Title

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

Authors

Brian Appavu, Department of Neurosciences, Barrow Neurological Institute at 14524Phoenix Children's Hospital, University of Arizona College of Medicine-Phoenix, Phoenix, AZ, USA.
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.
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.
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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