Quantifying Preclinical and Clinical EEG Biomarkers in Neurodevelopmental Disorders

Successful clinical trials for neurodevelopmental disorders will require reliable biomarkers that are quantitative, biologically based, and linked to clinically meaningful deficits. Ideally, such biomarkers will also have face validity in animal models. We will describe quantitative EEG biomarkers for Angelman syndrome (AS), a neurodevelopmental disorder caused by loss of neuronal expression of the maternally inherited UBE3A gene. Symptoms of AS include intellectual disability, impaired speech and motor function, epilepsy, and disrupted sleep. We will discuss and evaluate three recently described quantitative EEG biomarkers in AS: enhanced delta rhythms, abnormal coherence, and disrupted sleep spindles. We anticipate that the use of these biomarkers will have value both in preclinical testing of compounds and in the eventual design of clinical trials.