Advertisement

Periodic Plasma Exchanges in the Treatment of Familial Amyloid Polyneuropathy: Preliminary Results

  • Paula Coutinho
  • Eugénia Macedo
  • Admar Estácio
  • Albina Saraiva
  • Pedro P. Costa
  • Maria João M. Saraiva
Chapter

Abstract

The presence of an abnormal protein (TTRMet 30) in the plasma and CSF of Familial Amyloid Polyneuropathy (FAP) patients as well as its generalized deposition as amyloid have been thoroughly demonstrated1,2. The next step will be the clarification of its role in the pathogenesis of the peripheral neuropathy. Several hypotheses must be considered:
  1. 1.

    TTRMet 30 interferes directly with the nerve fiber metabolism and its function.

     
  2. 2.

    The decrease of the normal TTR (TTRVal 30) is responsible for nerve fiber degeneration.

     
  3. 3.

    The presence of amyloid causes the neuropathy (but definitely not by a mechanical or vascular effect, as postulated before, otherwise it would correspond to a mononeuropathy multiplex and not to a generalized polyneuropathy).

     

Keywords

Autonomic Neuropathy Neurological Score Familial Amyloid Polyneuropathy Familial Amyloidotic Polyneuropathy Partial Score 
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.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. 1.
    M. J. M. Saraiva, P. P. Costa, and D. S. Goodman, Studies on plasma trans-thyretin (prealbumin) in familial amyloidotic polyneuropathy, Portuguese type, J. Lab. Clin. Med., 102: 590 (1983).Google Scholar
  2. 2.
    P. P. Costa, A. S. Figueira, and Bravo F., Amyloid fibril protein related to prealbumin in familial amyloidotic polyneuropathy, Proc. Natl. Acad.Sci. USA, 75: 4499 (1978).CrossRefGoogle Scholar
  3. 3.
    Therapeutic plasmapheresis — Consensus Conference, JAMA, 256: 1333 (1986).Google Scholar
  4. 4.
    A. Vahlquist, P. A. Peterson, and L. Wibel, Metabolism of the vitamin A transporting protein complex. I-Turnover studies in normal persons and in patients with chronic renal failure, Europ. J. Clin. Invest., 3: 352 (1973).CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 1988

Authors and Affiliations

  • Paula Coutinho
    • 1
  • Eugénia Macedo
    • 1
  • Admar Estácio
    • 2
  • Albina Saraiva
    • 2
  • Pedro P. Costa
    • 3
  • Maria João M. Saraiva
    • 4
  1. 1.Serviço de Neurologia, Hospital Geral de Santo AntónioPortoPortugal
  2. 2.Serviço de HematologiaHospital Geral de Santo AntónioPortoPortugal
  3. 3.Centro de Estudos de ParamiloidosePortoPortugal
  4. 4.Departamento de BioquímicaInstituto de Ciências BiomédicasAbel Salazar, PortoPortugal

Personalised recommendations