Clinical Features, Treatment, and Outcomes Among Chinese Children With Anti-methyl-D-aspartate Receptor (Anti-NMDAR) Encephalitis
Objective: Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is the most common form of autoimmune encephalitis in pediatric patients. In this study, we aimed to investigate the clinical features and long-term outcomes of pediatric patients with anti-NMDAR encephalitis in China.Methods: W...
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| Format: | Article |
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Frontiers Media S.A.
2019-06-01
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| Series: | Frontiers in Neurology |
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| Online Access: | https://www.frontiersin.org/article/10.3389/fneur.2019.00596/full |
| _version_ | 1819069838945943552 |
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| author | Min Zhang Wenhui Li Shuizhen Zhou Yuanfeng Zhou Haowei Yang Lifei Yu Ji Wang Yi Wang Linmei Zhang |
| author_facet | Min Zhang Wenhui Li Shuizhen Zhou Yuanfeng Zhou Haowei Yang Lifei Yu Ji Wang Yi Wang Linmei Zhang |
| author_sort | Min Zhang |
| collection | DOAJ |
| description | Objective: Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is the most common form of autoimmune encephalitis in pediatric patients. In this study, we aimed to investigate the clinical features and long-term outcomes of pediatric patients with anti-NMDAR encephalitis in China.Methods: We conducted a retrospective study of children (age range: 0–18 years) with anti-NMDAR encephalitis treated at Children's Hospital of Fudan University between July 2015 and November 2018. Demographic characteristics, clinical features, treatment, and outcomes were reviewed.Results: Thirty-four patients with anti-NMDAR encephalitis were enrolled (age range: 5 months to 14 years; median age: 7 years; female: 18). The median follow- up duration was 20 months (range: 6–39 months). Eighteen (52.9%) patients initially presented with seizures and 10 (29.4%) with abnormal (psychiatric) behaviors or cognitive dysfunction. Thirty (88.2%) patients exhibited more than two symptoms during the disease course. No neoplasms were detected. Twelve (35.2%) patients had abnormal cerebrospinal fluid (CSF) findings, including leukocytosis, and increased protein concentration. Eighteen (52.9%) patients exhibited normal brain MRI findings. Electroencephalography revealed abnormal background activity in 27 (79.4%) patients, and epileptiform discharges in 16 (47.0%) patients prior to immunotherapy. All patients received first-line immunotherapy, with 30 (88.2%) and four (11.8%) patients achieving good (Modified Rankin Scale [mRS] score of 0–2) and poor outcomes (mRS score of 3–5), respectively. Initial mRS scores differed significantly between the good and poor outcome groups. Fourteen out of 18 patients (77.7%) with seizures accepted anti-epileptic drug (AED) administration, and seizure freedom was achieved in 12 out of 14 (85.7%) patients at the last follow-up. Ten of these 12 (83.3%) patients withdrew from AED treatment within 1 year.Conclusions: Most patients achieved seizure freedom, so long-term use of AEDs may not be necessary for pediatric patients with anti-NMDAR encephalitis. Among our patients, 83.3% were sensitive to first-line immunotherapy and achieved good outcomes. Higher mRS scores before immunotherapy predicted poor outcomes, highlighting the need for a comprehensive assessment of patients with anti-NMDAR encephalitis. |
| first_indexed | 2024-12-21T16:56:25Z |
| format | Article |
| id | doaj.art-dc3651dc41234b34adf0992a3cbc26e3 |
| institution | Directory Open Access Journal |
| issn | 1664-2295 |
| language | English |
| last_indexed | 2024-12-21T16:56:25Z |
| publishDate | 2019-06-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Neurology |
| spelling | doaj.art-dc3651dc41234b34adf0992a3cbc26e32022-12-21T18:56:44ZengFrontiers Media S.A.Frontiers in Neurology1664-22952019-06-011010.3389/fneur.2019.00596443854Clinical Features, Treatment, and Outcomes Among Chinese Children With Anti-methyl-D-aspartate Receptor (Anti-NMDAR) EncephalitisMin Zhang0Wenhui Li1Shuizhen Zhou2Yuanfeng Zhou3Haowei Yang4Lifei Yu5Ji Wang6Yi Wang7Linmei Zhang8Department of Neurology, Children's Hospital of Fudan University, Shanghai, ChinaDepartment of Neurology, Children's Hospital of Fudan University, Shanghai, ChinaDepartment of Neurology, Children's Hospital of Fudan University, Shanghai, ChinaDepartment of Neurology, Children's Hospital of Fudan University, Shanghai, ChinaDepartment of Radiology, Children's Hospital of Fudan University, Shanghai, ChinaDepartment of Neurology, Children's Hospital of Fudan University, Shanghai, ChinaDepartment of Neurology, Children's Hospital of Fudan University, Shanghai, ChinaDepartment of Neurology, Children's Hospital of Fudan University, Shanghai, ChinaDepartment of Neurology, Children's Hospital of Fudan University, Shanghai, ChinaObjective: Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is the most common form of autoimmune encephalitis in pediatric patients. In this study, we aimed to investigate the clinical features and long-term outcomes of pediatric patients with anti-NMDAR encephalitis in China.Methods: We conducted a retrospective study of children (age range: 0–18 years) with anti-NMDAR encephalitis treated at Children's Hospital of Fudan University between July 2015 and November 2018. Demographic characteristics, clinical features, treatment, and outcomes were reviewed.Results: Thirty-four patients with anti-NMDAR encephalitis were enrolled (age range: 5 months to 14 years; median age: 7 years; female: 18). The median follow- up duration was 20 months (range: 6–39 months). Eighteen (52.9%) patients initially presented with seizures and 10 (29.4%) with abnormal (psychiatric) behaviors or cognitive dysfunction. Thirty (88.2%) patients exhibited more than two symptoms during the disease course. No neoplasms were detected. Twelve (35.2%) patients had abnormal cerebrospinal fluid (CSF) findings, including leukocytosis, and increased protein concentration. Eighteen (52.9%) patients exhibited normal brain MRI findings. Electroencephalography revealed abnormal background activity in 27 (79.4%) patients, and epileptiform discharges in 16 (47.0%) patients prior to immunotherapy. All patients received first-line immunotherapy, with 30 (88.2%) and four (11.8%) patients achieving good (Modified Rankin Scale [mRS] score of 0–2) and poor outcomes (mRS score of 3–5), respectively. Initial mRS scores differed significantly between the good and poor outcome groups. Fourteen out of 18 patients (77.7%) with seizures accepted anti-epileptic drug (AED) administration, and seizure freedom was achieved in 12 out of 14 (85.7%) patients at the last follow-up. Ten of these 12 (83.3%) patients withdrew from AED treatment within 1 year.Conclusions: Most patients achieved seizure freedom, so long-term use of AEDs may not be necessary for pediatric patients with anti-NMDAR encephalitis. Among our patients, 83.3% were sensitive to first-line immunotherapy and achieved good outcomes. Higher mRS scores before immunotherapy predicted poor outcomes, highlighting the need for a comprehensive assessment of patients with anti-NMDAR encephalitis.https://www.frontiersin.org/article/10.3389/fneur.2019.00596/fullanti-NMDAR encephalitisautoimmune encephalitisanti-N-methyl-D-aspartate receptorchildrenimmunotherapy |
| spellingShingle | Min Zhang Wenhui Li Shuizhen Zhou Yuanfeng Zhou Haowei Yang Lifei Yu Ji Wang Yi Wang Linmei Zhang Clinical Features, Treatment, and Outcomes Among Chinese Children With Anti-methyl-D-aspartate Receptor (Anti-NMDAR) Encephalitis Frontiers in Neurology anti-NMDAR encephalitis autoimmune encephalitis anti-N-methyl-D-aspartate receptor children immunotherapy |
| title | Clinical Features, Treatment, and Outcomes Among Chinese Children With Anti-methyl-D-aspartate Receptor (Anti-NMDAR) Encephalitis |
| title_full | Clinical Features, Treatment, and Outcomes Among Chinese Children With Anti-methyl-D-aspartate Receptor (Anti-NMDAR) Encephalitis |
| title_fullStr | Clinical Features, Treatment, and Outcomes Among Chinese Children With Anti-methyl-D-aspartate Receptor (Anti-NMDAR) Encephalitis |
| title_full_unstemmed | Clinical Features, Treatment, and Outcomes Among Chinese Children With Anti-methyl-D-aspartate Receptor (Anti-NMDAR) Encephalitis |
| title_short | Clinical Features, Treatment, and Outcomes Among Chinese Children With Anti-methyl-D-aspartate Receptor (Anti-NMDAR) Encephalitis |
| title_sort | clinical features treatment and outcomes among chinese children with anti methyl d aspartate receptor anti nmdar encephalitis |
| topic | anti-NMDAR encephalitis autoimmune encephalitis anti-N-methyl-D-aspartate receptor children immunotherapy |
| url | https://www.frontiersin.org/article/10.3389/fneur.2019.00596/full |
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