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Childhood Absence Epilepsy evolving to Eyelid Myoclonia with Absence Epilepsy

Jessica Galli1, Serena Micheletti2, Laura Malerba3

  • 1Clinical and Experimental Sciences Department, University of Brescia, Italy; Child Neurology and Psychiatry Unit, ASST Civil Hospital -Brescia, Italy.

Seizure
|July 21, 2018
PubMed

Insights

Childhood Absence Epilepsy (CAE) can evolve into Eyelid Myoclonia with Absence Epilepsy (EMA) after therapy withdrawal. This suggests CAE and EMA are dynamic, interconnected "system epilepsies" in children.

Area of Science:

  • Pediatric Neurology
  • Epileptology
  • Clinical Neuroscience

Background:

  • Childhood Absence Epilepsy (CAE) is known to potentially evolve into generalized tonic-clonic seizures or juvenile myoclonic epilepsy.
  • The evolution of CAE to Eyelid Myoclonia with Absence Epilepsy (EMA) has not been previously documented.

Purpose of the Study:

  • To report the electroclinical features of children with CAE who developed EMA following therapy withdrawal.
  • To investigate the potential link and dynamic evolution between CAE and EMA.

Main Methods:

  • A case series of 5 pediatric patients with CAE diagnosed over ten years at an Epilepsy Center.
  • Inclusion criteria included CAE diagnosis, a minimum 3-year follow-up, and documented progression to EMA after discontinuing anti-epileptic drugs.

Main Results:

  • All 6 evaluated subjects were female, presenting typical absences and negative intermittent photic stimulation (IPS).
  • Following valproate withdrawal, all patients developed EMA, characterized by eyelid myoclonia and/or brief absences associated with generalized spike/polyspike-wave discharges, often triggered by IPS or eye closure.

Conclusions:

  • This study documents a novel evolution of CAE to EMA, supporting the hypothesis that these conditions represent dynamic, interconnected processes.
  • CAE and EMA may be classified as
  • system epilepsies,
  • highlighting brain network susceptibility during developmental periods like childhood and puberty.
Abstract

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