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Peripheral Nerves Regenerated in Familial Amyloid Polyneuropathy after Liver Transplantation

Shu-ichi Ikeda, MD; Yo-ichi Takei, MD; Nobuo Yanagisawa, MD; Hidetoshi Matsunami, MD; Yasuhiko Hashikura, MD; Toshihiko Ikegami, MD; and Seiji Kawasaki, MD
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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.

Copyright ©2004 by the American College of Physicians

Ann Intern Med. 1997;127(8_Part_1):618-620. doi:10.7326/0003-4819-127-8_Part_1-199710150-00006
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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.


Grahic Jump Location
Figure 2.
Diameter-frequency histogram of myelinated fibers in the sural nerves before transplantation (black bars) and after transplantation (white bars).
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Grahic Jump Location
Figure 1.
Histopathologic and morphometric findings in the biopsy specimens obtained from the sural nerves.Left.Middle.

Sural nerve before transplantation. Diffuse fiber loss is seen. Arrowheads indicate a nodular deposit of amyloid. (Original magnification, x140.) Sural nerve after transplantation. Depleted areas of myelinated fibers are less extensive than areas in the left sural nerve. Inset reveals amyloid deposits in the endoneurium after staining with Congo red. (Original magnification, x140.) Right. The area framed in the middle panel at higher magnification. Many small myelinated fibers are present; the arrow indicates a cluster of these fibers. All sections were embedded in epoxy resin and stained with toluidine blue. (Original magnification, x400.) Bars = 50 µm.

Grahic Jump Location




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