Abstract
We studied 14 patients with severe myoclonic epilepsy of infancy (SMEI), mean age
± SD 8.5 ± 4.14 yr, 10 girls, and 13 patients with myoclonic astatic epilepsy (MAE),
mean age ± SD 11.8 ± 5.19 yr, six girls. All patients underwent EEGs, cranial magnetic
resonance imaging (MRI) and the Vineland scale for assessment of adaptive behavior
in areas related to communication, activities of daily living, socialization, and
motor skills. Our study revealed abnormalities in the neurological examination (ataxia,
mild pyramidal tract abnormalities, hyperactivity, autism spectrum disorder) in all
14 patients with SMEI and 4 of the 8 patients with MAE. EEGs showed background slowing,
and focal and generalized epileptiform activity in 10 patients with SMEI. In patients
with MAE EEGs showed monomorphic centroparietal theta rhythm in nine patients, and
generalized epileptiform activity in all patients. Deterioration of adaptive behavior
occurred in all the patients with SMEI and in eight patients with MAE. SMEI and MAE
showed clinical and EEG findings according to those previously described. Nevertheless,
severe epileptic encephalopathy was detected in patients with SMEI while patients
with MAE presented with a milder form of epileptic encephalopathy. The outcome was
good in approximately one third of the patients with MAE.
Keywords
Myoclonic astatic epilepsy - myoclonic epilepsy of infancy - EEG - Vineland scale
