Amyloidosis

Abstract

Heredofamilial Amyloidoses Primary familial amyloidosis with polyneuropathy (Portuguese type). Perhaps the first clear-cut documentation of a hereditary amyloid syndrome was reported in detail by Andrade⁸² in 1952, with further pathological observations by Silva Horta⁸³ three years later. This disorder is endemic to several areas of Portugal,⁸⁴ where over 200 patients are being followed. Clinically, it is an insidious, usually symmetrical, progressive neuropathy affecting primarily the lower limb (so much so that it has been called "foot disease"). It is inherited as an autosomal dominant. A recent chromosome analysis of 1 patient said to have this type of amyloid showed . . .