Developmental encephalopathies and epileptic encephalopathies – diagnosis and options for modern treatment of Dravet syndrome and Lennox – Gastaut syndrome

Epileptic encephalopathies are defined as drug-resistant epilepsies in which seizures and/or EEG discharges impair neuropsychiatric development and cognitive functions. Developmental encephalopathies occur with delayed neuropsychiatric development or intellectual deficit, caused by non-progressive brain damage, combined or not with epilepsy, in which the neurocognitive and behavioral deficit remains, regardless of the control of epilepsy. A new concept for developmental encephalopathies and epileptic encephalopathies is that epilepsy and developmental delay are two conditions with a common etiology (structural or genetic causes) in which aggressive antiepileptic treatment is necessary. Epileptic encephalopathies are defined as a process of neurological damage caused by epileptic activity with variable potential for treatment. Epileptic activity affects neurogenesis, synaptogenesis, the normal organization of neuronal networks, triggers neuroinflammation, with possible pathophysiological mechanisms for neuronal damage. According to the age of onset, epileptic encephalopathies are: early infantile (Otahara syndrome, syndrome with epileptic infantile spasms or West syndrome, migratory focal seizures, Dravet syndrome) and late ( Lennox – G astaut s yndrome, e pileptic e ncephalopathies with sharp – slow wave activation in sleep, Landau Kleffner syndrome, atypical benign focal epilepsy of childhood, hemiconvulsion-hemiplegia syndrome, epileptic syndrome associated with febrile infections); With onset at different ages are epileptic syndromes with progressive neurological damage, Rasmussen syndrome, progressive myoclonic epilepsy, Febrile-infectious associated epileptic syndromes. In them, continuous epileptic activity or frequent epileptic seizures cause cognitive regression. Neurocognitive and behavioral abnormalities can improve with control of epileptic seizures and reduction of interictal epileptic activity. Timely diagnosis of epileptic encephalopathies with knowledge of their main clinical characteristics, especially Lennox – Gastaut syndrome and Dravet syndrome and their early treatment improves their prognosis. Therapeutic approaches for the treatment of epileptic encephalopathies are monotherapy and subsequent rational polytherapy including new anticonvulsant drugs (AEDs). Information is presented on the diagnosis, clinical characteristics and modern treatment of Lennox – Gastaut syndrome and Dravet syndrome.