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Published on: December 18, 2016
Epilepsy surgery for early infantile epileptic encephalopathy (ohtahara syndrome)
Saleem I Malik1, Carlos A Galliani, Angel W Hernandez
11Department of Neurology, Cook Children's Medical Center, Fort Worth, TX, USA.
Insights
Epilepsy surgery offers a favorable outcome for infants with Ohtahara syndrome, a severe early infantile epileptic encephalopathy. Surgical intervention resulted in seizure freedom or infrequent seizures and improved development in most patients.
Area of Science:
- Neurology
- Pediatric Neurology
- Epileptology
Background:
- Ohtahara syndrome is the earliest and most severe form of age-dependent epileptic encephalopathies.
- It is characterized by tonic spasms, focal motor seizures, suppression-burst EEG patterns, and pharmaco-resistance.
- The prognosis is typically dismal with high mortality and severe impairment in survivors.
Purpose of the Study:
- To evaluate the effectiveness of epilepsy surgery in infants diagnosed with Ohtahara syndrome.
- To compare surgical outcomes with those of pharmacotherapy-managed patients.
Main Methods:
- A retrospective review of 11 infants meeting Ohtahara syndrome criteria who underwent epilepsy surgery in infancy.
- Data was compiled from 9 literature cases and 2 cases from the authors' institution.
Main Results:
- Seven of 11 infants (63.6%) achieved complete seizure freedom (Engel class IA).
- Four infants (36.4%) experienced rare to infrequent seizures (Engel class IIB).
- All surgically treated infants demonstrated catch-up development.
Conclusions:
- Epilepsy surgery is a highly effective treatment for selected infants with Ohtahara syndrome.
- Surgical management offers a significantly more favorable outcome compared to pharmacotherapy, with reduced mortality and improved developmental trajectories.
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.
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