Childhood absence epilepsy
Childhood absence epilepsy (CAE), formerly known as pyknolepsy, is an idiopathic generalized epilepsy syndrome that begins in childhood, typically between the ages of 4 and 10, with a peak onset between 5 and 7 years. It is characterized by frequent absence seizures — brief episodes of impaired awareness that start and end suddenly, often accompanied by subtle automatisms such as eyelid fluttering or lip smacking. Seizures usually last less than 30 seconds and may occur dozens or even hundreds of times per day. Children with CAE are otherwise developmentally normal, and the electroencephalogram (EEG) shows characteristic generalized 3 Hz spike-and-wave discharges. The syndrome is genetically complex, with seizures believed to arise from thalamocortical network dysfunction. Prognosis is generally favorable, with many children achieving seizure remission during adolescence.[1][2] Ethosuximide is the preferred first-line treatment.[3]
- ^ Hirsch, Edouard; French, Jacqueline; Scheffer, Ingrid E.; Bogacz, Alicia; Alsaadi, Taoufik; Sperling, Michael R.; Abdulla, Fatema; Zuberi, Sameer M.; Trinka, Eugen; Specchio, Nicola; Somerville, Ernest (June 2022). "ILAE definition of the Idiopathic Generalized Epilepsy Syndromes: Position statement by the ILAE Task Force on Nosology and Definitions". Epilepsia. 63 (6): 1475–1499. doi:10.1111/epi.17236. ISSN 1528-1167. PMID 35503716. S2CID 248504453.
- ^ Hirsch E, Thomas P, Panayiotopoulos C (2007). "Childhood and absence epilepsies". Epilepsy: A Comprehensive Textbook: 2397–2411.
- ^ Kessler, Sudha Kilaru; McGinnis, Emily (2019). "A Practical Guide to Treatment of Childhood Absence Epilepsy". Paediatric Drugs. 21 (1): 15–24. doi:10.1007/s40272-019-00325-x. ISSN 1179-2019. PMC 6394437. PMID 30734897.