Abstract
Objective: Anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis is the most common autoimmune encephalitis in pediatric patients. The study aimed to investigate the clinical characteristics and prognostic factors of anti-NMDA receptor encephalitis in children in South China.Methods: This was a retrospective study of children diagnosed with anti-NMDA receptor encephalitis between 01/2014 and 12/2017 at Hunan Children's Hospital. Laboratory, brain magnetic resonance imaging (MRI), and electroencephalography data were collected. The short-term (6-month) outcomes were assessed using the Liverpool score by the same pediatric neurologist. The children were divided into good (scores 4–5) and poor (score <3) clinical outcomes.Results: Among the 51 patients, 21 (41.2%) were male. The most common clinical symptoms were dyskinesia (88.2%), personality change (84.3%), seizure (82.4%), and cognitive disorder (31.4%). Two were transferred to another hospital, 45 (91.8%) received intravenous immunoglobulins, 41 (83.7%) received methylprednisolone, and 8 (16.3%) received plasma exchange. Eight (16.3%) received rituximab for second-line treatment, six after intravenous immunoglobulin and methylprednisolone treatment, and two after plasma exchange therapy failed. Seven were lost to follow-up. The short-term outcome was good in 23 patients. Cognitive disorder [odds ratio (OR): 23.97, 95% confidence interval (CI): 1.12–513.30, P = 0.042) and abnormal brain MRI (OR: 14.29, 95% CI: 1.36–150.10, P = 0.027] were independently associated with a poor short-term outcome after adjustment for age, GCS, and rituximab use.Conclusions: MRI abnormalities and cognitive disorders are independently associated with poor short-term outcomes in children with anti-NMDA receptor encephalitis. The use of rituximab is not associated with the 6-month outcomes.
Highlights
N-methyl-D-aspartate (NMDA)-receptor encephalitis is an acute form of encephalitis caused by an autoimmune reaction to the GluN1 subunit of the NMDA neuronal receptor [1, 2]
This study aimed to describe the clinical characteristics of children with anti-NMDA receptor encephalitis in South China and explore the prognostic factors associated with short-term outcomes
Brain magnetic resonance imaging (MRI) showed brain parenchymal damage in 27 patients (52.9%); among them, the abnormalities were observed in the whole brain (n = 1), temporal lobe (n = 3), temporal lobe and other parts (n = 12), frontal lobe (n = 4), frontal lobe, and other parts (n = 5), and atypical pathological changes (n = 2)
Summary
N-methyl-D-aspartate (NMDA)-receptor encephalitis is an acute form of encephalitis caused by an autoimmune reaction to the GluN1 subunit of the NMDA neuronal receptor [1, 2]. It usually affects women of reproductive age but can occur in men and patients of all ages [2, 3]. Anti-NMDA receptor encephalitis is a leading cause of autoimmune encephalitis [7, 8]. Anti-NMDA receptor encephalitis in children is characterized mainly by seizures, dyskinesia, and localized neurological symptoms [5, 9]. The disease is fatal in 4% of the cases [3]
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