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Development and Validation of a Comprehensive Acute Encephalitis Syndrome Severity Score in Children with Acute

Indar K Sharawat1,2, Prateek K Panda1,2, Vyas K Rathaur3,2

  • 1Department of Pediatrics, Pediatric Neurology Division, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India.

Indian Journal of Critical Care Medicine : Peer-Reviewed, Official Publication of Indian Society of Critical Care Medicine
|May 18, 2026
PubMed

Insights

A new scoring system, the Comprehensive Acute Encephalitis Syndrome Severity Score (CAESS), accurately predicts outcomes in children with acute encephalitis syndrome. This validated tool aids in assessing neurological function and guiding treatment for pediatric AES patients.

Area of Science:

  • Pediatric Neurology
  • Infectious Diseases
  • Clinical Scoring Systems

Background:

  • Acute encephalitis syndrome (AES) poses a significant threat to children, with a lack of reliable tools to predict neurological outcomes.
  • Existing scoring systems do not comprehensively assess the severity and potential long-term functional impact of AES in pediatric populations.

Purpose of the Study:

  • To develop and validate a novel scoring system, the Comprehensive Acute Encephalitis Syndrome Severity Score (CAESS), for predicting functional neurological outcomes in children with AES.
  • To establish the reliability, validity, and predictive accuracy of the CAESS score in a cohort of pediatric AES patients.

Main Methods:

  • The CAESS score was developed using five key indicators: Glasgow Coma Scale (GCS), focal deficit, status epilepticus, raised intracranial pressure (ICP) features, and the Pediatric Logistic Organ Dysfunction-2 (PELOD-2) score.
  • Content validity was assessed using the Content Validity Index (CVI) and Content Validity Ratio (CVR); model fit was evaluated via Confirmatory Factor Analysis (CFA).
  • Functional outcomes were assessed at 6 months using the Pediatric Cerebral Performance Category (PCPC) score, with construct validity confirmed through correlations with FSS, IQ, and GMFM-88.

Main Results:

  • The CAESS score demonstrated good to excellent inter-rater reliability (ICC: 0.82-0.94, kappa: 0.80-0.92) and good internal consistency (Cronbach's alpha = 0.80).
  • The score showed strong discriminant validity (AUC = 0.91) and, at a cut-off of ≥6, achieved 88.9% sensitivity and 84.2% specificity for predicting poor outcomes.
  • CAESS scores correlated strongly with functional status scales, including FSS (r=0.82), PCPC (r=0.84), GMFM-88 (r=-0.83), IQ (r=-0.71), and PedsQL (r=-0.74).

Conclusions:

  • The CAESS score is a potentially valid and reliable tool for predicting functional outcomes in children diagnosed with acute encephalitis syndrome.
  • Further prospective studies with robust internal and external validation are recommended before routine clinical implementation of the CAESS score.
Abstract

Keywords:
Acute encephalitis syndromeChildrenFunctional status scale scoreNeurocritical careNeurological outcomePediatric cerebral performance category score

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