Polyneuritic Amyloidosis in a Japanese Family

Abstract

AMYLOIDOSIS, a rare disease of unknown etiology, is characterized by deposition of amorphous hyaline-like material in connective tissues and by slow involvement of various organs. The clinical manifestations depend upon the rapidity of the involvements and the location of the lesions. The primary type of amyloidosis affects mainly tissues of mesodermal origin, especially the heart, tongue, lungs, striated muscle, gastrointestinal tract, and blood vessels; death is usually due to the cardiac changes. Nervous system involvement in sporadic cases of primary amyloidosis has been reported by several authors who found that the peripheral nerve deposits were prominent, but brain and spinal cord were usually unaffected. 1,2 In 1950 Ostertag 3 first reported a familial form of primary amyloidosis. Subsequently there were several reports of similar families 4-8 in which peripheral neuropathy occurred in 93% of the cases reported. In contrast, neuropathy is uncommon in sporadic cases. 7 In Japan, reports