Clinical characteristics and long-term prognosis of relapsing anti-N-methyl-d-aspartate receptor encephalitis: a retrospective, multicenter, self-controlled study

Abstract

Objective To analyze the clinical profile and long-term prognosis of relapsing anti- N -methyl- d -aspartate receptor (NMDAR) encephalitis. Method This 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. Results The 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. Conclusions Patients 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.

Neuropsychological Sequelae, Quality of Life and Adaptive Behavior in Children and Adolescents with Anti-NMDAR Encephalitis: A Narrative Review

“…In both pediatric and adult patients, a low score on the Glasgow Outcome Scale (used to investigate the outcome in head injury patients) [ 61 ] was strongly associated with poor quality of life [ 59 ]. In a recent study based on adults and children with anti-NMDAR encephalitis, Zeng et al showed that patients’ quality of life, evaluated by the Modified Rankin Scale, was better at relapse than at the onset [ 62 ]. Moreover, they found a positive influence of active immunotherapy on quality of life both at the onset and at the relapse.…”

Anti-NMDA receptor encephalitis and vaccination: A disproportionality analysis

“…However, full recovery is slow, and sequelae of varying intensity may persist, including memory, humour or behavioural disorders ( Irani et al, 2010 ; Barbagallo et al, 2017 ). Relapse has even been observed in 15%–25% of patients, albeit with less severe symptoms than during the first episode ( Zeng et al, 2021 ).…”

Use of anti-seizure medications in different types of autoimmune encephalitis: A narrative review

“…Early studies have found that ASMs can be gradually reduced during follow-up and that most patients who discontinue the drug within 2 years achieved seizure freedom (19). Furthermore, Zeng W et al found that most anti-NMDAR AE patients adhered to ASMs for 3-12 months (median 0.5 years), with a mean duration of less than 1 year on ASMs and a low relapse rate (20). Qi Huang Another study with a median follow-up of 2 years found that 33 patients (38.3%) on ASM discontinued their medication after 3 months of follow-up, 53 (61.7%) continued on ASM medication after 3 months of follow-up; additionally, no seizures were observed in patients with >2 years of follow-up, shortterm (<3 months) ASM treatment was associated with long-term (>3 months) ASM treatment, and there was no difference in seizure risk factors between these groups (14).…”