Treatment, outcome, and relapses

About 75% of patients with NMDAR antibodies recover or have mild sequelae; all other patients remain severely disabled or die. Management of anti-NMDAR encephalitis should initially focus on immunotherapy and the detection and removal of a teratoma. Most patients receive corticosteroids, intravenous immunoglobulins (IVIg) or plasma exchange as first-line of immunotherapy. These treatments have enhanced effectiveness and speed of action when patients have an underlying tumor that is removed. In patients without a tumour or with delayed diagnosis, additional treatment with second-line immunotherapy (a rituximab or cyclophosphamide, or both) is usually needed. Our experience with 105 consecutively diagnosed patients in 2009 accord with these findings. Although 80% of patients with a tumour (mostly teratomas) had substantial improvement after tumour removal and first-line immunotherapy, only 48% of those without a tumor had a similar degree of improvement after first-line immunotherapy and needed second-line immunotherapy more often. Overall, second-line immunotherapy resulted in substantial improvement in 15 of 23 (65%) patients. The final outcome, substantial improvement (as previously defined), was much the same in patients with or without tumor (84% vs 71%), but the five patients who died did not have a tumor and did not receive second-line immunotherapy.
Treatment and outcome in 105 patients comparing presence and absence of tumour and the use of second-line immunotherapy