EPILEPSY WITH MYOCLONIC ABSENCES (EMA)

Overview

Epilepsy with myoclonic absences is a rare syndrome and 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 impairment is eventually seen in 70% of cases. Other seizure types (generalized tonic-clonic and atonic seizures) occur in the majority of patients.

Clinical context

Seizures begin between 1 to 12 years of age (peak 7 years). There is a male predominance (70%). Antecedent and birth history is unremarkable. Head size and neurological examination are typically normal. At presentation approximately half of cases have learning impairment, and this eventually affects 70% of cases. Myoclonic absence seizures are often treatment resistant. Remission occurs in about 40% of cases and prognosis is better if myoclonic absence seizures are the only seizure type and are controlled with medication.