D. Hata et al., Intraoperative electrocorticography and successful focus resection in a case of Sturge-Weber syndrome, SEIZURE-E J, 7(6), 1998, pp. 505-508
This paper reports a surgically treated case of Sturge-Weber syndrome (SWS)
in which the epileptic foci and haemangiomatosis were successfully resecte
d under monitoring of intraoperative electrocorticography. The patient was
a 19-month-old female infant who was referred to our hospital because of fr
equent hemi-tonic-clonic convulsions that were resistant to anticonvulsant
therapy. Serial MRI showed progressive atrophy in the left fronto-parieto-t
emporal lobe, and gyral enhancement by gadolinium corresponded to venous ha
emangiomatosis of SWS. Three-dimensional reconstruction of the MR images wa
s performed using the Viewing Wand System. Conventional EEG taken before th
e operation showed slow activity in the left frontal lobe. Intraoperative E
CoG revealed spike focus at the posterior temporal cortex to the margin of
the haemangiomatosis. Lesionectomy with lobar corticectomy of the total fro
ntal and parietal lobe and part of the temporal lobe was performed. The epi
leptogenic focus detected by ECoG in the posterior temporal lobe was also r
esected. In post-excisional ECoG, epileptogenic activities had disappeared.
The patient had hemiparesis and hemihypesthesia just after the surgery, bu
t gradually recovered from the paresis and almost has normal motor function
except for right-hand clumsiness up to 1 year after surgery. The present s
tudy demonstrated that lobar corticectomy of the haemangiomatosis-affected
cortex with resection of the neighbouring epileptogenic focus is a good sur
gical alternative even if a haemangiomatosis of the SWS affected multilobar
corti of the hemisphere.