Evaluation of multiple consensus criteria for autoimmune encephalitis and temporal analysis of symptoms in a

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Tiffany Pointon1, Ryan Ward2, Anusha Yeshokumar3

  • 1Section of Child Neurology, Department of Pediatrics, University of Colorado School of Medicine, Aurora, CO, United States.

Frontiers in Neurology
|October 14, 2022
PubMed

Insights

Diagnosing pediatric autoimmune encephalitis (AE) is challenging. Current criteria show high sensitivity by day 28, but specificity improves significantly with infectious testing, aiding early detection.

Area of Science:

  • Neurology
  • Immunology
  • Pediatrics

Background:

  • Autoimmune encephalitis (AE) diagnosis is complex due to symptom overlap with infectious encephalitis (IE).
  • Established criteria like Graus and Cellucci, and the Determining Etiology in Encephalitis (DEE) score, aim to aid diagnosis.
  • Early and accurate diagnosis of AE is critical for improved patient outcomes.

Purpose of the Study:

  • To assess the sensitivity and specificity of current diagnostic criteria for pediatric autoimmune encephalitis (AE).
  • To evaluate the temporal onset of neuropsychiatric symptoms in pediatric encephalitis patients.
  • To compare the diagnostic performance of AE criteria against infectious encephalitis (IE).

Main Methods:

  • Retrospective review of pediatric patients diagnosed with AE or IE between 2000-2021.
  • Analysis included etiology-confirmed IE and antibody-positive/negative AE cases.
  • Sensitivity and specificity of diagnostic criteria were calculated within the first month post-symptom onset.

Main Results:

  • By day 28, pediatric AE sensitivity approached 90% for Cellucci and Graus criteria.
  • Specificity improved to ~90% with rapid PCR or infectious antibody testing.
  • The DEE score showed 100% specificity for IE but low sensitivity (29%); IE symptoms clustered, while AE symptoms were more spread out.

Conclusions:

  • This study first evaluates Cellucci criteria and validates the DEE score for differentiating pediatric AE and IE.
  • Current AE criteria demonstrate good sensitivity but require refinement for improved early detection.
  • Further improvements in diagnostic criteria are essential for timely AE treatment in children.
Abstract

Keywords:
autoimmune encephalitisdiagnostic criteriainfectious encephalitisneuroimmune diseasepediatric neurologytemporal analysis and evaluation

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