[A recently acknowledged state with great variation in severity. NMDAR encephalitis frequently misinterpreted as...

Since first described less than ten years ago, anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is now recognized as one of the most common causes of autoimmune encephalitis. The disorder is more common in women (80%), where it is frequently associated with an underlying ovarian teratoma. Most patients with anti-NMDAR encephalitis develop a multi-stage illness that progresses from initially psychiatric symptoms to subsequent mnemonic disturbances, seizures, dyskinesias, catatonia and even coma. Since the neurological and psychiatric symptoms can be highly variable upon presentation, the disease is probably under-diagnosed. Outcome is likely to be improved with prompt diagnosis and early treatment. Here we describe a hospital-based case series of six patients that illustrate the wide clinical spectrum of the condition. The series also includes one severely ill patient with treatment-refractory disease who was hospitalized with invasive ventilation for seven months, and eventually responded to a combination of rituximab, cyclophosphamide and bortezomib.