Brief History – Evolution of the Concept – Classification
Severe myoclonic epilepsy in infancy (SMEI) was described by Dravet in 1978 (Dravet 1978). In 1989, the revised classification of the International League Against Epilepsy placed this syndrome under “epilepsies and syndromes undetermined as to whether they are focal or generalized,” since the syndrome shows both generalized and localized seizure types and EEG paroxysms (Commission on Classification and Terminology of the International League Against Epilepsy 1989). Because of the lack of myoclonias in similar cases with the same course and outcome and the same SCN1A gene mutation, the name was changed to Dravet Syndrome (DS), and classified among “epileptic encephalopathies” in the newer classification scheme proposed by the ILAE (Engel 2001).
Epidemiology
Dravet syndrome is rare, with an incidence of probably less than 1 per 40,000 (Hurst 1990) Males are more often affected, with a 2/1 ratio.
Etiology (Including Genetics)
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Abbreviations
- AEDs:
-
Antiepileptic drugs
- BME:
-
Benign myoclonic epilepsy
- SMEI:
-
Severe myoclonic epilepsy in infancy
References
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Bureau, M., Genton, P., Dravet, C. (2010). Dravet Syndrome. In: Panayiotopoulos, C.P. (eds) Atlas of Epilepsies. Springer, London. https://doi.org/10.1007/978-1-84882-128-6_128
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