Abstract
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.
Highlights
Anti-N-methyl-D-aspartate receptorencephalitis is a recently recognized autoimmune disorder in which auto-antibodies mainly target the NMDAR1 subunit (NR1) subunit of the NMDA receptor, leading to a series of complex neuropsychiatric symptoms [1, 2]
Increased clinical recognition of this disease has led to an increase in the number of patients diagnosed with anti-NMDAR encephalitis
Each of the 18 patients who experienced seizures had onset during the acute phase of anti-NMDAR encephalitis, which was defined as
Summary
Encephalitis is a recently recognized autoimmune disorder in which auto-antibodies mainly target the NR1 subunit of the NMDA receptor, leading to a series of complex neuropsychiatric symptoms [1, 2]. Reports of anti-NMDAR encephalitis have become more frequent over recent years, shedding light on the clinical characteristics of the disease. Anti-NMDAR encephalitis is a form of autoimmune encephalitis. Patients typically present with psychiatric symptoms, behavioral dysfunction, seizures, speech impairment, cognitive impairment, movement disorders, decreased consciousness, autonomic instability, and central hypoventilation. The disease is observed in patients of different ages and genders and may or may not be accompanied by ovarian teratomas or other tumors. Increased clinical recognition of this disease has led to an increase in the number of patients diagnosed with anti-NMDAR encephalitis
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