Overview of clinical and physiologic aspects of status epilepticus in the developmental and epileptic encephalopathies

Abstract

The Developmental and Epileptic Encephalopathies are a group of severe epilepsies which onset in early life and are characterized by recurrent, drug-resistant seizures and multiple comorbidities including intellectual disability, behavior and sleep problems, gait concerns, amongst others. Both convulsive and nonconvulsive status epilepticus are frequently seen, however the exact risk and type of status epilepticus depends on the underlying syndrome or etiology. The baseline degree of developmental impairment and EEG features characterized by background slowing and persistent, and at times, near-continuous high-amplitude epileptiform discharges can make a clearcut diagnosis of nonconvulsive status epilepticus, in a timely manner, very challenging. Furthermore, there is limited guidance on best management of nonconvulsive status epilepticus, and success of therapy is often suboptimal.