難治性てんかんを呈した結節性硬化症の1手術治験例

A successfully operated case of tuberous sclerosis presenting with intractable epilepsy

I．はじめに

　結節性硬化症は，顔面の皮脂腺腫，知能発達遅延，てんかん発作を3徴候とする常染色体優性の遺伝性疾患である^10）．一般に多発性の脳内病変を伴い，てんかん発作も多発性焦点のことが多いので，てんかんに対する外科的治療は困難とされている^12）われわれは結節性硬化症に伴った難治性てんかんに対し，頭蓋内電極を留置して確認した焦点を含む側頭葉を切除して良好な結果を得たので報告する．

A 20-year-old male patient with tuberous sclerosis was admitted to our hospital complaining of intractable epilepsy. He had been suffering from frequent seizures despite anticonvulsant treatment since the age of 1 year. CT scan and magnetic resonance images showed multiple calcified lesions at the paraventricle and tem-poral lobe on both sides. Electroencephalogram showed left temporal lobe dominant spike waves. After deep electrodes were inserted into the left amygdala and hip-pocampus and subclural electrodes were placed on the left temporal cortex, clinical seizures were monitored by video and EEG. The primary focus was found in the amygdala. Left temporal lobectomy was carried Outthrough the cranio-orbital zygomatic approach. Post-operatively, the patient has been seizure-free for one year. Despite multiple intracerebral lesions in tuberous sclerosis, resection of the primary epileptogenic focus is needed to solve the seizure problem if the focus is localized.