FACTORS ASSOCIATED WITH POOR CORTICOSTEROID RESPONSE IN PEDIATRIC PATIENTS WITH FIRST-DIAGNOSED AUTOIMMUNE ENCEPHALITIS

Khánh Huyền Lê, Đắc Anh Quân Trần, Hải Uyên Phạm, Lê Trung Hiếu Nguyễn

Main Article Content

Abstract

Background: Corticosteroids are the first-line immunotherapy for autoimmune encephalitis (AE) in children. Early prediction of treatment response is essential for selecting optimal therapeutic strategies and improving patient outcomes. Methods: A retrospective study was conducted on 85 pediatric patients newly diagnosed with AE at Children’s Hospital 2, from January 2021 to June 2024, based on Cellucci criteria. Clinical outcomes were assessed using the Clinical Assessment Scale for Autoimmune Encephalitis (CASE) at baseline, 1–2 weeks post-treatment, and after 3 months of follow-up. Results: 70.6% of patients showed improvement in CASE scores after 1–2 weeks of corticosteroid therapy; 25.9% required additional immunotherapy (IVIG, plasmapheresis, or rituximab). The overall corticosteroid response rate for 3 months was 68%. Patients with poor response had significantly higher rates of cognitive impairment (96.3% vs. 75.9%, p=0.03), consciousness disorders (70.4% vs. 44.8%, p=0.028), motor dysfunction (85.2% vs. 50%, p=0.002), and elevated cerebrospinal fluid (CSF) lactate levels (p=0.017). Conclusions: Clinical features such as consciousness disturbance, speech impairment, motor dysfunction, higher initial CASE scores, and elevated CSF lactate are associated with poor corticosteroid response in children with AE. These indicators may aid in early identification of patients requiring escalated immunotherapy.

Article Details

References

1. Uy CE, Binks S, Irani SR. Autoimmune encephalitis: clinical spectrum and management. Practical neurology. Oct 2021;21(5):412-423. doi:10.1136/practneurol-2020-002567
2. Liu F, Zhang B, Huang T, et al. Influential Factors, Treatment and Prognosis of Autoimmune Encephalitis Patients With Poor Response to Short-Term First-Line Treatment. Frontiers in neurology. 2022;13:861988. doi:10.3389/fneur. 2022.861988
3. Titulaer MJ, McCracken L, Gabilondo I, et al. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. The Lancet Neurology. Feb 2013;12(2):157-65. doi:10.1016/s1474-4422(12)70310-1
4. Nosadini M, Thomas T, Eyre M, et al. International Consensus Recommendations for the Treatment of Pediatric NMDAR Antibody Encephalitis. Neurology(R) neuroimmunology & neuroinflammation. Jul 2021;8(5)doi:10.1212/ nxi.0000000000001052
5. Kang Q, Liao H, Yang L, Fang H, Hu W, Wu L. Clinical Characteristics and Short-Term Prognosis of Children With Antibody-Mediated Autoimmune Encephalitis: A Single-Center Cohort Study. Original Research. 2022-July-08 2022; 10doi:10.3389/fped.2022.880693
6. Zhang J, Ji T, Chen Q, et al. Pediatric Autoimmune Encephalitis: Case Series From Two Chinese Tertiary Pediatric Neurology Centers. Frontiers in neurology. 2019;10:906. doi:10.3389/ fneur.2019.00906
7. Kanmaz S, Yılmaz S, Toprak DE, et al. Assessment of Prognostic Factors and Validity of Scoring Models in Childhood Autoimmune Encephalitis. Turkish archives of pediatrics. Mar 2023;58(2): 142-153. doi:10.5152/TurkArch Pediatr.2023.2219850.
8. Zhou H, Deng Q, Yang Z, et al. Performance of the clinical assessment scale for autoimmune encephalitis in a pediatric autoimmune encephalitis cohort. Frontiers in immunology. 2022;13:915352. doi:10.3389/ fimmu.2022. 915352