Shimojima Yoshio, Morita Hiroshi, Kobayashi Sachio, Takei Yo-ichi, Ikeda Shu-ichi
Dept. of Medicine (Neurology and Rheumatology), Shinshu University, School of Medicine, Asahi 3-1-1, Matsumoto, 390-8621, Japan.
J Neurol. 2008 Aug;255(8):1220-5. doi: 10.1007/s00415-008-0884-9. Epub 2008 May 20.
The electrophysiological long-term effects of liver transplantation on peripheral nerve function in patients with familial amyloid polyneuropathy (FAP) have not been evaluated.
Eight FAP patients with a proven ATTRVal30Met gene were observed for 10 years after liver transplantation. We performed repeated measurement of maximal motor nerve conduction velocity (MCV), distal latency, size of compound muscle action potential (CMAP) and maximal sensory nerve conduction velocity (SCV) in both the ulnar and tibial nerves. We also recorded the coefficients of variance in the R-R interval on the electrocardiogram (CV(R-R)).
Some autonomic symptoms subsided but motor and sensory symptoms 10 years after transplantation were either slightly improved or almost the same as before surgery in 7 of 8 patients. These 7 have returned to their previous social lives including their jobs. The MCV of the tibial nerve slightly improved, and other parameters of motor and sensory nerve function and CV(R-R) did not show any deterioration during the 10-year observation period.
Liver transplantation can halt the progression of peripheral neuropathy in FAP patients.
肝移植对家族性淀粉样多神经病（FAP）患者周围神经功能的电生理长期影响尚未得到评估。
对8例经证实携带ATTRVal30Met基因的FAP患者在肝移植后进行了10年的观察。我们对尺神经和胫神经的最大运动神经传导速度（MCV）、远端潜伏期、复合肌肉动作电位（CMAP）大小以及最大感觉神经传导速度（SCV）进行了重复测量。我们还记录了心电图R-R间期的变异系数（CV（R-R））。
一些自主神经症状有所缓解，但8例患者中有7例在移植10年后运动和感觉症状略有改善或与手术前几乎相同。这7例患者已恢复到包括工作在内的先前社交生活。在10年观察期内，胫神经的MCV略有改善，运动和感觉神经功能的其他参数以及CV（R-R）均未显示任何恶化。
肝移植可阻止FAP患者周围神经病变的进展。
