Late-Onset Forms of Familial Amyloid Polyneuropathy (Portuguese Type): A Reappraisal

  • Isabel Ribeiro
  • Paula Coutinho


Familial Amyloid Polyneuropathy (FAP) was classically described1,2 as affecting young adults, aged 20 to 40 years. Yet, for many years, late-onset cases or families were reported3-–. Among them the most important cluster is the Swedish3, comprising 33 families and 60 patients, of which 87% had the first symptoms after 40 and 68% after 50 years. Some of the pedigrees have only late-onset forms, while in others classical and late-onset cases are found. 13% of the cases are isolated. The same biochemical error was demonstrated in a Swedish-American family8 and in the Portuguese cluster10.


Classical Form Familial Amyloid Polyneuropathy Vitreous Opacity Typical Clinical Picture Important Cluster 
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Copyright information

© Springer Science+Business Media New York 1988

Authors and Affiliations

  • Isabel Ribeiro
    • 1
  • Paula Coutinho
    • 2
  1. 1.Serviço de NeurocirurgiaHospital Geral de Santo AntónioPortoPortugal
  2. 2.Serviço de NeurologiaHospital Geral de Santo AntónioPortoPortugal

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