期刊
MUSCLE & NERVE
卷 46, 期 6, 页码 964-970出版社
WILEY-BLACKWELL
DOI: 10.1002/mus.23480
关键词
amyloid; familial amyloid polyneuropathy; liver transplantation; neuropathy; transthyretin
资金
- Ministry of Health, Labor and Welfare
- Ministry of Education, Culture, Sports, Science and Technology of Japan
- Kimi Imai Memorial Foundation for Research of Incurable Neuromuscular Diseases
Introduction: Information related to the long-term follow-up of neuropathy in patients with familial amyloid polyneuropathy after liver transplantation is still scarce. Methods: We describe the neuropathic features of 3 patients with the transthyretin Val30Met mutation. Each patient underwent liver transplantation at an early stage of neuropathy, as indicated by the absence of motor dysfunction and relative preservation of myelinated fibers in sural nerve biopsy specimens. Results: Although the patient with late-onset disease (at age 60 years) presented with the least amount of amyloid deposition, he had neuropathic progression after liver transplantation. An older early-onset (at age 40 years) patient reported a slight exacerbation of both somatic and autonomic neuropathic symptoms 10 years after transplantation. However, the younger early-onset (at age 28 years) patient did not exhibit characteristics suggestive of neuropathy 7 years after transplantation. Conclusion: Aging may determine the progression of neuropathy after liver transplantation. Muscle Nerve 46: 964-970, 2012
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