Pediatric Epilepsy

**77**

**Chapter 6**

**Abstract**

Epileptic Encephalopathies in

*Otman Fernandez-Concepcion and Melvin Lopez-Jimenez*

Epileptic encephalopathies represent a group of devastating epileptic disorders that appear early in life. They are characterized by pharmacoresistant generalized or focal seizures, persistent severe EEG abnormalities, and cognitive dysfunction or decline. The ictal and interictal epileptic discharges are age-specific and either are the main cause or contribute to cognitive deterioration in the idiopathic or symptomatic group, respectively. Despite choosing the most appropriate antiepileptic drugs for the seizure type and syndrome, the results are often disappointing, and polytherapy and/ or alternative therapy becomes unavoidable; in those cases, consideration should be given to the quality of life of the child and carers. In this chapter, we will discuss the clinical and electroencephalographic characteristics and evolution and management of age-related epileptic encephalopathies, recognized by the International League Against Epilepsy, as follows: early infantile epileptic encephalopathy (Ohtahara syndrome), early myoclonic encephalopathy, epilepsy of infancy with migrating focal seizures, infantile spasms (West syndrome), severe myoclonic epilepsy in infancy (Dravet syndrome), myoclonic-atonic epilepsy (Doose syndrome), Lennox-Gastaut syndrome, epileptic encephalopathy with continuous spike-and-wave during sleep, and Landau-Kleffner syndrome. Their clinical features, prognosis, etiologies, and

**Keywords:** electroclinical seizures, epileptic encephalopathy, pharmacoresistant

with the first description of the Landau-Kleffner syndrome (LKS) [2].

The concept of epileptic encephalopathy (EE) is based on the clinical descriptions of some epileptic syndromes during the last century, such as West syndrome (WS) and Lennox-Gastaut syndrome (LGS). Delay in development in one, and intellectual disability in the other, was considered partly due to interictal epileptic discharges [1]. The idea that not only seizures but also apparently subclinical epileptic activity could affect cognitive functions was gaining strength in the scientific community related to epilepsy. According to this, the control of this epileptic activity could improve these deficits [1]. This notion was better profiled in the description of neuropsychological deficits related to continuous or sub-continuous paroxysmal activity during sleep,

The definition of EE was better defined by Dulac in the 1990s and was incorporated into the proposed classification of the International League Against Epilepsy (ILAE) in 2001 [3]. In this proposal, the term EE was used for disorders in which "the epileptiform anomalies themselves are believed to contribute to the progressive disturbance of

Infants and Children

treatment are presented and updated.

**1. Introduction**

epilepsy, epilepsy, electroencephalography
