Anti-NMDA Receptor Autoimmune Encephalitis: Diagnosis and Management Strategies

Abstract

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.

Keywords:

• anti-NMDAR encephalitis
• clinical features
• immunotherapies
• treatment options

Abbreviations

AE, autoimmune encephalitis; CASE, clinical assessment scale for autoimmune encephalitis; CASPR2, contactin-associated protein-like 2; CSF, cerebral spinal fluid; EEG, electroencephalogram; ICU, intensive care unit; IVIG, intravenous immunoglobulins; LGI1, leucine-rich glioma-inactivated 1; MRI, magnetic resonance imaging; mRS, modified Rankin Scale; NMDAR, N-methyl-d-aspartate receptor; PLEX, plasma exchange; SIRT-T, steroid, IVIG, rituximab and tocilizumab with tumor removal.

Disclosure

The authors report no conflicts of interest in this work.