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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.

Clinical context

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.

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