Abstract
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 treatment are presented and updated.
Highlights
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)
Electroclinical phenotype is similar in spite of the different etiologies because of a common underlying mechanism [83]; functional neuroimaging has indicated that epileptic activity in LGS recruits widespread areas of association cortex and that tonic seizures are expressed through the reticular formation of the pons [84]
Landau-Kleffner syndrome (LKS) is a type of electrical status epilepticus during slow sleep (ESES) syndrome which manifests with an acquired epileptic aphasia that occurs in the child with already developed ageappropriate speech
Summary
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). The idea that seizures and apparently subclinical epileptic activity could affect cognitive functions was gaining strength in the scientific community related to epilepsy. In 2012, Capovilla proposed the term epileptogenic encephalopathy They refer to progressive disorders of various etiologies that can cause deterioration and epilepsy, such as brain tumors, neurodegenerative or metabolic diseases, and presumed inflammatory or autoimmune conditions [1]. Deterioration is independent of epilepsy, even if epilepsy could worsen the clinical picture; in some cases, the same etiology can produce encephalopathy without epilepsy. This distinction is important for the treatment; in the EE the treatment must be aggressive. Neonatal epileptic encephalopathies: early infantile epileptic encephalopathy with suppression-burst pattern (Ohtahara syndrome) and early myoclonic encephalopathy (EME)
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