Childhood absence epilepsy

Introduction
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By C P Panayiotopoulos MD PhD

Childhood absence epilepsy is also known as or subsumes Pyknolepsy and Petit mal seizures. -ed.

Childhood absence epilepsy is the prototype genetic generalized epilepsy syndrome of typical absence seizures. It is common and age related, and it affects otherwise normal children. It manifests with severe and frequent (pyknoleptic) absence seizures, around 10 seconds each, many times per day. EEG shows classical generalized 3 Hz spike-and-slow wave discharges. Prognosis of childhood absence epilepsy is excellent if properly diagnosed and treated. In this update, the author details developments in the pathophysiology, genetics, and pharmacological treatment of childhood absence epilepsy. The focus is on the current understanding of its clinical and EEG phenotype, epidemiology, and outcome along with the differential diagnosis associated with other idiopathic/genetic generalized epilepsies involving absence seizures. The author particularly emphasizes that most of the older and newer antiepileptic drugs are contraindicated for the treatment of childhood absence epilepsy.

Key Points

  • Childhood absence epilepsy is the most characteristic and classic example of a genetic generalized epilepsy characterized by typical absence seizures of high daily frequency and severe impairment of consciousness.
  • Probably any other types of seizure in the active period of childhood absence epilepsy are incompatible with this diagnosis. Mild impairment of consciousness in untreated patients is an exclusion criterion.
  • Differential diagnosis includes other types of syndromes manifesting with typical absence seizures; “epilepsy with absences seizures of childhood onset” is not synonymous with childhood absence epilepsy.
  • Prognosis is usually excellent for patients diagnosed on strict inclusion and exclusion criteria for childhood absence epilepsy.
  • Ethosuximide, sodium valproate, and lamotrigine are the most effective antiepileptic drugs.

In This Article

Introduction
Historical note and nomenclature
Clinical manifestations
Clinical vignette
Etiology
Pathogenesis and pathophysiology
Epidemiology
Differential diagnosis
Diagnostic workup
Prognosis and complications
Management
References cited
Contributors