Amplitude-integrated electroencephalography in newborns with inborn errors of metabolism

Monika Olischar, Eilon Shany, Canan Aygn, Denis Azzopardi, Rod W. Hunt, Mona C. Toet, Ada Hamosh, Linda S. De Vries, Lena Hellstrm-Westas, Christiane Theda

Research output: Contribution to journalArticlepeer-review

16 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)203-211
Number of pages9
Issue number3
StatePublished - Sep 2012
Externally publishedYes


  • Amplitude integrated electroencephalography
  • Cerebral function monitoring
  • Electroencephalography
  • Inborn errors of metabolism
  • Metabolic disorders
  • Neonate
  • Newborn

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Developmental Biology


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