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Fatal outcome in hemiconvulsion-hemiplegia syndrome

Himali Jayakody1, Charuta Joshi

  • 11Division of Child Neurology, University of Iowa Children's Hospital, Iowa City, IA, USA.

Journal of Child Neurology
|December 29, 2012
PubMed

Insights

Hemiplegia-hemiconvulsion-epilepsy syndrome can lead to severe complications. A case report details a fatal outcome due to malignant cerebral edema, highlighting the importance of continuous EEG monitoring.

Area of Science:

  • Pediatric Neurology
  • Epileptology
  • Neurocritical Care

Background:

  • Hemiplegia-hemiconvulsion-epilepsy syndrome involves unilateral seizures and subsequent hemiplegia.
  • Focal status epilepticus can cause unilateral cerebral edema and hemiatrophy.

Observation:

  • A 3-year-old girl with this syndrome developed malignant cerebral edema and temporal lobe herniation.
  • Voltage suppression on continuous EEG indicated clinical status worsening after seizure freedom.

Findings:

  • The case presented with neuroimaging, EEG, and autopsy findings consistent with fatal cerebral edema.
  • Literature review revealed no recent reports of malignant edema or death in this syndrome.

Implications:

  • This case underscores the potential for severe, fatal complications in hemiplegia-hemiconvulsion-epilepsy syndrome.
  • Continuous EEG monitoring is crucial for detecting subtle clinical deterioration in the pediatric intensive care unit.

Keywords:
EEGacute encephalopathyelectroencephalographyepilepsyfocal status epilepticushemiconvulsionhemiplegia

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