Late-Onset Forms of Familial Amyloid Polyneuropathy (Portuguese Type): A Reappraisal
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.
KeywordsClassical Form Familial Amyloid Polyneuropathy Vitreous Opacity Typical Clinical Picture Important Cluster
Unable to display preview. Download preview PDF.
- 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.R. Andersson, Familial Amyloidosis with polyneuropathy, Acta Med. Scand. (Suppl), 590: 85 (1976).Google Scholar
- 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.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.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.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.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