Intraoperative electrocorticography and successful focus resection in a case of Sturge-Weber syndrome

This paper reports a surgically treated case of Sturge-Weber syndrome (SWS) in which the epileptic foci and haemangiomatosis were successfully resected under monitoring of intraoperative electrocorticography. The patient was a 19-month-old female infant who was referred to our hospital because of frequent hemi-tonic-clonic convulsions that were resistant to anticonvulsant therapy. Serial MRI showed progressive atrophy in the left fronto-parieto-temporal lobe, and gyral enhancement by gadolinium corresponded to venous haemangiomatosis of SWS. Three-dimensional reconstruction of the MR images was performed using the Viewing Wand System. Conventional EEG taken before the operation showed slow activity in the left frontal lobe. Intraoperative ECoG revealed spike focus at the posterior temporal cortex to the margin of the haemangiomatosis. Lesionectomy with lobar corticectomy of the total frontal and parietal lobe and part of the temporal lobe was performed. The epileptogenic focus detected by ECoG in the posterior temporal lobe was also resected. In post-excisional ECoG, epileptogenic activities had disappeared. The patient had hemiparesis and hemihypesthesia just after the surgery, but gradually recovered from the paresis and almost has normal motor function except for right-hand clumsiness up to 1 year after surgery. The present study demonstrated that lobar corticectomy of the haemangiomatosis-affected cortex with resection of the neighbouring epileptogenic focus is a good surgical alternative even if a haemangiomatosis of the SWS affected multilobar corti of the hemisphere.