BackgroundThe association of N-methyl d-aspartate receptor-antibodies (NMDAR-Abs) and encephalitis is now well recognised. MethodsRetrospective review of frequency of diagnosis and outcomes in encephalitis with NMDAR-Abs identified at the Walton Centre between 2012–2017.ResultsNMDAR-Abs were detected in 29/1131 (3%) of sera and/or CSF samples. Of 20 (69%) patients with encephalitis, 50% were identified in the last two years. Median onset age was 34(17–75) years and 60% (12/20) were female. Median symptom duration before diagnosis was 24(2–720) weeks between 2012–2015, improving to 14(1–96) weeks between 2015–2016. Five patients (25%) had an infectious prodrome (one prior HSV-1 encephalitis). Psychiatric/cognitive symptoms, seizures, and movement disorders were present in 80% (16/20), 70% (14/20), and 55% (11/20) of patients respectively. Three patients had ovarian teratomas.Electroencephalograms were abnormal in 65% (15/23) and MRI brain in 37% (7/19) patients. Unmatched intrathecal oligoclonal bands and CSF pleocytosis were present in 31% (5/16) and 60% (15/25) of samples respectively. Immunotherapy was beneficial in 73% (11/15) of patients. Two patients died (sepsis and multi-organ failure) and two improved spontaneously. At a median follow-up of 9 (5–180) months, 69% (11/16) of patients had an mRS ≤2. ConclusionAlthough the recognition of NMDAR-Ab encephalitis has improved, there is still a significant delay to diagnosis. The majority of patients have good outcomes.
Seizures and memory impairment induced by patient‐derived anti‐N‐methyl‐D‐aspartate receptor antibodies in mice are attenuated by anakinra, an interleukin‐1 receptor antagonist
