Abstracts

RATIONALE: In the surgical treatment for pediatric intractable epilepsy associated with extensive cortical dysplasia, it is often difficult to choose a suitable surgical procedure. We experienced three cases of intractable frontal lobe epilepsy in early chirdhood associated with diffuse cortical dysplasia who were treated by extensive frontal disconnection; i.e. resection of premotor and supplementary motor areas and total disconnection of the rostral frontal lobe and obtained good postoperative outcomes. The point and indication of the extensive frontal lobectomy are also described.
METHODS: Case 1: A 3-year and 5-month-old boy. His seizure started as brief tonic seizure at 10 days after birth. Daily epileptic spasms and complex partial seizures also developd at the age of 3 months and 18 months, respectively. Case 2: A 2-year and 2-month-old girl. Her seizure started as generalized tonic-clonic seizure at 7 months after birth. Daily generalized tonic seizures developd at the age of 14 months. Case 3: A 2-year and 4-month-old boy. His seizure started as myoclonic seizure in the face and bilateral arms at 20 days after birth. Daily epileptic spasms and nocturnal generalized tonic-clonic seizure also developd at the age of 1 month and 18 months, respectively.
All of three cases showed very similar findings in preoperative studies including physical examination, neuropsychological studies, and imaging studies. They exhibited developmental delay and mild paresis in the upper extremity contralateral to the cerebral lesion side. In MR imaging corticomedullary demarcation was not clear in the lesion-side frontal lobe. In [(18)F]-fluorodeoxyglucose ((18)FDG)-PET scan the finding of hypometabolism was detected more extensive area including the part of parietal lobe than the abnormal area shown in MRI. In magnetoencephalography and ictal SPECT showed the accumulation of spike dipoles and hyperperfusion in the frontal lesion, respectively.
RESULTS: An extensive frontal disconnection was performed on all of three patients. After resecting the premotor and supplementary motor corteices rostral to the precentral sulcus totally, residual frontal cortex was disconnected from the genu and the subcallosal area medially to the anterior border of the insular cortex along the sphenoidal ridge laterally. Postoperatively, the disappearance of seizures and developmental catch-up were obtained in all patients. The pathological diagnosis was cortical dysplasia in all patients.
CONCLUSIONS: Total frontal disconnection rostral to the precentral sulcus may be a good surgical alternative for the treatment of intractable pediatric frontal lobe epilepsy associated with diffuse cortical dysplasia.