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Autoimmune Encephalitis in Children: Differential Characteristics and Treatment Responses in Definite and Probable

Rocio Victoria Garcia1, Andrea Savransky1, Gabriela Reyes Valenzuela1

  • 1Department of Neurology, Hospital de Pediatría Prof. Dr. Juan P. Garrahan, Buenos Aires, Argentina.

Pediatric Neurology
|August 5, 2026
PubMed

Insights

Pediatric autoimmune encephalitis (AE) differs by antibody status. Definite AE (antibody-positive) presents more severely than probable AE (antibody-negative), but early treatment and rituximab (RTX) improve outcomes and prevent relapses.

Area of Science:

  • Pediatric Neurology
  • Neuroimmunology
  • Autoimmune Diseases

Background:

  • Autoimmune encephalitis (AE) is a group of rare neurological disorders.
  • Distinguishing between antibody-negative (probable) and antibody-positive (definite) AE in children is crucial for understanding disease characteristics.
  • Limited research exists on the specific differences and outcomes for these pediatric AE subtypes.

Purpose of the Study:

  • To compare clinical and paraclinical features of probable versus definite AE in pediatric patients.
  • To evaluate treatment responses and identify factors influencing short-term outcomes in pediatric AE.
  • To assess the incidence of AE-associated epilepsy and MRI findings in both AE groups.

Main Methods:

  • Retrospective analysis of pediatric patients diagnosed with immune-mediated acute/subacute encephalopathy.
  • Comparison of clinical data, including modified Rankin Scale scores and immunotherapy use.
  • Evaluation of AE-associated epilepsy, brain MRI findings, and relapse rates.

Main Results:

  • Definite AE patients were younger (median 4.9 vs 7 years) and presented with more severe symptoms (64.6% vs 21.9% high mRS).
  • Second-line immunotherapy was more frequent in definite AE (35.4% vs 15.6%).
  • AE relapses occurred in 22.6% of antibody-positive patients not receiving rituximab (RTX), versus 0% in RTX-treated and antibody-negative groups.

Conclusions:

  • Children with definite AE exhibit more severe presentations, higher relapse rates, and greater need for second-line treatments compared to probable AE.
  • Early intervention and rituximab (RTX) are vital for favorable outcomes and relapse prevention in pediatric AE.
  • While AE-associated epilepsy was similar, brain MRI abnormalities were more common in definite AE.
Abstract

Keywords:
ChildrenEpilepsyImmunotherapyN-methyl-D-aspartate encephalitisSeizures

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