炎症型汎発性硬化症（Schilder病）の同胞剖検例

Two Autopsy Cases of Diffuse Cerebral Sclerosis (Schilder's Disease) in Siblings

I．はじめに

　Schilder病はわが国においては1961年横井^1）が8例について総括的報告をなし，臨床診断上の手がかりと病理組織学的所見を述べ，本邦の症例と欧米のそれに臨床的にも病理組織学的にもほとんど差異は認められないと結論した。現在までおよそ22例の報告があるが，そのなかには家族発生はみられていない^1〜14）。

　いままでの報告からみた場合，Schilder病はつぎのように要約できる^1,15〜21）。

　Sibling cases with Schilder's disease were described. Their psychomotor developments were normal until the onset of the disease. The earliest signs were progressive loss of vision and hearing, declining of intellectual development and gait disturbance. They had asymmetrical pyramidal signs, convulsions, forced laughing and crying. In the terminal stage decorticated rigidity developed with pseudobulbar palsy and transient fever. Duration of the disease was one year and four months in younger brother and one year and one months in elder brother who had the onset of the illness four months after the death of his younger brother.