We reviewed 1233 cases of familial amyloidotic polyneuropathy (FAP) from 489 Portuguese families registered at the Centro de Estudos de Paramiloidose, Porto, Portugal. It was found that in 159 cases, neither parent had shown symptoms of this hereditary dominant form of peripheral neuropathy. These cases appear to form a distinct group, with a later age at onset (mean 45-1 years, SD 12-0) than the group of patients with one affected parent (mean 31-2 years, SD 6-9) and a geographical origin not quite in the areas where the disease is most prevalent.

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