Peripheral Nerves Regenerated in Familial Amyloid Polyneuropathy after Liver Transplantation

  1. Shu-ichi Ikeda, MD;
  2. Yo-ichi Takei, MD;
  3. Nobuo Yanagisawa, MD;
  4. Hidetoshi Matsunami, MD;
  5. Yasuhiko Hashikura, MD;
  6. Toshihiko Ikegami, MD; and
  7. Seiji Kawasaki, MD
  1. From Shinshu University School of Medicine, Matsumoto, Japan. Grant Support: In part by a research grant from the Intractable Disease Division, Ministry of Health and Welfare, Primary Amyloidosis Research Committee, Japan, and a Grant-in-Aid for Scientific Research, Ministry of Education, Science and Culture, Japan (No. 07457124). Requests for Reprints: Shu-ichi Ikeda, MD, Department of Medicine (Neurology), Shinshu University School of Medicine, Matsumoto 390, Japan. Current Author Addresses: Drs. Ikeda and Takei: Department of Medicine (Neurology), Shinshu University School of Medicine, Matsumoto 390, Japan. Dr. Yanagisawa: National Institute for Longevity Sciences and National Chuubu Hospital, Obu 474, Aichi, Japan. Dr. Matsunami: Department of Surgery, Matsunami General Hospital, Hashiba 501-61, Gifu, Japan. Drs. Hashikura, Ikegami, and Kawasaki: Department of Surgery, Shinshu University School of Medicine, Matsumoto 390, Japan.

    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 woman 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.

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