Läkartidningen
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Review Case Reports
[A recently acknowledged state with great variation in severity. NMDAR encephalitis frequently misinterpreted as psychiatric illness].
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. ⋯ 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.