Clinical Features, Treatment, and Outcomes Among Chinese Children With Anti-methyl-D-aspartate Receptor (Anti-NMDAR) Encephalitis

Zhang, Min; Li, Wenhui; Zhou, Shuizhen; Zhou, Yuanfeng; Yang, Haowei; Yu, Lingfeng; Ji, Wang; Wang, Yi; Zhang, Linmei

doi:10.3389/fneur.2019.00596

Cited by 33 publications

(37 citation statements)

References 45 publications

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“…In this present study, we described the clinical features, auxiliary examination features, treatment protocols and clinical outcomes of eight Chinese children treated with rituximab. All eight children had no detectable neoplasm supporting previous reports that pediatric patients are less likely to have associated tumors (1,(15)(16)(17)(18). Rituximab was administered as a second-line immunosuppressant after a median of 57 days of disease which was longer than other report at 0.1 months (2).…”

Section: Discussionsupporting

confidence: 74%

Efficacy and Safety of Rituximab in Chinese Children With Refractory Anti-NMDAR Encephalitis

Dou

Wang

et al. 2020

Front. Neurol.

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Purpose: To assess the efficacy and safety of rituximab treatment as second-line immunotherapy in pediatric cases of anti-NMDA receptor (NMDAR) encephalitis.Methods: We retrospectively recruited 8 patients with anti-NMDAR encephalitis who were treated with rituximab as second-line immunotherapy. We evaluated the clinical features, laboratory examination results and treatment protocols of the Chinese children and defined good outcomes based on the modified Rankin scale (mRS) score (0–2) at the last follow-up.Results: A total of eight pediatric patients (median age 6.7 years; four female) with refractory anti-NMDAR encephalitis were recruited to the study. Rituximab was given after a median duration of disease of 57 days (range 50.5–113.75 days). The use of rituximab led to a significant reduction in the mRS and CD19+ B-cells compared to before rituximab infusion (P < 0.05). Five patients (62.5%) had a good outcome (mRS ≤ 2) including four patients (50%) who showed complete recovery (mRS = 0) at the last follow-up. Transient infusion adverse events were recorded in 2 patients (25%). Two patients (25%) had severe infectious adverse events (AEs) and two patients with grade 5 (death). None of the patients developed progressive multifocal leukoencephalopathy (PML).Conclusion: Our study provides evidence that rituximab can efficiently improve the clinical symptoms of anti-NMDAR encephalitis in children. However, due to the risk of adverse infections, rituximab should be restricted in pediatric patients with high rates of mortality and disability.

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Section: Discussionsupporting

confidence: 74%

Efficacy and Safety of Rituximab in Chinese Children With Refractory Anti-NMDAR Encephalitis

Dou

Wang

et al. 2020

Front. Neurol.

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“…However, only 8.39% of the patients in our cohort had a tumor, with ovarian teratoma comprising 84.2% of the tumors diagnosed in females. Other studies of Chinese or Asian cohorts have also reported a relatively low prevalence of tumors ( Supplementary Table 4 ) [Lim et al ( 24 ), 22.7%; Wang et al ( 25 ), 8%; Liu et al ( 22 ), 6.7%; Zhang et al ( 26 ), 8.1%]. The heterogeneity among Asian and Western populations might be due to different genetic backgrounds and epidemiologic factors.…”

Section: Discussionmentioning

confidence: 86%

Neuronal Surface Antibody-Medicated Autoimmune Encephalitis (Limbic Encephalitis) in China: A Multiple-Center, Retrospective Study

2021

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Objective: The epidemiological characteristics of patients with antibody-medicated autoimmune encephalitis in China remain unclear, and a large-scale epidemiological survey is necessary.Methods: A multiple-center retrospective study was performed. We collected 1,047 patients with suspected autoimmune encephalitis and ultimately enrolled 778 defined patients across centers in China. All patients were positive for serum [or cerebrospinal fluid (CSF)] antibodies. Demographic information and clinical data from January 2014 to January 2019 from 22 centers in China were reviewed.Results: A total of 778 patients with autoimmune encephalitis were enrolled in the study. In general, the ratio of males to females was ~1.2:1. The main subtypes of autoimmune encephalitis were NMDAR-AE (61.35%), LGI-1-AE (20.61%), and GABAbR-AE (12.40%). According to the characteristics of age of onset, the incidence of autoimmune encephalitis showed a “double peak” distribution entailing a 20-year-old age group and a 60-year-old age group. We next analyzed the proportion of patients with tumors in this cohort. More specifically, there were 34 patients with tumors and 85 with tumor marker positivity. Relapse occurred in 81 patients within at least 1 year's follow up study: 52 with NMDAR-AE (18.2%); 19 with LGI-1-AE (16.8%); 5 with GABAbR-AE (9%); and 3 with CASPR2-AE.Interpretation: Due to the vast differences in demographic features, the incidence of cancer and the genetic characteristics between the populations in China and Western countries, the demographics, sex distribution, concomitant tumor rate, clinical features, and relapse characteristics associated with autoimmune encephalitis in China shows a similar profile with Western countries with some minor differences.

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“…Only one child in the ADEM with MOG-abs group was positive for anti-NMDAR antibodies in serum and CSF (1:320 in the serum and 1:3.2 in the CSF). However, the clinical presentation, cerebral MRI, clinical course, and prognosis were more in line with ADEM than with anti-NMDAR encephalitis ( 12 ). The median time from symptom onset to EEG recording was 25.5 (range: 4–57) days and 11.5 (range: 4–49) days in patients with MOG-abs and without MOG-abs, respectively.…”

Section: Resultsmentioning

confidence: 99%

“…Clinical outcomes were evaluated based on mRS before immunotherapy and during clinical visits to the neurologist or through a telephone interview after discharge. The evaluation standards were as follows: full recovery, mRS of 0; mild deficits, mRS of 1–2; severe deficits, mRS of 3–5; or death, mRS of 6 ( 12 ). We defined clinical improvement as an increase of 1 in mRS and clinical recovery as an mRS of 0–2.…”

Section: Methodsmentioning

confidence: 99%

Clinical and Neuroimaging Characteristics of Pediatric Acute Disseminating Encephalomyelitis With and Without Antibodies to Myelin Oligodendrocyte Glycoprotein

et al. 2020

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Objective: To compare the clinical and neuroimaging characteristics of anti-myelin oligodendrocyte glycoprotein antibody (MOG-ab) negative and positive pediatric acute disseminating encephalomyelitis (ADEM) patients.Methods: Clinical characteristics, neuroimaging features, ancillary examination results, and outcomes of pediatric ADEM patients were retrospectively reviewed between February 2016 and July 2019.Results: Among 37 pediatric ADEM patients, 24 patients (11 girls and 13 boys) fulfilled the inclusion criteria. The median age was 72 (range 19–156) months, and the median follow-up duration was 20 (range 12–48) months. Children with ADEM and MOG-abs presented with increased ataxia, reduced bladder/rectum dysfunction, and paralysis compared to children without MOG-abs. An important finding was that no significant differences existed in age at symptom onset, sex ratio, time from immunotherapy to clinical improvement and clinical recovery, or modified Rankin Scale (mRS) at the last follow-up. More typical cerebral MRI lesions were detected in patients with ADEM and MOG-abs than in children without MOG-abs [11/12 (91.7%) vs. 8/12 (66.7%)]. Cerebellar lesions were higher in ADEM patients with MOG-abs (7/12, 58.3%) than in those without MOG-abs (2/12, 16.7%). While seven children had abnormal spinal MRI findings (7/12, 58.3%) and five had longitudinally extensive transverse myelitis (LETM) (5/12, 41.7%) per group, the coexistence of spinal dysfunction and abnormal spinal MRI was lower in ADEM with MOG-abs (2/12, 16.7%) than in children without MOG-abs (7/12, 58.3%). Clinical improvement was achieved 1 week after immunotherapy. Most children in both groups achieved clinical recovery within 3 months after immunotherapy, although two (16.7%) patients with ADEM and MOG-abs had persistent neurological sequelae at the last follow-up.Conclusion: MOG-abs-positive ADEM is a major subtype of pediatric ADEM. Ataxia is the most common clinical presentation in pediatric ADEM and MOG-abs. Children with ADEM and MOG-abs have similar patterns of lesions characterized by large, bilateral, widespread lesions, as well as more cerebellar lesions than children without MOG-abs. Most spinal lesions were subclinical in pediatric ADEM with MOG-abs. A favorable prognosis can be achieved for pediatric ADEM regardless of the MOG-abs status. However, some patients with MOG-abs are likely to have more severe neurological sequelae.

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Clinical Features, Treatment, and Outcomes Among Chinese Children With Anti-methyl-D-aspartate Receptor (Anti-NMDAR) Encephalitis

Cited by 33 publications

References 45 publications

Efficacy and Safety of Rituximab in Chinese Children With Refractory Anti-NMDAR Encephalitis

Efficacy and Safety of Rituximab in Chinese Children With Refractory Anti-NMDAR Encephalitis

Neuronal Surface Antibody-Medicated Autoimmune Encephalitis (Limbic Encephalitis) in China: A Multiple-Center, Retrospective Study

Clinical and Neuroimaging Characteristics of Pediatric Acute Disseminating Encephalomyelitis With and Without Antibodies to Myelin Oligodendrocyte Glycoprotein

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