Ten-year follow-up of peripheral nerve function in patients with familial amyloid polyneuropathy after liver transplantation.

摘要

BACKGROUND : The electrophysiological long-term effects of liver transplantation on peripheral nerve function in patients with familial amyloid polyneuropathy (FAP) have not been evaluated. METHODS : 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)). RESULTS : 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. CONCLUSIONS: Liver transplantation can halt the progression of peripheral neuropathy in FAP patients.