyl-D-aspartate receptor (NMDAR)–antibody-mediated encephalitis, in particular, has been found to be common among teenagers and young adults1 and accounts for up to 86% of AE in patients aged <18 years.2 Other antibodies associated with AE (leucine-rich glioma-inactivated 1, contactin-associated protein-like 2, glutamic acid decarboxylase 65-kilodalton isoform, γ-aminobutyric acid A, and α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid) are reported in children as case reports or series and with less clear typical clinical syndromes.3–9
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Comprehensive B-Cell Immune Repertoire Analysis of Anti-NMDAR Encephalitis and Anti-LGI1 Encephalitis

Comprehensive B-Cell Immune Repertoire Analysis of Anti-NMDAR Encephalitis and Anti-LGI1 Encephalitis | AntiNMDA | Scoop.it
Anti-N-methyl-D-aspartate receptor encephalitis (anti-NMDARE) and anti-leucine-rich glioma-inactivated 1 encephalitis (anti-LGI1E) are the two most common types of antibody-mediated autoimmune encephalitis.
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