| Title: |
NMDAR Encephalitis, a model of synaptopathy ; L'encéphalite à auto-anticorps anti-NMDAR, un modèle de synaptopathie |
| Authors: |
Chefdeville, Aude |
| Contributors: |
Institut NeuroMyoGène (INMG); Université Claude Bernard Lyon 1 (UCBL); Université de Lyon-Université de Lyon-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS); Université de Lyon; Jérôme Honnorat; Virginie Desestret |
| Source: |
https://theses.hal.science/tel-01885503 ; Neuroscience. Université de Lyon, 2017. English. ⟨NNT : 2017LYSE1301⟩. |
| Publisher Information: |
CCSD |
| Publication Year: |
2017 |
| Collection: |
HAL Lyon 1 (University Claude Bernard Lyon 1) |
| Subject Terms: |
Encephalitis; Autoantibodies; NMDAR; Teratoma; Encéphalite; Autoanticorps; Tératome; [SCCO.NEUR]Cognitive science/Neuroscience |
| Description: |
Anti-NMDA receptor (NMDAR) encephalitis is a rare but severe neuropsychiatric disorder initially described by J. Dalmau and colleagues in 2007. Anti-NMDAR encephalitis is defined by a clinical picture of encephalitis associated with the presence of IgG directed against the GluN1 subunit of NMDAR (NMDAR-Abs) in the cerebrospinal fluid (CSF) of patients. This disorder predominantly affects young women. Clinical presentation usually includes psychiatric symptoms and/or neurological symptoms often accompanied by decreased responsiveness and autonomic instabilities during the course of the disease . Despite the severity of the disease, 81% of patients recover fully or with mild sequels . 38% of patients had an underlying neoplasm, 94% of which were ovarian teratomas , indicating a role for this tumor in the immunopathogenesis of the disease. Studies in vitro and on animal models have demonstrated the pathogenicity of NMDAR-Abs but more studies are required to decipher the pathological role of anti-NMDAR antibodies. Two main research focuses have emerged in our group: understanding the events leading to the immune dysregulation in the ovary teratoma and identifying the pathological element(s) and how they act at the molecular and cellular levels to cause the broad neurological spectrum of symptoms observed in patients. My PhD was especially focused on 1) understanding the involvement of the underlying ovary teratoma in the triggering of the immune response during anti-NMDAR encephalitis and 2) studying the impact of prolonged exposure of the neuronal network to patients’ NMDAR-Abs and the potential involvement of microglial cells in the physiopathology of the disease ; Que se passe-t-il quand le système immunitaire attaque le cerveau ? Dans l'encéphalite à autoanticorps dirigés contre les récepteurs NMDA, le système immunitaire des patients dysfonctionne : au lieu de produire des anticorps pour combattre des organismes pathogènes, le système immunitaire produit des anticorps qui attaquent une protéine spécifique dans ... |
| Document Type: |
doctoral or postdoctoral thesis |
| Language: |
English |
| Relation: |
NNT: 2017LYSE1301 |
| Availability: |
https://theses.hal.science/tel-01885503; https://theses.hal.science/tel-01885503v1/document; https://theses.hal.science/tel-01885503v1/file/TH2017CHEFDEVILLEAUDE.pdf |
| Rights: |
https://about.hal.science/hal-authorisation-v1/ ; info:eu-repo/semantics/OpenAccess |
| Accession Number: |
edsbas.922EBE43 |
| Database: |
BASE |