title: A severity score for acute necrotizing encephalopathy.
abstract: OBJECTIVE
  To develop a score that predicts the prognosis of children with acute necrotizing encephalopathy (ANE).
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  ANE-SS can be used to predict outcome in patients with ANE. More effective treatments need to be developed for high-risk patients.
authors.affiliation: Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan. Electronic address: h-yamamoto@med.nagoya-u.ac.jp.
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Yamamoto
authors.affiliation: Department of Pediatrics, Juntendo University Faculty of Medicine, Tokyo, Japan.
authors.forename: Akihisa
authors.initials: A
authors.lastname: Okumura
authors.affiliation: Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Jun
authors.initials: J
authors.lastname: Natsume
authors.affiliation: Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan.
authors.forename: Seiji
authors.initials: S
authors.lastname: Kojima
authors.affiliation: Department of Developmental Medical Sciences, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
authors.forename: Masashi
authors.initials: M
authors.lastname: Mizuguchi
mesh_terms: D002675:Child, Preschool; D006801:Humans; D004684:Leukoencephalitis, Acute Hemorrhagic / Q000175:diagnosis*; D011379:Prognosis; D012189:Retrospective Studies; D012306:Risk; D012720:Severity of Illness Index
keywords: ANE; Acute necrotizing encephalopathy; Prognosis; Risk; Severity score