Abstract
Objective
To develop a score that predicts the prognosis of children with acute necrotizing
encephalopathy (ANE).
Method
We retrospectively evaluated clinical variables and neurological outcome in two cohorts
of children with ANE. Firstly, we developed the ANE severity score (ANE-SS) according
to the clinical variables that correlated with neurological outcome in 41 children
who were included in our previous reports in 2009. We then applied the scoring system
to a second cohort of 32 patients who were newly collected in 2011. We investigated
the correlation between the ANE-SS and neurological outcome in all 73 patients.
Results
In the first cohort, brain stem lesions on MRI and state of shock at onset were significantly
correlated with outcome. Age over 48 months, elevated CSF protein, and low platelet counts tended to be correlated with
outcome. No types of treatment were correlated with outcome. The developed ANE-SS
ranged from 0 to 9 points, with 3 points for existence of shock, 2 points for brain
stem lesions, 2 points for age over 48 months, 1 point for platelet count below 100,000/μL, and 1 point for CSF protein above
60 mg/dl. Patients were classed as low risk (ANE-SS 0–1 points), medium risk (ANE-SS
2–4 points), or high risk (ANE-SS 5–9 points). ANE-SS was significantly correlated
with outcome in the group of 73 patients.
Conclusion
ANE-SS can be used to predict outcome in patients with ANE. More effective treatments
need to be developed for high-risk patients.
Keywords
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Article info
Publication history
Published online: June 12, 2014
Accepted:
May 23,
2014
Received in revised form:
May 21,
2014
Received:
January 18,
2014
Identification
Copyright
© 2014 The Japanese Society of Child Neurology. Published by Elsevier Inc. All rights reserved.
