Sturge-Weber氏病の不全型2例

Two Cases of Inconplete Sturge-Weber Disease

　母斑の如き皮膚疾患に於て，その病変が他の器官にも発生し一つの纒つた疾患を形成する場合，これ等疾患は母斑症Phakomatosisと名附けられている。母斑症にはブルヌビーユ・プリングル氏母斑症Bourneville-Pringle's Phakomatois （結節性脳硬化症），レクリングハウゼン氏母斑症Recklinghausen's Phakomatosisがあり，スタージユ・ウエーバー氏病，及びクリツペル・ウエーバー氏病も母斑症に準じたものとされている。

　さて1879年Sturge氏は右側の生来性緑内障と同側の火焔状母斑Naevus flammeus及びそれと反対側左上肢の癲癇性痙攣発作を有した少女の例を報告し，その病像から脳の右半球にも顔面と同様の母斑の存在を推測した。それより以前1860年R．Schirmer氏は右顔の火焔状母斑と同側の生来性緑内障と視神経萎縮の合併症を観察したが母斑と脳との関係に関する記述はなかつた。1897年Kalischer氏がはじめてその解剖例を報告し，Sturge氏の推測を証明すると共に，その臨床所見に対し病理解剖学的の根拠を与えた。

Since in 1890 Sturge described the case of a girl with an right-sides facial nevus, right-sides buphthalmos and epileptic convulsions, the rela-tion between the skin and the other organ, par-ticuraly the central nervous system was remar-ked. The Sturge-Weber Syndrome consists of nevus flammeus in the distribution of the tri-geminal nerve ; glaucoma ; convulsive seizures (Jakson's type) and hemiplegia which are con-tralateral to the facial nevus; mental retardati-on; characteristic calcifications which is evide-nced by roentgenologic examination of the skull. The Sturge-Weber syndrome, neurofibromatosis and tuberous sclerosis are grounded upon the same origin, then they are all the results of congenital malformation bearing selectively on the organ of ectodermal derivation. These three diseases are called phakomatosis, which is the term that has been applied to the neuro-cutaneous syndromes. Our two cases were the frust form of the Sturge-Weber syndrome, one had facial nevus, hemiplegia, convulsions of Jacksonia type, shadows of calcification in the X-ray photo of skull and mental retardation, the other, facial nevus, convulsion and mental retardation.