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

  • Isabel Ribeiro
  • Paula Coutinho
Chapter

Abstract

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.

Keywords

Classical Form Familial Amyloid Polyneuropathy Vitreous Opacity Typical Clinical Picture Important Cluster 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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References

  1. 1.
    C. Andrade, A peculiar form of peripheral neuropathy, Brain, 75: 408 (1952).CrossRefGoogle Scholar
  2. 2.
    P. Coutinho, A. Martins da Silva, J. Lopes Lima, and A. Resende Barbosa, Forty years of experience with Type I Amyloid Neuropathy (review of 483 cases), in: “Amyloid and Amyloidosis”, G. G. Glenner, P. Pinho e Costa & Falcão de Freitas, eds., Excerpta Medica, Amsterdam (1980).Google Scholar
  3. 3.
    R. Andersson, Familial Amyloidosis with polyneuropathy, Acta Med. Scand. (Suppl), 590: 85 (1976).Google Scholar
  4. 4.
    A. Zalin, A. Darby, S. Vaughan, and E. B. Raftery. Primary neuropathic amyloidosis in three brothers, Br. Med. J., 1: 65 (1974).CrossRefGoogle Scholar
  5. 5.
    C. A. Libbey, A. Rubinow, T. Shirahama, C. Deal, and A. S. Cohen, Familial Amyloid Polyneuropathy. Demonstration of prealbumin in a kinship of German/English ancestry with onset in the seventh decade, Amer. J. Med., 76: 18 (1984).CrossRefGoogle Scholar
  6. 6.
    J. Sequeiros and M. J. M. Saraiva, A family with late-onset atypical form of hereditary amyloid neuropathy, type I (Portuguese, Andrade), Am. J. Hum. Gen., 37: A 76 (1985).Google Scholar
  7. 7.
    F. Giangaspero, F. Salvi, C. Ceccarelli, G. Ambrosetto, E. Govoni, and C. A. Tassinari, Familial amyloid polyneuropathy: report of a family, Clin. Neuropathol., 4: 105 (1985).Google Scholar
  8. 8.
    M. D. Benson and F. E. Dwulet, Identification of a new aminoacid substitution in plasma prealbumin associated with hereditary amyloidosis, Clin. Res., 33: 590A (1985).Google Scholar
  9. 9.
    A. Bastos Lima and A. Martins da Silva, Clinical evaluation of late-onset cases in type I (Andrade) amyloid neuropathy, in: “Amyloid and Amyloidosis”, G. G. Glenner, P. Pinho e Costa & Falcão de Freitas, eds., Excerpta Medica, Amsterdam (1980).Google Scholar
  10. 10.
    M. J. M. Saraiva, P. P. Costa, and D. S. Goodman, Studies on plasma transthyretin (prealbumin) in familial amyloidotic polyneuropathy, Portuguese type, J. Lab. Clin. Med., 102: 590 (1983).Google Scholar
  11. 11.
    M. J. M. Saraiva, P. P. Costa, and D. S. Goodman, Genetic expression of a transthyretin mutation in typical and late-onset Portuguese families with familial amyloidotic polyneuropathy, Neurology, 36: 1413 (1986).CrossRefGoogle Scholar

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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