The Diagnostic Challenge and Prognosis of Autoimmune Encephalitis in Children: A Single-Center Retrospective Study

Pediatr Neurol. 2023 Sep:146:103-109. doi: 10.1016/j.pediatrneurol.2023.06.014. Epub 2023 Jun 23.

Abstract

Background: Autoimmune encephalitis (AE) is an immune-mediated encephalitis; nevertheless, its diagnosis in children remains challenging. This study aimed to reveal the clinical characteristics, diagnostic processes, and therapeutic outcomes of AE in children.

Methods: A total of 18 children with AE were enrolled. Antibody assay was performed in the cerebrospinal fluid (CSF) and serum samples by indirect immunofluorescence. Electroencephalography (EEG) and magnetic resonance imaging (MRI) were monitored to reflect abnormal neural signals. In addition, demographics data, neurological symptoms, therapeutic strategies, and outcomes were recorded and analyzed.

Results: Convulsion (50.00%) and emotional disturbance (44.44%) were common clinical symptoms of AE. The biochemical parameters in the CSF had a relatively low diagnostic value. Antibodies in the CSF were dominant in the diagnosis of AE but those in the serum were limited in the diagnosis of anti-MOG or anti-LGI1 AE. In addition, all children showed abnormalities in EEG (72.22%) or MRI (66.67%). Methylprednisolone combined with sequential oral prednisone (83.33%) and gamma globulin (88.89%) were the dominant drugs, achieving an overall recovery rate of 72.22%. However, there were still two patients who had poor outcomes, including Patient 3 with a young age (two years old) and progressive symptom and Patient 12 with a long disease course before treatment (>120 days).

Conclusions: The clinical manifestations of AE are varied in children. Antibody in the CSF was dominant, and EEG and MRI were instructive in the diagnosis of AE. Young age, progressive symptom, and prolonged disease course before treatment may contribute to poor outcomes.

Keywords: Antibody; Autoimmune encephalitis; Convulsion; Electroencephalography; Magnetic resonance imaging.

MeSH terms

  • Antibodies
  • Autoimmune Diseases of the Nervous System* / diagnosis
  • Child
  • Child, Preschool
  • Encephalitis* / pathology
  • Humans
  • Prognosis
  • Retrospective Studies

Substances

  • Antibodies

Supplementary concepts

  • Hashimoto's encephalitis