Epilepsy with myoclonic absences should be considered in a child who presents with frequent daily myoclonic absence seizures. At presentation approximately half the children are developmentally and neurologically normal, learning disability is eventually seen in 70% of cases. Other seizure types (generalized tonic-clonic and atonic seizures) occur in the majority of patients. Prognosis is more favorable if myoclonic absence seizures are controlled.
This syndrome is characterized by onset of seizures between 1 to 12 years of age (peak 7 years). Seizures are often difficult to treat and may continue into adulthood. Both sexes are affected, with a male predominance (70%). Head size and neurological examination are typically normal. At presentation approximately half of cases have learning disability. A diagnosis of learning disability is eventually found in 70% of cases. Cognitive function is preserved in children in whom myoclonic absences are controlled.