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I.はじめに
結節硬化症(Tuberöse Sklerose)が独立の一疾患として初めてその名称を与えられたのはBourneville (1880)によつてであるが,その後Brückner (1881),Hartdegen(1881), Tedeschi (1884), Scarpatetti (1898)などは本症の特異な病理解剖学的所見に注目し,さらにPelhzzi(1901),Geitlin (1905), Vogt (1908),Kufs (1913), Bielschowsky (1914〜24)1)2), Josephy (1921)7),Pollak(1922), Schob (1925), Meduna (1930)9)などによつてようやく本症の組織学的な諸変化の特徴が明らかにされ,その後も臨床および病理の両側面から多数の精細な論説および報告が蓄積され現在にいたつている。
わが国における本症の剖検例は,佐藤,新井の報告12)以来約34例に達するが,その病因,本態などについてはなお混沌たるものがあり,特に胎生分化,奇形性の形成異常,腫瘍性の増殖などの問題の追究には,本症が好個の材料を提供していることは現在もかわりはない。
Pathological study of a 33-year-old female with typical symptoms of tuberous sclerosis, who died after surgical operation for the kidney tumor, revealed following findings:
1) Histological examination disclosed that the main component of the ventricular tumor was spongioblast. The kidney tumor was angiomyolipoma and the adenoma sebaceum was Winkler's Pringle type.
2) Morphological characteristics of giant cells and dysgenetic cells in the tuberous part of the endbrain were discussed.
3) A group of undeveloped young cells were noted around the blood vessel at the heterotopia in the white matter.
4) Argyrophilic round structureless substances were found in the molecular layer with servere marginal gliosis and as well as in the deep layer of the tuberous cortex by means of Tsujiyama's glial fibre staining.
5) Nuclei and perikarya of giant cells and glias in or near the turberous part of the endbrain were found to be PAS-positive.
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