遺伝性原発性アミロイドージスの末梢神経および筋肉の病理所見—アミロイド線維の生成機序について

HISTOPATHOLOGICAL CHANGES OF A NERVE AND MUSCLE BIOPSY OF A CASE OF FAMILIAL PRIMARY AMYLOIDOSIS: MECHANISM OF AMYLOID PRODUCTION

I．はじめに

　末梢神経症状を主症状とする遺伝性原発性アミロイドージスには，今日までPortuguese-Japanese家系^1,2）Indiana-Maryland家系^10,13），Iowa家系^24），などが報告されている。これらの症例に関しては臨床症状，腓腹神経の病理組織所見，末梢神経障害の発生病理などの検討が行われている。

　また，アミロイド線維の生成機序については，colla—gen線維との関連性^8,9,19）や血管内皮細胞^5,22），Schwann細胞^5,22），線維芽細胞^5）などの関与についての報告があるが，原因については不明である。

T. I., a male aged 38, had a hereditary primary amyloidosis over four generation in his family history. He had peripheral neuropathy with dis-sociated sensory disturbances in the lower limbs, impotence, gastrointerstial dysfunction and ortho-static hypotention.

N. suralis and M. quadriceps femoralis taken from him were examined by light and electron micro-scopy. N. suralis contained a lot of amyloids re-acting with congo-red in the nerve fibres. Amyloid fibrils were remarkably observed around the blood vessels. They were continuous with the basement membrane of the endotherial cells. A few deposites were observed around the Schwann cell and fibro-blasts. In M. quadriceps femoralis, amyloid like fibrils were noted in the perivascular spaces. Es-pecially, a great deal of amyloid fibrils were con-tinuous with the basement menbranes. From this finding, it might be speculated that the basement membrane may play an important role in the pro-duction of amyloid fibrils.