Case of Severe Seronegative Autoimmune Rhombencephalitis Successfully Treated with Rituximab

Prompt treatment of autoimmune encephalitis can prevent long-term neurological sequelae. However, the data and evidence for the treatment of autoantibody negative encephalitis are limited, particularly so for rhombencephalitis. We report the case of a seronegative autoimmune rhombencephalitis successfully treated with rituximab. A 21-year-old woman was transferred to our center for progressive neuropsychiatric symptoms and neurological deficits progressing into a Locked-in state despite first-line immunotherapy with corticosteroids and immunoglobulins. She was started on a rituximab induction protocol with noticeable improvement within days. The patient then received a maintenance dose and during a follow-up after 9 months was still in remission, suggesting that rituximab can be an efficient treatment for severe refractory seronegative rhombencephalitis