Bilateral Resective Epilepsy Surgery in a Child with Tuberous Sclerosis: Case Report Pantaleo Romanelli, M.D., Howard L. Weiner, M.D., Souhel Najjar, M.D., Orrin Devinsky, M.D. Departments of Neurosurgery (PR, HLW, OD), Neurology (SN, OD), and Psychiatry (OD), and Division of Pediatric Neurosurgery (HLW), New York University-Mount Sinai Comprehensive Epilepsy Center, New York University Medical Center, New York, New York OBJECTIVE AND IMPORTANCE: Surgical intervention can reduce the bur- den of seizures in selected patients with tuberous sclerosis and medically refractory epilepsy. CLINICAL PRESENTATION: A child presented with tuberous sclerosis and se- vere epilepsy beginning in the first month of life and delayed development before 1 year of age. Video-electroencephalographic monitoring at the age of 1 year revealed a left temporal seizure focus. Repeat video- electroencephalography at 2 years of age revealed a right posterior quadrant seizure focus. Bilateral subdural electrodes were placed, confirming inde- pendent seizure onsets from the right parietal area (overlying a tuber) and prominent interictal activity over the left superior temporal region. INTERVENTION: The right parietal focus was resected, and electrodes were maintained over the left temporal focus. After right parietal resection, ictal discharges were recorded over the left temporal region; a corticec- tomy was performed 2 days later. No tonicoclonic or complex partial seizures have occurred during a follow-up period of more than 24 months. Simple partial motor seizures involving the right foot have been reduced by more than 80%, and other simple partial seizures have been eliminated. Postoperatively, there has been marked improvement in the patient’s cognitive and motor developmental status. CONCLUSION: In selected patients with bilateral seizure foci involving separate lobes, aggressive bilateral surgery can be safe and effective. (Neurosurgery 49:732–735, 2001) Key words: Epilepsy, Invasive monitoring, Pediatric, Tuberous sclerosis T uberous sclerosis is often associated with medically refractory epilepsy, usually resulting from cortical tubers, the areas of cortical dysplasia sur- rounded by reactive gliosis (2, 8, 9, 11). Seizures that begin in patients younger than 1 year are often refractory and can contribute to developmental delays (13, 17). Effective seizure control may re- duce the adverse developmental effects of chronic epilepsy. In some patients with tuberous scle- rosis, the epileptogenic foci are bilateral (2, 4, 10, 14, 16). Surgical therapy may be deferred because of the increased risks of bilateral surgery, or the predominant seizure focus may be resected in a pal- liative procedure (12). We report the case of a boy with progressive develop- mental delays who underwent a staged resection of independent right parietal and left temporal tubers and seizure foci, with an excellent seizure and de- velopmental outcome. CASE REPORT This male infant was the product of a normal full-term pregnancy. At 2 weeks of age, his mother noticed rhythmic slow movements of the right foot that recurred when the infant was 2 months old as clus- ters of tonic activity. At 3 months of age, the clusters involved the entire right lower extremity, and he was admitted to a local hospital for evaluation. A right focal motor seizure was secondarily general- ized. Electroencephalography revealed slowing of the left hemisphere. Com- puted tomographic scanning revealed multiple calcified nodules in the basal ganglia, and magnetic resonance imaging (MRI) revealed subependymal lesions consistent with tuberous sclerosis. Viga- batrin was gradually increased to 750 mg/d, but the patient continued to expe- rience four or five brief focal motor sei- zures per day. He then developed com- plex partial seizures, with right leg jerking and left head deviation. He was initially evaluated at our institution at 4.5 months of age. Examination revealed three hypopigmented macules; the neuro- logical examination was normal. Video- electroencephalography showed interictal left-sided slowing, frequent left posterior quadrant sharp waves, and bilateral myo- clonic jerks with generalized electrodecre- mental changes. Different combinations of medications, including carbamazepine, clonazepam, clorazepate, valproate, and gabapentin, were tried without success. The patient averaged two or three seizures per day, consisting of simple partial seizures in- volving the right arm and leg, complex partial seizures with head drop and a gelastic component, myoclonic clusters, and occasional tonicoclonic seizures. Dur- ing the next year, his social interactions regressed and a delay in motor and verbal skill acquisition became apparent. At age 2, video-electroencephalography moni- toring showed multifocal spikes and sharp waves predominantly over the right posterior quadrant, right posterior slowing, generalized polyspikes, and 732 Romanelli et al. Neurosurgery, Vol. 49, No. 3, September 2001