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Immunotherapy for anti-NMDA receptor encephalitis: Experience from a single center in Taiwan.
Pediatr Neonatol. 2019 08; 60(4):417-422.PN

Abstract

BACKGROUND

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is an anti-neuronal antibody-mediated inflammatory brain disease that causes severe psychiatric and neurological deficits in previously healthy patients. The aims of this study were to demonstrate the clinical characteristics of patients diagnosed with anti-NMDA receptor encephalitis and to compare the different treatment strategies among these patients.

METHODS

Patients presenting with newly acquired psychiatric and/or neurological deficits were studied retrospectively from 2009 to 2017. Patients with evidence of anti-NMDA receptor antibodies in serum and/or cerebrospinal fluid were enrolled. The modified Rankin scale was used to assess the initial status and outcomes of the enrolled patients. Details of the clinical presentations and results of investigations were analyzed.

RESULTS

All (n = 24) of the patients received first-line immunotherapy (steroids, and/or intravenous immunoglobulin, and/or plasma exchange), and 14 patients received second-line immunotherapy (rituximab and/or cyclophosphamide). The mean time between the first- and second-line treatment was 13 days. During the first 6 months, 20 patients (20/24, 83%) achieved a good outcome (modified Rankin Scale score ≤2) and 15 patients (15/24, 62.5%) completely recovered. Four patients (17.7%) relapsed, and three patients (12.5%) had associated tumors.

CONCLUSION

Immunotherapy is an effective treatment for anti-NMDA receptor encephalitis. Rituximab and/or cyclophosphamide are treatment options for those who cannot tolerate or do not respond to first-line immunotherapy. Prospective studies are necessary to investigate the role of rituximab and cyclophosphamide in anti-NMDA receptor encephalitis.

Authors+Show Affiliations

Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan. Electronic address: mr0701@cgmh.org.tw.Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan. Electronic address: mr0843@cgmh.org.tw.Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan. Electronic address: eternalmummi@gmail.com.Division of Pediatric Critical Care and Pediatric Neurocritical Care Center, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan; Graduate Institute of Clinical Medical Sciences, College of Medicine, Chang Gung University, Taoyuan, Taiwan; Chang Gung Children's Hospital Study Group for Children with Encephalitis/Encephalopathy Related Status Epilepticus and Epilepsy (CHEESE), Taoyuan, Taiwan. Electronic address: lin0227@cgmh.org.tw.Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan; Chang Gung Children's Hospital Study Group for Children with Encephalitis/Encephalopathy Related Status Epilepticus and Epilepsy (CHEESE), Taoyuan, Taiwan. Electronic address: ijun@adm.cgmh.org.tw.Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan; Chang Gung Children's Hospital Study Group for Children with Encephalitis/Encephalopathy Related Status Epilepticus and Epilepsy (CHEESE), Taoyuan, Taiwan. Electronic address: mlchou@adm.cgmh.org.tw.Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan; Chang Gung Children's Hospital Study Group for Children with Encephalitis/Encephalopathy Related Status Epilepticus and Epilepsy (CHEESE), Taoyuan, Taiwan. Electronic address: h2918@cgmh.org.tw.Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan; Chang Gung Children's Hospital Study Group for Children with Encephalitis/Encephalopathy Related Status Epilepticus and Epilepsy (CHEESE), Taoyuan, Taiwan. Electronic address: ying2014@adm.cgmh.org.tw.Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan; Chang Gung Children's Hospital Study Group for Children with Encephalitis/Encephalopathy Related Status Epilepticus and Epilepsy (CHEESE), Taoyuan, Taiwan. Electronic address: hermitage513@gmail.com.Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan; Chang Gung Children's Hospital Study Group for Children with Encephalitis/Encephalopathy Related Status Epilepticus and Epilepsy (CHEESE), Taoyuan, Taiwan; Division of Pediatrics, Keelung Chang Gung Memorial Hospital, Keelung, Taiwan. Electronic address: greatsam1137010016@gmail.com.Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan; Chang Gung Children's Hospital Study Group for Children with Encephalitis/Encephalopathy Related Status Epilepticus and Epilepsy (CHEESE), Taoyuan, Taiwan. Electronic address: wanghs444@cgmh.org.tw.Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan; Chang Gung Children's Hospital Study Group for Children with Encephalitis/Encephalopathy Related Status Epilepticus and Epilepsy (CHEESE), Taoyuan, Taiwan. Electronic address: lincgh@cgmh.org.tw.Chang Gung Children's Hospital Study Group for Children with Encephalitis/Encephalopathy Related Status Epilepticus and Epilepsy (CHEESE), Taoyuan, Taiwan.

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

30449706

Citation

Kong, Shu-Sing, et al. "Immunotherapy for anti-NMDA Receptor Encephalitis: Experience From a Single Center in Taiwan." Pediatrics and Neonatology, vol. 60, no. 4, 2019, pp. 417-422.
Kong SS, Chen YJ, Su IC, et al. Immunotherapy for anti-NMDA receptor encephalitis: Experience from a single center in Taiwan. Pediatr Neonatol. 2019;60(4):417-422.
Kong, S. S., Chen, Y. J., Su, I. C., Lin, J. J., Chou, I. J., Chou, M. L., Hung, P. C., Hsieh, M. Y., Wang, Y. S., Chou, C. C., Wang, H. S., & Lin, K. L. (2019). Immunotherapy for anti-NMDA receptor encephalitis: Experience from a single center in Taiwan. Pediatrics and Neonatology, 60(4), 417-422. https://doi.org/10.1016/j.pedneo.2018.10.006
Kong SS, et al. Immunotherapy for anti-NMDA Receptor Encephalitis: Experience From a Single Center in Taiwan. Pediatr Neonatol. 2019;60(4):417-422. PubMed PMID: 30449706.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Immunotherapy for anti-NMDA receptor encephalitis: Experience from a single center in Taiwan. AU - Kong,Shu-Sing, AU - Chen,Yun-Ju, AU - Su,I-Chen, AU - Lin,Jainn-Jim, AU - Chou,I-Jun, AU - Chou,Min-Liang, AU - Hung,Po-Cheng, AU - Hsieh,Meng-Ying, AU - Wang,Yi-Shan, AU - Chou,Cheng-Che, AU - Wang,Huei-Shyong, AU - Lin,Kuang-Lin, AU - ,, Y1 - 2018/10/31/ PY - 2018/06/21/received PY - 2018/09/05/revised PY - 2018/10/25/accepted PY - 2018/11/20/pubmed PY - 2020/1/29/medline PY - 2018/11/20/entrez KW - anti-NMDAR encephalitis KW - cyclophosphamide KW - immunotherapy KW - intravenous immunoglobulin KW - rituximab SP - 417 EP - 422 JF - Pediatrics and neonatology JO - Pediatr Neonatol VL - 60 IS - 4 N2 - BACKGROUND: Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is an anti-neuronal antibody-mediated inflammatory brain disease that causes severe psychiatric and neurological deficits in previously healthy patients. The aims of this study were to demonstrate the clinical characteristics of patients diagnosed with anti-NMDA receptor encephalitis and to compare the different treatment strategies among these patients. METHODS: Patients presenting with newly acquired psychiatric and/or neurological deficits were studied retrospectively from 2009 to 2017. Patients with evidence of anti-NMDA receptor antibodies in serum and/or cerebrospinal fluid were enrolled. The modified Rankin scale was used to assess the initial status and outcomes of the enrolled patients. Details of the clinical presentations and results of investigations were analyzed. RESULTS: All (n = 24) of the patients received first-line immunotherapy (steroids, and/or intravenous immunoglobulin, and/or plasma exchange), and 14 patients received second-line immunotherapy (rituximab and/or cyclophosphamide). The mean time between the first- and second-line treatment was 13 days. During the first 6 months, 20 patients (20/24, 83%) achieved a good outcome (modified Rankin Scale score ≤2) and 15 patients (15/24, 62.5%) completely recovered. Four patients (17.7%) relapsed, and three patients (12.5%) had associated tumors. CONCLUSION: Immunotherapy is an effective treatment for anti-NMDA receptor encephalitis. Rituximab and/or cyclophosphamide are treatment options for those who cannot tolerate or do not respond to first-line immunotherapy. Prospective studies are necessary to investigate the role of rituximab and cyclophosphamide in anti-NMDA receptor encephalitis. SN - 2212-1692 UR - https://www.unboundmedicine.com/medline/citation/30449706/Immunotherapy_for_anti_NMDA_receptor_encephalitis:_Experience_from_a_single_center_in_Taiwan_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1875-9572(18)30354-1 DB - PRIME DP - Unbound Medicine ER -