Immunotherapy for anti-NMDA receptor encephalitis: Experience from a single center in Taiwan
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 pati...
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Format: | Article |
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Elsevier
2019-08-01
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Series: | Pediatrics and Neonatology |
Online Access: | http://www.sciencedirect.com/science/article/pii/S1875957218303541 |
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doaj-f190db7eca6d4e3db52e315146654abe |
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Article |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Shu-Sing Kong Yun-Ju Chen I-Chen Su Jainn-Jim Lin I-Jun Chou Min-Liang Chou Po-Cheng Hung Meng-Ying Hsieh Yi-Shan Wang Cheng-Che Chou Huei-Shyong Wang Kuang-Lin Lin |
spellingShingle |
Shu-Sing Kong Yun-Ju Chen I-Chen Su Jainn-Jim Lin I-Jun Chou Min-Liang Chou Po-Cheng Hung Meng-Ying Hsieh Yi-Shan Wang Cheng-Che Chou Huei-Shyong Wang Kuang-Lin Lin Immunotherapy for anti-NMDA receptor encephalitis: Experience from a single center in Taiwan Pediatrics and Neonatology |
author_facet |
Shu-Sing Kong Yun-Ju Chen I-Chen Su Jainn-Jim Lin I-Jun Chou Min-Liang Chou Po-Cheng Hung Meng-Ying Hsieh Yi-Shan Wang Cheng-Che Chou Huei-Shyong Wang Kuang-Lin Lin |
author_sort |
Shu-Sing Kong |
title |
Immunotherapy for anti-NMDA receptor encephalitis: Experience from a single center in Taiwan |
title_short |
Immunotherapy for anti-NMDA receptor encephalitis: Experience from a single center in Taiwan |
title_full |
Immunotherapy for anti-NMDA receptor encephalitis: Experience from a single center in Taiwan |
title_fullStr |
Immunotherapy for anti-NMDA receptor encephalitis: Experience from a single center in Taiwan |
title_full_unstemmed |
Immunotherapy for anti-NMDA receptor encephalitis: Experience from a single center in Taiwan |
title_sort |
immunotherapy for anti-nmda receptor encephalitis: experience from a single center in taiwan |
publisher |
Elsevier |
series |
Pediatrics and Neonatology |
issn |
1875-9572 |
publishDate |
2019-08-01 |
description |
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. Key Words: cyclophosphamide, anti-NMDAR encephalitis, immunotherapy, intravenous immunoglobulin, rituximab |
url |
http://www.sciencedirect.com/science/article/pii/S1875957218303541 |
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doaj-f190db7eca6d4e3db52e315146654abe2020-11-25T00:44:06ZengElsevierPediatrics and Neonatology1875-95722019-08-01604417422Immunotherapy for anti-NMDA receptor encephalitis: Experience from a single center in TaiwanShu-Sing Kong0Yun-Ju Chen1I-Chen Su2Jainn-Jim Lin3I-Jun Chou4Min-Liang Chou5Po-Cheng Hung6Meng-Ying Hsieh7Yi-Shan Wang8Cheng-Che Chou9Huei-Shyong Wang10Kuang-Lin Lin11Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, TaiwanDivision of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, TaiwanDivision of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, TaiwanDivision 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, TaiwanDivision 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, TaiwanDivision 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, TaiwanDivision 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, TaiwanDivision 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, TaiwanDivision 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, TaiwanDivision 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, TaiwanDivision 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, TaiwanDivision 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; Corresponding author. Division of Pediatric Neurology, Chang Gung Children's Hospital, 5 Fu-Shin Street, Kwei-Shan, Taoyuan, 333, Taiwan. Tel.: +886 3 3281200 ext. 8200. Fax: +886 3 3288957.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. Key Words: cyclophosphamide, anti-NMDAR encephalitis, immunotherapy, intravenous immunoglobulin, rituximabhttp://www.sciencedirect.com/science/article/pii/S1875957218303541 |