Clinical Manifestations and Outcomes of Pediatric Anti-N-methyl-D-aspartate Receptor (NMDAR) Encephalitis in Srinagarind Hospital
Keywords:
Anti-N-methyl-D-aspartate receptor encephalitis, children, clinical manifestation, outcome, relapseAbstract
Background and Objective: Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is common and treatable autoimmune encephalitis in children. This study aimed to describe clinical manifestation, investigation, treatment, outcome, and possible factors associated with relapse in pediatric anti-NMDAR encephalitis in Srinagarind Hospital.
Methods: The medical records of 10 children diagnosed with anti-NMDAR encephalitis at Srinagarind Hospital during January 2016-March 2020 were retrospectively reviewed.
Results: The median age of patients was 9.5 years (1-16 years), 90% were female. The most common presentation in pre-puberty was seizure, post-puberty was behavioral symptoms. CSF and serum NMDAR antibodies were identified in all patients. Three patients had evidence of previous viral encephalitis (2 Herpes simplex encephalitis (HSE),1 Varicella zoster encephalitis). Electroencephalography revealed focal epileptiform discharge (60%), extreme delta brush (40%), generalized slow activity (30%). Neuroimaging of previous HSE showed temporal lobe abnormalities (20%), the rest of patients showed non-specific disease finding. Neoplasms were not detected. All received first-line and maintenance immunotherapy. Second-line immunotherapy was given to 4 patients. Six patients (60%) improved after first-line immunotherapy, 2/6 patients developed relapse. Five patients (50%) had complete recovery, Pediatric cerebral performance category (PCPC) score of 1 at last follow-up. The median interval between symptom onset and initiation of immunotherapy was significantly longer in relapse group (78.5 days, IQR 74-83) than non-relapse group (19 days, IQR 13.5-28.5) (p = 0.036).
Conclusions: Seizure was predominated in young children while behavior change typically presented in adolescents. Preceding viral infection may be a trigger and associated with unfavorable outcome. Prolonged interval between symptom onset and first-line immunotherapy was one of possible associated factor of relapse.
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