Peripheral Nerves Regenerated in Familial Amyloid Polyneuropathy after Liver Transplantation

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.