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Epilepsy with myoclonic absences with early onset: a follow-up study
A Verrotti1, R Greco, F Chiarelli
1Department of Medicine, Pediatrics Section, University of Chieti, Italy. chiarelli@unich.it
Insights
Early-onset cryptogenic myoclonic absence seizures can have a good long-term prognosis. Valproate monotherapy is effective, and mental retardation is linked to poor seizure control in these pediatric epilepsy cases.
Area of Science:
- Pediatric Neurology
- Epileptology
- Clinical Neuroscience
Background:
- Cryptogenic myoclonic absence seizures are a form of epilepsy presenting in early childhood.
- The long-term prognosis and potential for developmental impact require further investigation.
Observation:
- Six children (four girls, two boys) with early-onset cryptogenic myoclonic absence seizures were studied.
- Neurologic evaluations were initially normal; follow-up lasted at least five years.
Findings:
- Two patients developed severe mental retardation, frequent seizures, and abnormal EEGs despite polytherapy.
- The remaining four patients achieved seizure freedom, normal EEGs, and no mental retardation with monotherapy (valproate).
Implications:
- Early-onset myoclonic absence seizures may have a favorable long-term outcome.
- Valproate demonstrates efficacy as a monotherapy for these seizures.
- Poor seizure control is associated with the development of mental retardation.
Abstract:
We studied six children (four girls and two boys) suffering from cryptogenic myoclonic absence seizures with early onset. The age at onset of the seizures ranged between 6 and 27.8 months (mean age +/- SD: 18.5+/-12.4 months). The neurologic evaluation was normal in all patients at the first hospital admission. After the diagnosis, we followed up all children for at least 5 years. At the end of follow-up, two of these patients (a girl and a boy) showed severe mental retardation, a high number (from one to three per day) of seizures, and persistent pathologic electroencephalograms. The other patients showed normal electroencephalograms: all of them were seizure free and without mental retardation. The two patients with mental retardation have been treated with polytherapy. In all other children we used valproate alone successfully. Our data suggest that myoclonic absence seizures with early onset can have a good long-term prognosis. Valproate is a useful anticonvulsant drug in these patients. Mental retardation is present only in patients with poor seizure control.