Deivasumathy Muthugovindan1, Adam L Hartman. 1. Division of Pediatric Neurology, John M. Freeman Pediatric Epilepsy Center, Johns Hopkins Medicine, Baltimore, MD 21287, USA.
Abstract
BACKGROUND: Pediatric epilepsies are a heterogeneous group of disorders that encompass various epilepsy syndromes ranging from benign to progressive and catastrophic. The past 2 decades have seen major advancements in the appreciation of epilepsy syndromes and their underlying mechanisms. Modern neuroimaging methods have helped to identify structural pathologies and genetic discoveries led to identifying new syndromes and characterization of old syndromes. Epilepsy syndromes are categorized on the basis of a number of features including seizure types, age of onset, clinical features, electroencephalographic expression, and response to treatment. Multiple seizure types, evolution from one type to and another and age-dependent expression are some of the unique features of epilepsy syndromes. The International League Against Epilepsy uses the age-related expression of these syndromes and classifies them by different age groups, allowing easy identification. Early recognition and identification of epilepsy syndromes is essential to provide valuable information on management and prognosis. REVIEW SUMMARY: The purpose of this article is to review the literature data on the prevalence, clinical manifestations, electrographic findings, pathophysiology, prognosis, and treatment options for these syndromes. CONCLUSIONS: Accurate and early diagnosis of epilepsy syndrome, when possible, is essential for both evaluation and treatment because specific antiepileptic therapies are increasingly recognized.
BACKGROUND: Pediatric epilepsies are a heterogeneous group of disorders that encompass various epilepsy syndromes ranging from benign to progressive and catastrophic. The past 2 decades have seen major advancements in the appreciation of epilepsy syndromes and their underlying mechanisms. Modern neuroimaging methods have helped to identify structural pathologies and genetic discoveries led to identifying new syndromes and characterization of old syndromes. Epilepsy syndromes are categorized on the basis of a number of features including seizure types, age of onset, clinical features, electroencephalographic expression, and response to treatment. Multiple seizure types, evolution from one type to and another and age-dependent expression are some of the unique features of epilepsy syndromes. The International League Against Epilepsy uses the age-related expression of these syndromes and classifies them by different age groups, allowing easy identification. Early recognition and identification of epilepsy syndromes is essential to provide valuable information on management and prognosis. REVIEW SUMMARY: The purpose of this article is to review the literature data on the prevalence, clinical manifestations, electrographic findings, pathophysiology, prognosis, and treatment options for these syndromes. CONCLUSIONS: Accurate and early diagnosis of epilepsy syndrome, when possible, is essential for both evaluation and treatment because specific antiepileptic therapies are increasingly recognized.