Familial amyloid polyneuropathy. Demonstration of prealbumin in a kinship of German/English ancestry with onset in the seventh decade.
A family with autosomal dominant transmitted familial amyloid polyneuropathy residing in Texas is described. Clinically, the prominent sensory and severe autonomic nervous system involvement resembles the Andrade (Portuguese) type I familial amyloid polyneuropathy but is unique in that the age of onset is in the seventh decade in all family members affected to date. Using an immunoperoxidase technique, prealbumin was demonstrated in the amyloid deposits. This finding suggests that this family shares biochemical as well as clinical characteristics consistent with similar kinships with type I familial amyloid polyneuropathy of diverse geographic origin.
D000367:Age Factors; D000368:Aged; D000686:Amyloidosis / Q000235:genetics* / Q000473:pathology; D004739:England / Q000208:ethnology; D005260:Female; D005858:Germany / Q000208:ethnology; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D011115:Polyneuropathies / Q000235:genetics* / Q000473:pathology; D011228:Prealbumin / Q000032:analysis*; D013781:Texas
D011228:Prealbumin