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Early-onset absence epilepsy at eight months of age

Yu Kobayashi1, Noriyuki Akasaka, Tsukasa Ohashi

  • 1Department of Pediatrics, Nishi-Niigata Chuo National Hospital, Niigata. u-kb@masa.go.jp

Epileptic Disorders : International Epilepsy Journal with Videotape
|January 20, 2012
PubMed

Insights

A rare case of early-onset absence epilepsy in an infant girl showed intractable seizures. This severe form of epilepsy, with myoclonic features, suggests a potential new syndrome with poor prognosis.

Area of Science:

  • Neurology
  • Pediatric Neurology
  • Epileptology

Background:

  • Early-onset absence epilepsy (EOAE) is a heterogeneous group of epilepsies with onset before age four.
  • Absence seizures beginning in the first year of life are exceptionally rare.

Observation:

  • A case of a female infant presenting with intractable absence seizures starting at eight months of age.
  • Seizure semiology included loss of responsiveness, upward eye deviation, and myoclonic jerks.
  • Electroencephalogram revealed generalized 2-2.5 Hz spike-and-wave discharges.

Findings:

  • The patient's seizures were refractory to standard antiepileptic drugs.
  • Adrenocorticotropic hormone (ACTH) provided only transient seizure control.
  • Extensive metabolic, cytogenetic, and genetic analyses failed to identify an etiology.

Implications:

  • Intractable, very early-onset absence epilepsy with myoclonic features indicates a poor prognosis.
  • This presentation may represent a distinct epileptic syndrome, potentially termed 'early infantile absence epilepsy'.
  • Further research is needed to understand the underlying mechanisms and optimize treatment for this rare epilepsy subtype.

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