Hereditary Amyloidosis With Polyneuropathy

Abstract. An account, mainly clinical, is given of 18 cases of hereditary amyloidosis with polyneuropathy. Common to all cases were progressive peripheral sensory‐motor nervous disturbances starting in the legs. Gastrointestinal dysfunction with malabsorption occurred frequently, as did various symptoms of autonomic nervous disturbances. Characteristic opacities of the corpus vitreum were found. Various ECG changes occurred, indicative of amyloidotic heart involvement, which also may have contributed to congestive cardiac failure in some of the cases. The value of examining biopsy specimens in polarized light is emphasized. Familial cases of this form of amyloidosis have not been reported from Scandinavia before.