Document Detail

Amplitude-Integrated Electroencephalography in Newborns with Inborn Errors of Metabolism.
MedLine Citation:
PMID:  22797054     Owner:  NLM     Status:  Publisher    
Background: The utility of amplitude-integrated electroencephalography (aEEG) monitoring has been established for patients with neonatal hypoxic-ischemic encephalopathy. Objective: To evaluate the role of aEEG in the diagnostic process and treatment of patients with encephalopathy due to inborn errors of metabolism. Methods: Cases collected through an international registry were divided into 5 groups of metabolic disorders. Common aEEG features were sought for each group. Results: In total, 21/30 (70%) cases had abnormal aEEG background patterns, 18/30 (60%) showed seizure activity. Patients with disorders of energy metabolism, hyperammonemia, and organic/amino acidopathies often showed marked aEEG depression with seizure activity. In contrast, aEEGs of patients with peroxisomal disorders did not show major background abnormalities but seizures were present in 5/6 subjects. We report two features of interest: firstly, two tracings displayed an unusual upward shift of the lower aEEG amplitude margin. Secondly, aEEGs of infants with non-ketotic hyperglycinemia showed a pattern we refer to as 'high-frequency burst-suppression pattern'. Conclusions: aEEG in patients with inborn errors of metabolism frequently reveals abnormalities and assists clinicians in the clinical assessment, management and monitoring of these patients.
Monika Olischar; Eilon Shany; Canan Aygün; Denis Azzopardi; Rod W Hunt; Mona C Toet; Ada Hamosh; Linda S de Vries; Lena Hellström-Westas; Christiane Theda
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-7-12
Journal Detail:
Title:  Neonatology     Volume:  102     ISSN:  1661-7819     ISO Abbreviation:  -     Publication Date:  2012 Jul 
Date Detail:
Created Date:  2012-7-16     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101286577     Medline TA:  Neonatology     Country:  -    
Other Details:
Languages:  ENG     Pagination:  203-211     Citation Subset:  -    
Copyright Information:
Copyright © 2012 S. Karger AG, Basel.
Department of Neonatology, University Children's Hospital Vienna, Vienna, Austria.
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