CHILDHOOD ABSENCE EPILEPSY (CAE)

OVERVIEW

Childhood absence epilepsy is an idiopathic generalized epilepsy that should be considered in an otherwise normal child with multiple daily absence seizures associated with 2.5-4Hz generalized spike-wave. Absence seizures are provoked by hyperventilation.

Clinical context

This syndrome is characterized by onset of frequent absence seizures between the ages of 2 to 13 years (usually 4-10 years). In children with onset >10 years of age the clinical distinction between this syndrome and juvenile absence epilepsy is largely based on frequency of absence seizures - if absences are multiple daily, this syndrome is more likely. Childhood absence epilepsy is more common in girls (60-75% of cases). Antecedent and birth history is normal. A previous history of febrile seizures may occur (seen in 10-15% of cases). Neurological examination and head size are normal. Development and cognition are typically normal. Attention deficit hyperactivity disorder and cognitive/intellectual impairment may occur. Seizures remit with age in 60% of individuals, remaining patients evolve to have other idiopathic generalised epilepsy syndromes. Presence of automatisms during seizures may be associated with better outcome.

CAUTION Onset of absence seizures <4 years consider glucose transporter 1 deficiency syndrome.