Clinical Characteristics and Prognostic Factors of Children With Anti-N-Methyl-D-Aspartate Receptor Encephalitis

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.

A Systematic Review of Severe Neurological Manifestations in Pediatric Patients with Coexisting SARS-CoV-2 Infection

“…In a previous multi-institutional observational study, children accounted for 37% of the patients with this type of encephalitis [71]. The most common symptoms were dyskinesia, personality change, seizures, and cognitive disorders [72]. This type of encephalitis has also been reported in adults [73,74].…”

Encefalite autoimune antirreceptor NMDA na infância e causas virais: uma revisão bibliográfica

“…O quadro clínico da encefalite NMDAR por COVID-19 mais comum visto em crianças foi: discinesia, alteração de personalidade, convulsões e distúrbios cognitivos (Yang, et al, 2021). Titulaer, et al, (2013) relatou em seu estudo observacional multiinstitucional que os pacientes apresentaram ilusões, psicose, catatonia, discinesias orofaciais e coreatose.…”

Effectiveness of Intra-thecal Methotrexate in Refractory Anti-N-methyl-d-aspartate Receptor Encephalitis

“…10 International consensus recommendations for the treatment of pediatric NMDAR antibody encephalitis have been established to treat patients with autoimmune encephalitis. 11 This management protocol starts with rst-line therapy, which involves intravenous administration of methylprednisolone with a tapering plan of prednisolone. [1][2][3][4][5][6][7][8][9][10][11] Additional rst line therapy is therapeutic plasma exchange and/or intravenous immunoglobulin ("IVIG").…”

“…11 This management protocol starts with rst-line therapy, which involves intravenous administration of methylprednisolone with a tapering plan of prednisolone. [1][2][3][4][5][6][7][8][9][10][11] Additional rst line therapy is therapeutic plasma exchange and/or intravenous immunoglobulin ("IVIG"). Second line therapy consists of Rituximab or Cyclophosphamide.…”