Amplitude-integrated electroencephalography in newborns with inborn errors of metabolism.
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.
Department of Neonatology, University Children's Hospital Vienna, Vienna, Austria.
Monika
M
Olischar
Eilon
E
Shany
Canan
C
Aygün
Denis
D
Azzopardi
Rod W
RW
Hunt
Mona C
MC
Toet
Ada
A
Hamosh
Linda S
LS
de Vries
Lena
L
Hellström-Westas
Christiane
C
Theda
D000465:Algorithms; D001921:Brain / Q000502:physiology; D004569:Electroencephalography / Q000379:methods; D006801:Humans; D020925:Hypoxia-Ischemia, Brain / Q000175:diagnosis* / Q000453:epidemiology / Q000209:etiology*; D007231:Infant, Newborn; D008661:Metabolism, Inborn Errors / Q000150:complications* / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology; D008991:Monitoring, Physiologic / Q000379:methods; D011379:Prognosis; D012042:Registries / Q000706:statistics & numerical data; D012640:Seizures / Q000175:diagnosis / Q000209:etiology; D012890:Sleep / Q000502:physiology