Anti-NMDA Receptor Encephalitis: Clinical Features and Basic Mechanisms
Autor: | Amy Rattelle, Amy J. Gleichman, Jessica A. Panzer, Kylie Roslin, David A. Lynch, Yi Na Dong |
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Rok vydání: | 2018 |
Předmět: |
0301 basic medicine
Anti-NMDA receptor encephalitis Psychosis business.industry media_common.quotation_subject medicine.disease Pathophysiology 03 medical and health sciences 030104 developmental biology 0302 clinical medicine nervous system medicine NMDA receptor Ovarian Teratoma Internalization Receptor business Neuroscience 030217 neurology & neurosurgery Encephalitis media_common |
Popis: | In slightly more than 10 years, anti-NMDA receptor (NMDAR) encephalitis has changed from a rare paraneoplastic syndrome to the most common cause of nonviral encephalitis. It presents fulminantly with progressive psychosis, seizures, and autonomic dysfunction, leading to death if untreated. However, rapid recognition and treatment can lead to survival and a return to baseline levels of functioning in many patients. While initially associated with ovarian teratomas, it is now associated with other tumors and can reflect a postviral event. The antibodies to the NMDAR made in this syndrome are pathogenic and are directed at the extracellular domain of the GluN1 subunit. Such antibodies lead to internalization of NMDARs in model systems, leading to a physiological state characterized by NMDAR hypofunction. Analogous disorders, characterized by antibodies to other synaptic receptors, present with neurological and psychiatric dysfunction and also appear to reflect antibody-induced internalization of receptors. However, this simple pathophysiology may be too simplistic to reflect the complexity of events in anti-NMDAR encephalitis. Future scientific investigations may allow a more complete understanding of this disorder and improve treatment of anti-NMDAR encephalitis. |
Databáze: | OpenAIRE |
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