engleski

Typical absence epilepsy with onset before the age of four years-report of eleven patient

Purpose: Childhood absence epilepsy (CAE) typically starts between 4 and 7 years of age. Onset before 4 years is rare, and children with onset in first year of life are considered almost exceptional. Methods: We report the clinical and electrophysiologic features with follow -up of 11 children with typical absence epilepsy with range of onset from 6 months to 3.6 years (mean age of seizure onset 26 months), 9 girls and 2 boys. Results: All patient had episodes of unresponsiveness associated with bilaterally synchronous spike-and-wave discharges at 3 Hz with a normal background on EEG. Brain CT/MR was done in 9/11 patient, 7 was normal, one showed arachnoid cyst and one porencephalic cysts. Eight of 11 patient had normal neurodevelopment, one had cerebral palsy, and two speech delay with ADHD. Seven pateints were seizure-fee (EEG normal) with initial monotherapy with valproic acid (VPA). In 1 patients first therapy was ethosuximide and in two lamotrigine without efect. Therapy was changed in VPA and from that moment there are seizure -free with normal EEG .In girl with Dravet syndrome presented with absence combinated with other types of seizures therapy with VPA was unsuccesful. One patient still had absence seizures with multiple AEDs (genetic evaluation of SLC2A1gene was negative). The youngest patient (onset of seizure -6 months) is seizure free for 3 years after medication withdrawal without neurodevelopmental delay. Conclusion: In our patients absence seizures presenting before the age of 4 appeared to have quite a good long-term clinical prognosis, the neu ropsychological outcome was comparable to that of childhood typical absence epilepsy presenting after 4 years of age. The exceptions are patients with absence seizure associated with other forms of attacks.

typical absence epilepsy ; early childhood

nije evidentirano