Absence epilepsy starting before the age of 3 years is uncommon and its nosological placement is still debated: a distinct and well-defined form of idiopathic generalized epilepsy (IGE) or the first manifestation of different types of less benign IGE syndromes. The purpose of our work was to define electroclinical features, therapy and outcome of early-onset absence epilepsy. We conducted a retrospective multicentre study. The inclusion criteria were: 1) typical absence with onset before the age of 3 years of; 2) 3 Hz bilateral and symmetrical spike-wave discharges and normal background activity on EEG recordings; 3) absence of neurological signs or development delay at onset; 4) unremarkable pregnancy, delivery and labour. We enrolled 33 patients with typical absence seizure that started at 8 to 36 months (mean 27.3) of age. At follow-up (range from 1 to 38.7 years, mean 9.3), 31/33 (94%) patients presented normal cognitive skills, while two had a borderline cognitive level. Monotherapy with VPA was effective in 27/32 treated children. In 4 patients VPA was combined with ESM, and in one with LEV. One patient received LTG with complete control of seizures. Later, two/33 (6%) patients presented Juvenile myoclonic epilepsy, and two/33 developed an epilepsy with generalized tonic-clonic seizures. Conclusion: The data presented here delineate a rare form of IGE characterized by typical absences before the age of 3 years and a favourable course, which shares similarities with childhood and juvenile absence epilepsies. This entity might be recognized by the International League Against Epilepsy (ILAE) as a distinct IGE syndromes called ???Early-onset absence epilepsy??? (EOAE).
Assenze ad esordio precoce: una sindrome isolata? Studio collaborativo LICE su 33 casi
GALLI, Jessica;FAZZI, Elisa Maria;
2011-01-01
Abstract
Absence epilepsy starting before the age of 3 years is uncommon and its nosological placement is still debated: a distinct and well-defined form of idiopathic generalized epilepsy (IGE) or the first manifestation of different types of less benign IGE syndromes. The purpose of our work was to define electroclinical features, therapy and outcome of early-onset absence epilepsy. We conducted a retrospective multicentre study. The inclusion criteria were: 1) typical absence with onset before the age of 3 years of; 2) 3 Hz bilateral and symmetrical spike-wave discharges and normal background activity on EEG recordings; 3) absence of neurological signs or development delay at onset; 4) unremarkable pregnancy, delivery and labour. We enrolled 33 patients with typical absence seizure that started at 8 to 36 months (mean 27.3) of age. At follow-up (range from 1 to 38.7 years, mean 9.3), 31/33 (94%) patients presented normal cognitive skills, while two had a borderline cognitive level. Monotherapy with VPA was effective in 27/32 treated children. In 4 patients VPA was combined with ESM, and in one with LEV. One patient received LTG with complete control of seizures. Later, two/33 (6%) patients presented Juvenile myoclonic epilepsy, and two/33 developed an epilepsy with generalized tonic-clonic seizures. Conclusion: The data presented here delineate a rare form of IGE characterized by typical absences before the age of 3 years and a favourable course, which shares similarities with childhood and juvenile absence epilepsies. This entity might be recognized by the International League Against Epilepsy (ILAE) as a distinct IGE syndromes called ???Early-onset absence epilepsy??? (EOAE).I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
