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.

The Emerging Role of N-Methyl-D-Aspartate (NMDA) Receptors in the Cardiovascular System: Physiological Implications, Pathological Consequences, and Therapeutic Perspectives

“…Finally, anti-NMDAR autoimmune encephalitis is a rare neurological disorder that is caused by autoreactive anti-NMDAR autoantibodies targeting GluN1 and produced by B lymphocytes [ 151 , 152 ]. This in turn results in NMDAR hypo-responsiveness and leads to a decline in cognitive function and neuropsychiatric symptoms [ 153 ]. Future work should assess whether anti-NMDAR autoantibodies also target the endothelial NMDARs, as suggested by the defect in BBB integrity that has been reported in both animal models [ 152 ] and patients [ 154 ] affected by this disease.…”

Immunopathology of herpes simplex virus‐associated neuroinflammation: Unveiling the mysteries

“…Confirmation of the diagnosis is facilitated through CSF testing for anti-NMDA receptor antibodies. 145,146 Recent studies have explored distinctive aspects of this disorder.…”

The Leading Role of Brain and Abdominal Radiological Features in the Work-Up of Anti-NMDAR Encephalitis in Children: An Up-To-Date Review

“…In adults, the use of methylprednisolone 1 g for 3–7 days is recommended as an initial approach [ 20 ]. First-line therapy also includes intravenous immunoglobulins (IVIg) or plasma exchange, often in conjunction with corticosteroids [ 8 , 50 ]. A second-line therapy with rituximab and/or cyclophosphamide is uncommonly required [ 8 , 50 ].…”

“…First-line therapy also includes intravenous immunoglobulins (IVIg) or plasma exchange, often in conjunction with corticosteroids [ 8 , 50 ]. A second-line therapy with rituximab and/or cyclophosphamide is uncommonly required [ 8 , 50 ]. Additional complementary immunotherapies, such as bortezomib, and/or tocilizumab, may be considered in resistant cases [ 50 ].…”

“…A second-line therapy with rituximab and/or cyclophosphamide is uncommonly required [ 8 , 50 ]. Additional complementary immunotherapies, such as bortezomib, and/or tocilizumab, may be considered in resistant cases [ 50 ]. According to the literature, corticosteroids are the most popular therapeutic choice in NMDARe patients, although they may potentially cause an initial worsening of the behavioral or psychiatric symptoms, hampering a timely evaluation of the treatment response [ 20 ].…”