Amplitude-Integrated Electroencephalography in Newborns with Inborn Errors of Metabolism

Abstract

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.

The investigation of inborn errors of metabolism as an underlying cause of idiopathic intellectual disability in adults in Norway

“…Two articles were found regarding electroencephalography (EEG) and IEMs, but they focused on children . Information found in the article regarding IEMs and epilepsy contained information regarding EEGs in adults .…”

Guiding Antiepileptic Therapy in a Pediatric Patient with Severe Meningoencephalitis and Decompressive Craniectomy with the Use of Amplitude-Integrated Electroencephalography

“…Indications and usefulness in neonatology include hypoxic-ischemic encephalopathy, 3 seizure detection and monitoring, 4 posthemorrhagic ventricular dilation, 6 and other forms of increased intracranial pressure, as well as encephalopathy in inborn errors of metabolism. 7 It also may be used in prediction of neurologic outcome in very small preterm infants. 8 So far, the use of aEEG in children is mainly reported in case reports.…”

“…2 The main indications for monitoring with an aEEG in neonatology are asphyxia 3 and seizure detection. 4 Numerous other possibilities of use of aEEG have been published in neonatal patients, such as monitoring brain activity after intracranial hemorrhage, 5 posthemorrhagic ventricular dilation, 6 metabolic encephalopathy, 7 and predicting outcome in preterm infants. 8 Despite the very successful and promising indications in neonatology, aEEG has been rarely used in the pediatric intensive care or adult setting.…”

“…Mainly brief reports and case reports have been published thus far. 9 Neuromonitoring in the pediatric intensive care unit may be beneficial in cardiac surgery, 10 neurocritical care, 11 severe metabolic conditions, 7 and various forms of epilepsy that are difficult to treat. 12 We describe a case of a teenage patient with severe neurologic pathology, where we continuously monitored seizure and electrical brain activity.…”

Bispectral Index and Lower Margin Amplitude of the Amplitude-Integrated Electroencephalogram in Neonates

“…The amplitude-integrated electroencephalogram (aEEG) has become the standard cerebral function monitoring in neonatal intensive care and proved to be useful mainly for the classification of hypoxic-ischemic encephalopathy and seizure detection, but also for neuromonitoring of preterm infants [1,2,3,4,5,6,7]. Several aEEG reports indicate that opiates and benzodiazepines, routinely used drugs in the neonatal intensive care setting, can suppress the lower margin amplitude (LMA) of the aEEG, a parameter that describes the EEG discontinuity [8,9,10,11].…”