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Amplitude-Integrated Electroencephalography in Newborns with Inborn Errors of Metabolism
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2012 (English)In: Neonatology, ISSN 1661-7800, Vol. 102, no 3, 203-211 p.Article in journal (Refereed) Published
Abstract [en]

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.

Place, publisher, year, edition, pages
2012. Vol. 102, no 3, 203-211 p.
Keyword [en]
Electroencephalography, Amplitude integrated electroencephalography, Inborn errors of metabolism, Newborn, Metabolic disorders, Neonate, Cerebral function monitoring
National Category
Medical and Health Sciences
URN: urn:nbn:se:uu:diva-182044DOI: 10.1159/000339567ISI: 000307877600010OAI: oai:DiVA.org:uu-182044DiVA: diva2:558880
Available from: 2012-10-05 Created: 2012-10-02 Last updated: 2012-10-05Bibliographically approved

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Hellström-Westas, Lena
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