[Histopathological findings of the nerve and muscles in familial primary amyloidosis, with special reference to the mechanism of amyloid fibril production].
T. I., a male aged 38, had a hereditary primary amyloidosis over four generation in his family history. He had peripheral neuropathy with dissociated sensory disturbances in the lower limbs, impotence, gastrointerstial dysfunction and orthostatic hypotention. N. suralis and M. quadriceps femoralis taken from him were examined by light and electron microscopy. N. suralis contained a lot of amyloids reacting 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 fibroblasts. In M. quadriceps femoralis, amyloid like fibrils were noted in the perivascular spaces. Especially, a great deal of amyloid fibrils were continuous with the basement membranes. From this finding, it might be speculated that the basement membrane may play an important role in the production of amyloid fibrils.
D000328:Adult; D000682:Amyloid / Q000378:metabolism*; D000686:Amyloidosis / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D008297:Male; D009132:Muscles / Q000648:ultrastructure*; D013127:Spinal Nerves / Q000648:ultrastructure*; D013497:Sural Nerve / Q000648:ultrastructure*
D000682:Amyloid