結節性硬化症の痙攣発作に対するForel-H—Tomyの経験

Forel-H-Tomy for the Treatment of Seizures in a Case of Tuberous Sclerosis

I．緒言

　最近私どもは，種々の抗てんかん剤によつては発作を抑制しえず，全身痙攣発作ならびにstatus epilepticusを訴えた結節性硬化症の1例に対してForel-H—tomy （陣内^{14）〜17）}）を行ない，その痙攣発作に著明な効果を得たので報告する。

We have reported a case of tuberous sclerosis hav-ing epileptic seizures, which had been markedly im-proved by Forel-H-tomy. The patient was a 15-year-old girl. At age 4 she had skin lesion of the face which was diagnosed as tuberous sclerosis at the Department of Dermatology. At age 10 she began having seizures consisting of generalized convulsion, starting from left upper extremity. Thereafter, she had the various types of seizures as myoclonic jerk of face, absence and automatism several times a month.

She was admitted to our hospital because of status epilepticus on February 2, 1968. Seizures were strong-ly resistant to any kinds of anticonvulsant such as diphenylhydantoin, phenobarbital, GABOB, and oth-ers. The results of the general physical and neuro-logical examinations were within normal except for the presence of typical adenoma sebaceum at face and hypoactivity of deep tendon reflexes. The skull x-ray films and pneumoencephalogram showed cal-cified lesions located in the left side ventricle, the foramina of Monro, and the third ventricle. The EEG showed slow waves in the right parieto-occipital region, but no paroxysmal abnormalities.

On March 5, 1968, Forel-H-tomy was carried out unilaterally at the right side by Cooper's cryosurgical units. Its target point was 2mm posterior, 3mm ven-tral to the middle of AC-PC line and 8. 5mm lateral to the sagittal line.

During 11 months follwing the surgery, she has had no convulsion, but leaving a few automatisms intact. It agrees with our clinical and experimental data of the Forel-H-tomy which does not have any effectiveness to the temporal lobe epilepsy. This in-tact automatism, which had been associated with spike focus at the right temporal region was com-pletely inhibited by single medication of Tegretol.