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Frontal Disconnection for Treating Mild Malformation of Cortical Development with Oligodendroglial Hyperplasia in Epilepsy (MOGHE) in the Frontal Lobe
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Total callosotomy for a case of lissencephaly presenting with West syndrome and generalized seizures

T Kamida1, T Maruyama, M Fujiki

  • 1Department of Neurosurgery, Oita University Faculty of Medicine, 1-1 Idaigaoka, Hasama-machi, Oita, 879-5593, Japan. kamida@med.oita-u.ac.jp

Child'S Nervous System : Chns : Official Journal of the International Society for Pediatric Neurosurgery
|January 18, 2005
PubMed

Insights

This case report highlights a rare instance of West syndrome in an infant with lissencephaly. Corpus callosotomy effectively reduced intractable seizures and improved developmental trajectory.

Area of Science:

  • Pediatric Neurology
  • Epileptology
  • Neuroscience

Background:

  • West syndrome, a severe infant epilepsy, is often associated with underlying brain abnormalities.
  • Lissencephaly, a malformation of cortical development, presents significant neurological challenges.
  • Intractable seizures in infants necessitate exploration of advanced therapeutic options.

Observation:

  • An 11-month-old infant with lissencephaly presented with medically intractable West syndrome and generalized tonic seizures.
  • Electroencephalography (EEG) revealed hypsarrhythmia and focal epileptiform discharges.
  • Magnetic Resonance Imaging (MRI) confirmed severe bilateral lissencephaly.

Findings:

  • Total callosotomy was performed at 11 months of age.
  • Postoperatively, there was a prominent decrease in seizure frequency.
  • The infant demonstrated resumed developmental progression after the surgical intervention.

Implications:

  • This case suggests the corpus callosum's significant role in certain forms of symptomatic West syndrome.
  • Corpus callosotomy emerges as a viable surgical consideration for carefully selected cases of symptomatic West syndrome.
  • Timing of surgical intervention is a critical factor in managing intractable infant epilepsies associated with brain malformations.
Abstract

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