Developmental and epileptic encephalopathies: the clinical approach to investigation and management

Epileptic encephalopathies (EE) and developmental and epileptic encephalopathies (DEE) are a group of severe brain disorders in which epileptic electrical discharge contributes to progressive psychomotor dysfunction. These include rare but important conditions such as neonatal epileptic encephalopathy, West, Dravet, Lennox Gastaut and Landau Kleffner syndromes. These conditions result in a characteristic cluster of clinical, EEG and aetiological features which together, help us understand the condition, use more targeted therapies and prognosticate with regards to progression with age. Electroencephalography (EEG) is crucial in determining the causative epilepsy syndrome. Since the availability of next generation sequencing, genetic testing has assumed increasing importance in the diagnosis of specific syndromes. Multidisciplinary support from medical, nursing and therapy teams is essential in helping these children reach the best of their abilities, skills and to optimize their long-term outcomes. In this article we describe some of the more commonly seen EE/DEE and discuss management and prognosis of these.