難治性てんかん発作を呈する多発性病変を伴う結節性硬化症の1手術例

Surgical Treatment for Intractable Epilepsy Caused by Tuberous Sclerosis Complex with Multiple Tubers: Case Report

Ⅰ．はじめに

　難治性てんかんに対する外科的治療については，その有用性が広く知られている．しかし，外科的治療ではてんかん発作の抑制が困難な症例も存在し，結節性硬化症（tuberous sclerosis complex；TSC）もその1つとして認識されてきた．しかし近年，十分な術前検索ならびに適切な治療法の選択により，外科的治療にてTSCに伴うてんかん発作を抑制できたとの報告がなされるようになっている^{2,3,5,8-10,12,13)}．今回われわれも，十分な術前の検討により，結節が複数個存在したにもかかわらずsingle resectionにてseizure freeとなった1症例を経験したので文献的考察を加え報告する．

　We report a case of intractable epilepsy caused by tuberous sclerosis with multiple tubers that was treated successfully by single resection of a tuber. A 2-year-old boy developed tonic spasm-like seizures at the age of 4 months and the frequency of the seizures increased despite therapy with various anticonvulsants. He had no neurological deficits, and his intelligence was normal. MRI showed lesions in the left frontal and the right parietal lobe, and EEG (electroencephalogram) demonstrated sharp waves and spike- and- wave complex bursts over the right parietal region. Subdural electrodes were implanted over these lesions, and ictal low-voltage fast waves were demonstrated on the right parietal lesion. Therefore the right cortical lesion and the epileptogenic cortex were resected. Multiple subpial transaction was also added in the irritative cortex. Histological examination proved that the lesion was a tuberous sclerosis complex (TSC) tuber. Since surgery, the patient has remained seizure-free for 2 years. In patients with multiple TSC tubers in the cortex, we suggest that seizures can be suppressed by single resection of a tuber if the tuber can be identified as epileptogenic before surgery.