Clinical characteristic and prognostic analysis of severe anti-N-methyl-D-aspartate receptor encephalitis / 临床神经病学杂志

ABSTRACT

Objective To observe the clinical characteristic and prognostic analysis of severe anti-N-methyl-D-aspartate receptor ( anti-NMDAR) encephalitis.Methods The clinical data of 9 patients with severe anti-NMDAR encephalitis were retrospective analyzed.Results Nine patients ( two male and seven females ) were taken care at ICU.They were 27.7 years old at average.The median course was 22.4 d and median length of stay in ICU was 50. 9 d.The mainly clinical characteristics were fever(7 cases), psychiatric behavioral symptoms(9 cases), seizures(9 cases) , decreased consciousness ( 8 cases ) , abnormal movement ( 7 cases ) , automatic instability ( 9 cases ) and hypoventilation(6 cases) .Three cases had ovarian teratoma.Anti-NMDAR antibodies in CSF of all patients were testing positive, while in serum were 6 cases.CSF-IgA in 7 cases were increased.The brain MRI indicated abnormal signals in temporal lobe, hippocampus in 3 patients.The EEG showed slowing wave abnormal in 5 patients.All patients were treated with combined first-line immunomodulatory therapies with steroids, intravenous immunoglobulin, or plasma exchange. Five patients were fullly recoveried, 4 patients were improved with residual symptoms. Conclusions Severe anti-NMDAR encephalitis may present progress quickly mental behavior change, epilepsy, and disturbance of consciousness, movement disorders, autonomic nerve dysfunction.Which accompanied with tumor is rare.Most patients with active immunotherapy can get good prognosis.