Neuropediatrics 2006; 37 - THP71
DOI: 10.1055/s-2006-945894

EPILEPSY SURGERY FOR SYMPTOMATIC WEST SYNDROME WITH CORTICAL MALFORMATIONS

K Kagitani-Shimono 1, K Imai 1, T Okinaga 1, Y Mogami 1, K Araya 1, S Nabatame 1, A Kato 1, K Ozono 1, T Nagai 1
  • 1Department of Developmental Medicine (Pediatrics), Osaka University Graduate School of Medicine, Suita, Osaka, Japan

Objectives: West syndrome is one of the most intractable child epilepsy syndromes with poor prognosis. In recent years, some patients of symptomatic West syndrome (S-West) become to be considered as candidate for epilepsy surgery. We investigated the timing of surgical treatment, pathological findings and prognosis in the operated S-West patients with cortical malformations (CM).

Methods: We studied consecutive operated S-West patients, who had spasms and modified hypsarrhythmia on scalp EEG between 2002 and 2004 in our hospital. All patients were intractable against ACTH therapy and more than three conventional antiepileptic drugs. Four patients revealed abnormal findings in magnetic resonance imaging (one patient with hemimegalencephaly, and three with cortical dysplasia). All patients had evaluated with scalp interictal and ictal EEG, single photon emission computed tomography (SPECT), 18F-fluorodeoxyglucose positron emission tomography (FDG-PET), and magnetoencephalogram (MEG).

Results: There are 2 males and 2 females. The mean age at onset of spasms was 4 M (1–8M). The mean age at surgery was 1Y7M (9M- 2Y1M). The mean period between seizure onset and surgery was 14 M (6–24M). The surgical proceedings were functional hemispherectomy for two patients, focal resection for one patient and focal corticectomy with disconnection to temporal lobe for the other one. The mean follow-up period was 20M (13–36M). All patients showed good seizure outcomes (Engel classification I and II in each two patients). Surgical intervention had favorable effects on development in all patients.

Conclusion: Four patients, who underwent epilepsy surgery for S-West syndrome, were reported. Though all suspected poor prognosis in seizures and development with extensive congenital brain anomaly, epilepsy surgery led good outcome for both seizure reduction and psychomotor development. Surgery would be considered before accompanying marked developmental delay in S-West patients with CM.