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Published on: July 4, 2007
Development and validation of the Pediatric Autoimmune encephalitis Severity Scale in children with autoimmune
Priyanka Madaan1, Suvasini Sharma2, Ava Easton3
1Pediatric Neurology Unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Insights
The Pediatric Autoimmune encephalitis Severity Scale (PASS) was developed and validated for children with autoimmune encephalitis. This new scale offers a reliable tool for assessing disease severity in pediatric patients.
Area of Science:
- Pediatric Neurology
- Autoimmune Diseases
- Clinical Assessment Tools
Background:
- Autoimmune encephalitis (AE) in children requires accurate severity assessment.
- Existing adult scales may not be suitable for pediatric populations.
- Development of a validated pediatric-specific tool is needed.
Purpose of the Study:
- To develop the Pediatric Autoimmune encephalitis Severity Scale (PASS) using expert consensus and the Delphi process.
- To validate the PASS in children diagnosed with autoimmune encephalitis.
- To establish PASS as a reliable severity assessment tool for pediatric AE.
Main Methods:
- Prospective observational study involving 27 children with AE.
- Serial rating using PASS, CASE, and modified Rankin Scale over eight time points up to 6 months.
- Assessment of clinical outcomes including PedsQL and developmental/intellectual outcomes.
Main Results:
- Excellent interrater agreement for PASS (ICC=0.984) and CASE (ICC=0.983).
- The worst PASS score showed an inverse correlation with the final PedsQL score (r=-0.42, p=0.047).
- Factor analysis identified distinct 'cognitive and behavioural' and 'disinhibition and excitability' dimensions within PASS.
Conclusions:
- The Pediatric Autoimmune encephalitis Severity Scale (PASS) is a validated tool for assessing AE severity in children.
- PASS provides a suitable alternative to adult-derived scales for pediatric populations.
- This scale aids in the objective evaluation of disease severity and its impact on quality of life.
Aim:
To develop the Pediatric Autoimmune encephalitis Severity Scale (PASS) using expert consensus and the Delphi process, and validate it in children with autoimmune encephalitis.
Method:
This prospective observational study enrolled children who underwent serial rating with PASS, the Clinical Assessment Scale in Autoimmune Encephalitis (CASE), and the modified Rankin Scale, at eight time points until 6 months of presentation. Clinical outcomes included the Pediatric Quality of Life Inventory (PedsQL) score (parent-rated) and developmental and intellectual outcome at 6 months. Interrater agreement, internal consistency, and correlations of PASS and CASE with clinical outcomes were compared.
Results:
There were 361 assessments in 27 children (11 females, 16 males) with autoimmune encephalitis (15 with antibody-negative autoimmune encephalitis, 11 with N-methyl-D-aspartate receptor antibody encephalitis, and one with steroid-responsive encephalopathy associated with autoimmune thyroiditis) with a median age at onset of 4 years (interquartile range = 3 years to 6 years 5 months). There was excellent interrater agreement for PASS and CASE with comparable intraclass correlation coefficients (0.984 vs 0.983). The worst PASS score was inversely correlated with the final PedsQL score (r = -0.42, p = 0.047). Factor analysis revealed distinct 'cognitive and behavioural' and 'disinhibition and excitability' dimensions in the PASS items.
Interpretation:
PASS is a severity assessment tool for autoimmune encephalitis in children and offers an alternative to adult scales, which may be difficult to use in children.
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01:19Encephalitis l: Introduction
01:26Encephalitis ll: Pathophysiology