J Clin Neurol.  2021 Apr;17(2):300-306. 10.3988/jcn.2021.17.2.300.

Clinical Features and Treatment Outcomes of Seronegative Pediatric Autoimmune Encephalitis

• Lee S
• Kim HD
• Lee JS
• Kang HC
• Kim SH

• KMID: 2514551
• DOI: http://doi.org/10.3988/jcn.2021.17.2.300

Abstract

Background and Purpose
New diagnostic criteria for pediatric autoimmune encephalitis (AIE) have been introduced recently. A substantial proportion of cases of pediatric AIE are diagnosed as seronegative based on these criteria, and so the clinical characteristics of this group remain to be investigated.
Methods
This study included 46 pediatric patients younger than 18 years with suspected AIE. Clinical features, laboratory or radiological findings, and treatment outcomes were compared between seronegative and seropositive patients.
Results
Nine (19.6%) of the 46 patients were diagnosed as seropositive AIE. All of the patients with seropositive AIE had anti-N-methyl-D-aspartate receptor antibodies. Commonly identified neuropsychiatric symptoms were altered mental status, cognitive dysfunction, seizure, speech dysfunction, and psychotic disorder in both the seronegative and seropositive groups. Immunotherapy produced favorable treatment outcomes in both the seropositive (n=7, 77.8%) and seronegative (n=35, 94.6%) AIE patients. Treatment outcomes for first-line immunotherapy were better in seronegative AIE than seropositive AIE patients (p=0.003), and hence a smaller proportion of seronegative patients required second-line treatment (p=0.015).
Conclusions
Pediatric seronegative AIE patients showed clinical presentations similar to those of seropositive AIE patients, with favorable treatment outcomes after immunotherapy.

Keyword

encephalitis; autoimmune encephalitis; seronegative encephalitis.