Gargoylism脳の電子顕微鏡的所見

An electron microscopic study on Gargoynism

I．序

　Gargoylismは脳Lipoidosisの一型として，臨床のみならず，組織病理学的に，また最近では組織化学的に研究されており，特に黒内障性白痴などの脂質代謝障害との比較において，大きな関心の寄せられている疾患であるが，検索例の少ないために，その知見は不十分であり，病理成因に関連しても，種々の論議がなさてはいるが，いまだ定説の確立しない現状である．電子顕微鏡による検索がこの種の疾患の研究に寄与することは，すでに発表された黒内障性白痴についての報告^{11）12）13）}によつても期待されるが，固定包埋の問題，生検材料などという方法上の制約もあつて，その数はいまだ少なく，殊にGargoylism脳についての報告は，われわれの知る限り，いまだにこれを見ない。

　ここに報告するものは，臨床的に確診されたGargoylism（順天堂大学神経科）についての電顕的所見であるが，脳Lipoidosisについて知られているものと趣きを異にする特殊の所見が少なくなく，各病型間の異同の考察にとつても寄与する点があると考えられるものである．なお本例は組織化学（東大脳研）組織病理学（東大神経科）もくわしく検索されたものであるが，本稿ではわれわれの分担した電顕部門における知見のみを記載するに止める。

　Cooperative studies on the bioptic cerebral tissue from a seven yc ars old male with Gargoylism have been performed in various fields. This report describes electron microscopic observation.

　-Nerve cell A number of granular substances with irregular shapes from 0.5 to 3 microns in diameter, were found in the swollen cytoplasm. These granular substances would be classified into the two types, high electron dense granular substance (H.Gs.) and low electron dense granular substance (L.Gs.). Additionally, there were some transitional type. Mainly, H.Gs. was mingled, and L.Gs. isolated. Among the H.Gs. at least two forms were observed. The first was oval form with many membranous layers in parallel with its short axis. This was unique. The second was round form with membranous layers concentrated, which was similar to the M.C.B. by Terry et al. The main pathological changes in the nerve cell were mingled state of different granular substances with the oval H.Gs. as a core and lesser normal mitochondrien.

　Blood vessels

　The cytoplasm of the swollen pericytes of the capillary was filled by the vacuolens from 1 to 3 microns in diameter. The cytoplasm of the perithelial cells increased up to the Virchow-Robin Space of small vessels was filled also by the similar vacuolens. The nuclei were compressed and deformed by these vacuolens and changed to the high electron density. These vacuolens consisted of low electron dense non structural homogeneous substances.

　This observation is the first report on the Gargoylism using the electron microscope.

　The above particular findings were discussed in comparison with the picture of the cerebral lipoidosis. The chacteristics of the granular substances in the nerve cell suggested that its origin might be the mitochondria. The vacuolens in the vessel would have any relation to the acid mucopolysaccaride. These findings would contribute to the differentiation between the gargoylism and the other lipoidosis.