Dravet Syndrome: A Technologist's Perspective

Abstract

ABSTRACT. Dravet Syndrome (DS), also known as Severe Myoclonic Epilepsy in Infancy (SMEI) is a rare, primarily genetic disorder which develops in infancy. The characteristics of DS are frequent, prolonged, primarily generalized seizures which occur initially with fever, and eventually evolve to multiple afebrile seizure types such as myoclonic, atypical absence, and complex partial seizures. Patients, who are initially developmentally normal, will experience concomitant developmental regression as the syndrome progresses. Because it is a childhood disorder, DS is not well known outside the realm of pediatrics. An astute EEG technologist should be able to recognize key factors both clinically and electrographically which point suspicion to the diagnosis of Dravet Syndrome.