Phacomatosis Bourneville-Pringleの1例

A Case of Phacomatosis Bourneville-Pringle

　顔面の皮脂腺腫，並に特異な網膜扁平腫瘍を呈する眼底変状，加えて癲癇発作，知能発育障害等を兼ね備えたPhacomatosis Bourneville-Pringleの1例を経験したので追加報告する。

A case of phacomatosis Bourneville-Pringle in 20 years old female was reported. The patient's intelligence was equivalent to 7 years old. Episodes of epileptiform convulsion repeatedly that in occurred since 5 years old. She had organic nevus, symmetrical multiple small nodules (the so-called adenoma sebaceum), on her face. Its histological pictures were hyperplasia and hypertrophy of sebaceous gland, follicular cyst formation and telangiectasis.

A flat tumor in the retina with white sheaths around the vessels was found in the upper nasal region of the fundus near the optic nerve of the left eye.