結節性脳硬化症の3例

Three cases of Tuberous Sclerosis (Bournevillés Disease)

緒言

　結節性脳硬化症は1880年Bournevilleにより始めて記載された比較的稀な疾患で，定型的なものは，①顔面皮膚に発生する特有な小腫瘤，②精神発育不良，③癲癇様痙攣発作，の3主徴を有するが不全型も亦多い。而も本疾患は皮膚神経系のみならず，発生学上外胚葉・中胚葉に属する内臓諸器官にも病変が見られ，心臓・腎臓・眼底・骨諸粘膜に変化を見る事が多い。

　本邦に於いては，大正5年佐藤氏による10歳女子の報告以来現在迄に300例近くの報告がある。本症は精神科からは結節性脳硬化症として発表され，皮膚科からはBourneville-Pringle氏病として報告多く，又眼科方面からも眼症状を主とした発表がある。我々は最近巨大なグラービッツ腫瘍を併発した39歳の女，その娘である19歳の女子及び17歳男子の3症例を経験したので報告する。

We report the follow three cases of tuber-ous sclerosis (Bourneville's Disease).

Case 1: A 39-year-old female.She has these three chief symptoms: (1) numerous brown small tumors distribute in the skin of face, (2) the intellectual defect, and (3) epilepti-form cramp.

In the course of treatment, she suffered from tumor of left kidney. This tumor was extirpated, and microscopically proved so-ca-lled Gramitz's tumor. It was 2.15kg. in wei-ght and measures 18cm. in diameter. Now, the patient is healthy.

This case of tuberous sclerosis with gigan-tic Grawitz's tumor is the first case in Ja-pan.

Case 2: A 19 year-old female.

A daughter of the former waman. This ca-se has three chief symptoms and tuo retinal tumors in the left eye. By trepanation, nu-merous sharply circumscribed sclerotic nodu-les were found in the cerebral cortex. The findings proved in microscopically were as follows: (1) proliferation of the glia cells and fibers. (2) large ganglion cells, and (3) atro-phy and ruin of the ganglion cells.

Case 3: A 17-year-old male.