TY - JOUR
T1 - Anti-NMDA Receptor Autoimmune Encephalitis
T2 - Diagnosis and Management Strategies
AU - Nguyen, Linda
AU - Wang, Cynthia
N1 - Publisher Copyright:
© 2023 Nguyen and Wang.
PY - 2023
Y1 - 2023
N2 - Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is the most recognized form of autoimmune encephalitis. It is characterized by a constellation of neurologic and psychiatric features along with positive NMDAR antibody, which is more sensitive and specific in CSF than serum. All patients should be screened at least once for neoplasm, with ovarian teratoma being found in most tumor-related cases. In the acute phase, first-line immunotherapy, often a combination of high-dose steroids, immunoglobulins, and/or plasma exchange, is strongly recommended. When first-line therapy fails, escalation to second-line immunotherapy, particularly rituximab, can further improve outcomes and prevent relapses. In refractory cases, additional complementary immunotherapies, such as cyclophosphamide, bortezomib and/or tocilizumab may be considered. Relapses occur in 10–30% of cases, mostly within the first two years from onset. Individuals should be followed up to determine if chronic maintenance therapy is required.
AB - Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is the most recognized form of autoimmune encephalitis. It is characterized by a constellation of neurologic and psychiatric features along with positive NMDAR antibody, which is more sensitive and specific in CSF than serum. All patients should be screened at least once for neoplasm, with ovarian teratoma being found in most tumor-related cases. In the acute phase, first-line immunotherapy, often a combination of high-dose steroids, immunoglobulins, and/or plasma exchange, is strongly recommended. When first-line therapy fails, escalation to second-line immunotherapy, particularly rituximab, can further improve outcomes and prevent relapses. In refractory cases, additional complementary immunotherapies, such as cyclophosphamide, bortezomib and/or tocilizumab may be considered. Relapses occur in 10–30% of cases, mostly within the first two years from onset. Individuals should be followed up to determine if chronic maintenance therapy is required.
KW - anti-NMDAR encephalitis
KW - clinical features
KW - immunotherapies
KW - treatment options
UR - http://www.scopus.com/inward/record.url?scp=85146174112&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85146174112&partnerID=8YFLogxK
U2 - 10.2147/IJGM.S397429
DO - 10.2147/IJGM.S397429
M3 - Review article
C2 - 36628299
AN - SCOPUS:85146174112
SN - 1178-7074
VL - 16
SP - 7
EP - 21
JO - International Journal of General Medicine
JF - International Journal of General Medicine
ER -