Epilepsy surgery for early infantile epileptic encephalopathy (ohtahara syndrome)

Saleem I. Malik, Carlos A. Galliani, Angel W. Hernandez, David J. Donahue

Research output: Contribution to journalArticlepeer-review

Abstract

Early infantile epileptic encephalopathy or Ohtahara syndrome is the earliest form of the age-dependent epileptic encephalopathies. Its manifestations include tonic spasms, focal motor seizures, suppression burst pattern, pharmaco-resistance, and dismal prognosis. The purpose of this study was to evaluate the effectiveness of epilepsy surgery in selected infants. We identified 11 patients, 9 from the literature and 2 from our institution that fulfilled diagnostic criteria of Ohtahara syndrome and had undergone epilepsy surgery in infancy. Seven of the 11 infants have remained seizure free (Engel class IA) and four are reportedly having rare to infrequent seizures (Engel class IIB). All patients experienced "catch up" development. In contrast to Ohtahara's15 pharmacotherapy managed patients, who had a mortality rate of approximately fifty percent, and those that survived continued to have seizures and were severely impaired, the outcome of selected surgically managed patients is much more favorable.

Original languageEnglish (US)
Pages (from-to)1607-1617
Number of pages11
JournalJournal of child neurology
Volume28
Issue number12
DOIs
StatePublished - Dec 2013
Externally publishedYes

Keywords

  • cortical resection
  • Early infantile epileptic encephalopathy
  • epilepsy surgery
  • Ohtahara syndrome
  • suppression bursts

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

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