Identification of primary epileptogenic tuber is often challenging in patients with bilateral multiple tubers in tuberous sclerosis complex. We report a 3 year old girl with tuberous sclerosis complex presenting with intractable epilepsy and multiple tubers, who was successfully treated by corpus callosotomy and subsequent resective surgery. She initially presented with West syndrome which was intractable to ACTH therapy and multiple antiepileptic medications. Her EEG was characterized by generalized and multifocal spikes, and by non-focal changes at seizure onset. Ictal single photon emission computed tomography (SPECT) showed no focal hyperperfusion. Total corpus callosotomy was performed to alleviate her drop attacks. Postoperatively, interictal spikes were completely lateralized to the right hemisphere. Since her seizures were still kept uncontrolled with medications, second pre-surgical evaluation was planned and ictal SPECT disclosed focal hyperperfusion at a tuber in the right frontal lobe. After complete resection of the right frontal tuber, she was completely seizure free on antiepileptic medications for 1 year with no additional neurological deficits. Generalized or multifocal electroencephalographic (EEG) spikes are occasionally lateralized to one hemisphere after corpus callosotomy, which may help identifying the primary epileptogenic focus. Repeat pre-surgical evaluation is important after corpus callosotomy in patients with generalized or multifocal epileptiforms in EEG.
|出版ステータス||出版済み - 2013 5月|