Abstract
ObjectiveTo analyze the clinical profile and long-term prognosis of relapsing anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis.MethodThis is a retrospective, multicenter, self-controlled study of 10 patients with relapsing anti-NMDAR encephalitis. Relapse was defined as new psychiatric or neurologic syndrome unexplainable by other causes that improved after immunotherapy.ResultsThe main symptoms at first onset and relapse included psychiatric symptoms, cognitive impairment, speech dysfunction, seizures, consciousness disturbance, movement disorders, central hypoventilation, and autonomic dysfunction. There were significantly fewer seizures and consciousness disturbances at relapse. At the first onset, the antibody positivity rate was significantly higher in the cerebrospinal fluid (CSF) than in the serum, and abnormal electroencephalograms results were noted in all patients. The relapse rate was 12.2%. After first-onset discharge, the duration of medication intake was 3.10 ± 2.69 months; the relapse time was 18.3 ± 16.5 months. The Modified Rankin Scale (MRS) score at relapse was significantly lower than that at the first onset. The MRS scores at relapse and first onset after immunotherapy were significantly lower than those before immunotherapy. At follow-up, the average duration of antiepileptic drug (AED) intake was < 1 year; the relapse rate was low.ConclusionsPatients have fewer symptoms and better quality of life at relapse than at the first onset. Active immunotherapy can significantly improve the quality of life during first onset and relapse. Encephalitis antibody testing in the CSF is preferred at first onset and relapse. Increasing antibody titers suggest clinical relapse. Prematurely stopping immunotherapy may lead to relapse, but prolonged AED intake is unnecessary.
Highlights
Anti-N-methyl-D-aspartate receptor encephalitis is a rapidly progressing encephalopathy characterized by abnormal behavior, cognitive dysfunction, speech dysfunction, seizures, movement disorders, consciousness disturbance, Material and methodsThis retrospective, multicenter, self-controlled study analyzed 82 patients with anti-NMDAR encephalitis from February 2013 to September 2018
Relapsing anti-NMDAR encephalitis [5] was defined as any new psychiatric or neurologic syndrome that cannot be explained by other causes and that improved after immunotherapy or, less frequently, spontaneously
Patients were less likely to be admitted to the intensive care unit (ICU) at relapse than at first onset
Summary
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a rapidly progressing encephalopathy characterized by abnormal behavior, cognitive dysfunction, speech dysfunction, seizures, movement disorders, consciousness disturbance, Material and methods. This retrospective, multicenter, self-controlled study analyzed 82 patients with anti-NMDAR encephalitis from February 2013 to September 2018. NMDAR encephalitis [4] are rapid onset (course < 3 months) of at least one of the following symptoms: abnormal behavior (psychiatric symptoms) or cognitive dysfunction, speech dysfunction (uninterruptible imperative language, being quiet), epileptic seizure, movement disorders (dyskinesia, rigidity, or postural difficulties), decreased level of consciousness, autonomic dysfunction, or central hypoventilation; positive cerebrospinal fluid (CSF) anti-NMDAR antibody test; and no other possible causes, such as viral encephalitis and herpes simplex encephalitis. 10 patients with relapse (9 patients at the First Affiliated Hospital of Guangxi Medical University and 1 patient at Hechi People’s Hospital; 6 males and 4 females) with a mean age of 25.6 ± 14.1 years (range, 8–50 years) at the first onset were included
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