Anti-NMDAR Encephalitis Complicated by Super Refractory Status Epilepticus: A Case Report and a Complex Review of Literature

Anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis characterized by the presence of immunoglobulin G-type autoantibodies against the NR1 subunit of the NMDA receptor is a novel disease. Main symptoms include complex neuropsychiatric features, seizures, or even super refractory status epilepticus (SRSE). Due to its debilitating manner, early recognition and a multidisciplinary approach including immunotherapy, neurointensive care, and rehabilitation are essential for proper treatment. We report a case of a 21-year-old male with anti-NMDAR encephalitis complicated by SRSE. He was admitted to the Psychiatric Ward due to hallucinations and aggressive behaviour. When he developed generalized seizures not responding to combined antiepileptic treatment, he was transferred to the Intensive Care Unit (ICU), where he underwent comprehensive neurocritical care including medical treatment, mechanical ventilation, hemodynamic support and close neuromonitoring. Cerebrospinal fluid and blood sample analysis confirmed anti-NMDAR encephalitis. Since first-line immunotherapies including high-dose steroids, intravenous immunoglobulins, and therapeutic plasma exchange failed, second-line treatment using rituximab was initiated. Despite some improvement, generalized seizures could not be controlled with standard antiepileptic therapy, thus continuous ketamine infusion was administered. After seven days on ketamine, cerebral convulsive activity resolved, and during the next five days ketamine was gradually withdrawn, meanwhile, a ketogenic diet was initiated. Our patient’s recovery was almost complete. He left the ICU and the hospital after 93 and 152 days, respectively. Our case report and literature review highlight the challenges and the urgent need to establish practice guidelines for the treatment of this novel disease, while emphasizes the indispensable role of interdisciplinary cooperation to achieve positive outcomes.