Peripheral nerves regenerated in familial amyloid polyneuropathy after liver transplantation

Ann Intern Med. 1997 Oct 15;127(8 Pt 1):618-20. doi: 10.7326/0003-4819-127-8_part_1-199710150-00006.

Abstract

Background: Liver transplantation holds promise as a treatment for familial amyloid polyneuropathy.

Objective: To determine whether peripheral nerves regenerate in patients with familial amyloid polyneuropathy after liver transplantation.

Design: Case report.

Setting: University hospital in Matsumoto, Japan.

Patient: A 34 year-old-women with familial amyloid polyneuropathy who had liver transplantation and showed marked clinical improvement 3 years after surgery.

Measurements: Histopathologic examination and morphometric analysis of biopsy specimens taken from sural nerves.

Results: Diffuse fiber loss and amyloid deposits were seen in a biopsy specimen of the left sural nerve obtained before liver transplantation (total number of myelinated fibers, 1326/mm2 of the endoneurial area). In the biopsy specimen of the right sural nerve, which was obtained 3 years after transplantation, amyloid deposits remained but the number of myelinated fibers was markedly increased (total number of myelinated fibers, 4740/mm2).

Conclusion: Peripheral nerves regenerated in a patient with familial amyloid polyneuropathy after liver transplantation.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Amyloid / metabolism
  • Amyloid Neuropathies / pathology
  • Amyloid Neuropathies / physiopathology*
  • Amyloid Neuropathies / surgery*
  • Female
  • Humans
  • Liver Transplantation*
  • Nerve Regeneration / physiology*
  • Peripheral Nervous System / physiopathology*
  • Sural Nerve / metabolism
  • Sural Nerve / pathology
  • Sural Nerve / physiopathology

Substances

  • Amyloid