title: Clinical characteristics, treatments, and outcomes of patients with anti-N-methyl-d-aspartate receptor encephalitis: A systematic review of reported cases.
abstract: Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is a recently recognized autoimmune disorder which is responsive to immunotherapy. However, the outcomes of different immunotherapies have not been defined and there have been few studies that carried out a comparison among them. To provide an overview of the clinical characteristics, treatments, and outcomes of anti-NMDAR encephalitis, we systematically reviewed the literature in the PubMed, Medline, Embase, Cochrane Library, BioMedical Literature Database (CBM), China National Knowledge Infrastructure (CNKI), and Wan-fang databases. Eighty-three studies with a total of 432 patients were included. The median age was 22years. Two hundred ninety-three (68%) patients were female, 87 (21%) of 412 patients had a tumor, including 68 (78%) patients with ovarian teratoma. Pediatric patients had a higher ratio of seizures to psychiatric symptoms as the initial manifestation (p=0.0012), a lower proportion with a tumor (p<0.0001) and CSF pleocytosis (p=0.0163), and a better outcome (p=0.0064) than adults. Patients who died had a higher proportion of CSF pleocytosis than the patients who survived (p=0.0021). There were no significant differences among three first-line immunotherapy used alone (p=0.9172) or among combinations of every two of them (p=0.3059). With regard to the use of corticosteroid and IVIG, there were no significant differences between the outcomes of early combined treatment and sequential treatment (p=0.7277), or between using corticosteroid first and IVIG first (p=0.5422). Our findings suggest that the clinical characteristics and outcomes for pediatric patients were different from adult patients, and no significant differences were found among different immunotherapies.
authors.affiliation: Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China.
authors.forename: Le
authors.initials: L
authors.lastname: Zhang
authors.affiliation: Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China.
authors.forename: Meng-Qian
authors.initials: MQ
authors.lastname: Wu
authors.affiliation: Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China.
authors.forename: Zi-Long
authors.initials: ZL
authors.lastname: Hao
authors.affiliation: West China College of Medicine, Sichuan University, Chengdu, Sichuan Province, China.
authors.forename: Siew Mun Vance
authors.initials: SM
authors.lastname: Chiang
authors.affiliation: West China College of Medicine, Sichuan University, Chengdu, Sichuan Province, China.
authors.forename: Kun
authors.initials: K
authors.lastname: Shuang
authors.affiliation: West China College of Medicine, Sichuan University, Chengdu, Sichuan Province, China.
authors.forename: Min-Tao
authors.initials: MT
authors.lastname: Lin
authors.affiliation: Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China.
authors.forename: Xiao-Sa
authors.initials: XS
authors.lastname: Chi
authors.affiliation: Department of Neurology, Fourth Affiliated Hospital, School of Medicine, Zhejiang University, Yiwu, Zhejiang Province, China.
authors.forename: Jia-Jia
authors.initials: JJ
authors.lastname: Fang
authors.affiliation: Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China. Electronic address: zhoudong66@yahoo.de.
authors.forename: Dong
authors.initials: D
authors.lastname: Zhou
authors.affiliation: Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan Province, China. Electronic address: jinmeili-neuro@qq.com.
authors.forename: Jin-Mei
authors.initials: JM
authors.lastname: Li
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D060426:Anti-N-Methyl-D-Aspartate Receptor Encephalitis / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D002681:China; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008297:Male; D016194:Receptors, N-Methyl-D-Aspartate / Q000276:immunology*; D012640:Seizures / Q000209:etiology*; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D016756:Immunoglobulins, Intravenous; D016194:Receptors, N-Methyl-D-Aspartate
keywords: Anti-N-methyl-d-aspartate receptor encephalitis; Clinical characteristics; Outcomes; Systematic review; Treatments