EEG Scoring in Degenerative Brain Diseases of Childhood: The Pediatric Neurodegeneration EEG Scoring (PNES)

An increasing number of pediatric degenerative brain diseases is approaching curative therapy by new drugs and procedures (e.g. stem cell transplantation, gene therapy). In recent years, the natural course of clinical and neuroimaging findings in some of the neurodegenerative diseases has been described, offering the opportunity to quantify or estimate the efficacy of the treatment. To define the course of EEG changes as an additional and important parameter of brain function and maturation, we developed a visual EEG rating system (PNES, Pediatric Neurodegeneration EEG Scoring). The PNES contains a total of seven items: dominant rhythm of background activity (BA), medium amplitude of BA, intermittent slowing of BA, anterior-posterior gradient of BA, reaction to eye opening of BA, frequency of epileptic activity and personal rating of the analyzer. Additionally, the description of 1. localization and type of epileptic discharges and the seizure type and 2. the response to photic stimulation can be carried out when appropriate. The scoring includes 2 steps: 1. scoring the EEG for each item and 2. calculation of the final score.

The PNES is a simple EEG scoring method for awake children beyond the age of 1 year that can be used in the routine clinical setting to define EEG abnormalities and also to evaluate therapeutic effects of standard (i.e. anticonvulsive) and experimental therapies.

No conflict of interest has been declared by the author(s).