Current terminology, diagnosis and modern treatment options

Authors

Keywords:

Epilepsies, terminology, epileptic/developmental encephalopathies, Lennox-Gastaut syndrome, Dravet syndrome, antiseizure medications

Abstract

Task Force on Nosology and Definitions proposes a classification and definition of epilepsy syndromes according to the age of onset from 2022, some terminologies are changed. Only Dravet syndrome, Lennox-Gastaut syndrome and Rasmussen syndrome remain with names of authors who described the syndromes. West syndrome becomes „Syndrome with epileptic infantile spasms”; Benign focal childhood epilepsies become „self-limited epilepsies”: Idiopathic epilepsy with centrotemporal spikes (Rolandic epilepsy becomes „Self-limited epilepsy with centrotemporal spikes”; Panayiotopoulos syndrome or early onset benign occipital epilepsy becomes „Self-limited epilepsy with autonomic seizures”; Occipital epilepsy Gastaut type (late onset benign occipital epilepsy) becomes „Childhood occipital visual epilepsy”. The new change in terminology classifies epilepsies according to age of onset. According to the new concept of developmental and/or epileptic encephalopathy and epileptic encephalopathy, epilepsy and delayed development are two conditions with a common etiology (structural and genetic causes), in which aggressive antiepileptic treatment is necessary. In epileptic encephalopathies that are pharmacoresistant, seizures and/or EEG discharges impair neuropsychic development and cognitive functions.
According to the age of onset, epileptic encephalopathies are: early infantile (Early-infantile developmental and epileptic encephalopathy, infantile epileptic spasms syndrome or West syndrome, migrating focal seizures, Dravet syndrome) and late (Lennox-Gastaut syndrome, epileptic encephalopathies with a sharp-slow wave activation in sleep, Landau Kleffner syndrome, atypical benign focal epilepsy in childhood, hemiconvulsion-hemiplegia-syndrome, febrile infection-related epilepsy syndrome; With onset at different ages are epileptic syndromes with progressive neurological damage, Rasmussen syndrome, progressive myoclonic epilepsy, febrile infection-related epilepsy syndrome. Neurocognitive and behavioral abnormalities may improve with seizure control and reduction of interictal epileptic activity. Developmental encephalopathies present with delayed neuropsychiatric development or intellectual deficit due to non-progressive brain damage, with or without epilepsy, and neurocognitive and behavioral deficits remain regardless of epilepsy control. Current algorithms for the treatment of epilepsies with monotherapy or rational polytherapy are presented, incl. with new antiseizure medications (ASM), emphasizing the treatment of epileptic encephalopathies.

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Published

30.12.2023

How to Cite

Bojinova, V., & Milanov, I. (2023). Current terminology, diagnosis and modern treatment options. Bulgarian Neurology, 24(3), 91–100. Retrieved from https://www.nevrologiabg.com/journal/index.php/neurology/article/view/146

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