Treatment Timing, EEG, Neuroimaging, and Outcomes After Acute Necrotizing Encephalopathy in Children

Brian Appavu, Department of Neurosciences, Barrow Neurological Institute at 14524Phoenix Children's Hospital, University of Arizona College of Medicine-Phoenix, Phoenix, AZ, USA.Follow
Stephen Foldes, Department of Neurosciences, Barrow Neurological Institute at 14524Phoenix Children's Hospital, University of Arizona College of Medicine-Phoenix, Phoenix, AZ, USA.Follow
Jordana Fox, Department of Neurosciences, Barrow Neurological Institute at 14524Phoenix Children's Hospital, University of Arizona College of Medicine-Phoenix, Phoenix, AZ, USA.
Sheetal Shetty, Department of Neurosciences, Barrow Neurological Institute at 14524Phoenix Children's Hospital, University of Arizona College of Medicine-Phoenix, Phoenix, AZ, USA.
Ann Oh, Department of Neurosciences, Barrow Neurological Institute at 14524Phoenix Children's Hospital, University of Arizona College of Medicine-Phoenix, Phoenix, AZ, USA.
Freddy Bassal, Department of Neurosciences, Barrow Neurological Institute at 14524Phoenix Children's Hospital, University of Arizona College of Medicine-Phoenix, Phoenix, AZ, USA.Follow
Iris Marku, Department of Neurosciences, Barrow Neurological Institute at 14524Phoenix Children's Hospital, University of Arizona College of Medicine-Phoenix, Phoenix, AZ, USA.
Tara Mangum, Department of Neurosciences, Barrow Neurological Institute at 14524Phoenix Children's Hospital, University of Arizona College of Medicine-Phoenix, Phoenix, AZ, USA.
Varina Boerwinkle, Department of Neurosciences, Barrow Neurological Institute at 14524Phoenix Children's Hospital, University of Arizona College of Medicine-Phoenix, Phoenix, AZ, USA.Follow
Derek Neilson, Department of Genetics, Phoenix Children's Hospital, 42283University of Arizona College of Medicine-Phoenix, Phoenix, AZ, USA.
Michael Kruer, Department of Neurosciences, Barrow Neurological Institute at 14524Phoenix Children's Hospital, University of Arizona College of Medicine-Phoenix, Phoenix, AZ, USA.

Document Type

Article

Abstract

BACKGROUND: Acute necrotizing encephalopathy (ANE) is a rare condition associated with rapid progression to coma and high incidence of morbidity and mortality. METHODS: Clinical, electroencephalographic (EEG), and brain magnetic resonance imaging (MRI) characteristics and immunomodulatory therapy timing were retrospectively analyzed in children with ANE. ANE severity scores (ANE-SS) and MRI scores were also assessed. The associations of patient characteristics with 6-month modified Rankin scale (mRS) and length of hospitalization were determined using either univariate linear regression or one-way analysis of variance. RESULTS: 7 children were retrospectively evaluated. Normal EEG sleep spindles ( = .024) and early treatment ( = .57, = .030) were associated with improved outcomes (ie, decreased mRS). Higher ANE-SS ( = .79, = .011), higher age ( = .62, = .038), and presence of brainstem lesions ( = .015) were associated with longer length of hospitalization. Other patient characteristics were not significantly associated with mRS or length of hospitalization. CONCLUSION: Early immunomodulatory therapy and normal sleep spindles are associated with better functional outcome in children with ANE.

Keywords

EEG, MRI, genetics, neuroimmunology, seizures

Medical Subject Headings

Adolescent; Child; Child, Preschool; Electroencephalography; Female; Humans; Immunomodulation; Infant; Length of Stay; Leukoencephalitis, Acute Hemorrhagic (diagnosis, therapy); Magnetic Resonance Imaging; Male; Neuroimaging; Outcome Assessment, Health Care; Retrospective Studies; Thalamus (pathology); Time-to-Treatment

Publication Date

6-1-2021

Publication Title

Journal of child neurology

E-ISSN

1708-8283

Volume

36

Issue

7

First Page

517

Last Page

524

PubMed ID

33393838

Digital Object Identifier (DOI)

10.1177/0883073820984063

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