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"Electro-clinical syndromes" with onset in paediatric age: the highlights of the clinical-EEG, genetic and therapeutic advances

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Author(s): Parisi Pasquale | Verrotti Alberto | Paolino Maria | Castaldo Rosa | Ianniello Filomena | Ferretti Alessandro | Chiarelli Francesco | Villa Maria

Journal: The Italian Journal of Pediatrics
ISSN 1720-8424

Volume: 37;
Issue: 1;
Start page: 58;
Date: 2011;
Original page

Keywords: Electro-clinical syndrome | Paediatric Epilepsy | EEG | Epileptic Syndrome | ILAE classification

ABSTRACT
Abstract The genetic causes underlying epilepsy remain largely unknown, and the impact of available genetic data on the nosology of epilepsy is still limited. Thus, at present, classification of epileptic disorders should be mainly based on electroclinical features. Electro-clinical syndrome is a term used to identify a group of clinical entities showing a cluster of electro-clinical characteristics, with signs and symptoms that together define a distinctive, recognizable, clinical disorder. These often become the focus of treatment trials as well as of genetic, neuropsychological, and neuroimaging investigations. They are distinctive disorders identifiable on the basis of a typical age onset, specific EEG characteristics, seizure types, and often other features which, when taken together, permit a specific diagnosis which, in turn, often has implications for treatment, management, and prognosis. Each electro-clinical syndrome can be classified according to age at onset, cognitive and developmental antecedents and consequences, motor and sensory examinations, EEG features, provoking or triggering factors, and patterns of seizure occurrence with respect to sleep. Therefore, according to the age at onset, here we review the more frequently observed paediatric electro-clinical syndrome from their clinical-EEG, genetic and therapeutic point of views.
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