Abstract
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 (CVR-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 CVR-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.
Similar content being viewed by others
References
Ikeda S, Nakazato M, Ando Y, Sobue G (2002) Familial transthyretin-type amyloid polyneuropathy in Japan: clinical and genetic heterogeneity. Neurology 58:1001–1007
Connors LH, Lim A, Prokaeva T, Roskens VA, Costello CE (2003) Tabulation of human transthyretin (TTR) variants, 2003. Amyloid 10:160–184
Holmgren G, Steen L, Ekstedt J, Groth CG, Ericzon BG, Eriksson S, Andersen O, Karlberg I, Nordén G, Nakazato M, Hawkins P, Richardson S, Pepys M (1991) Biochemical effect of liver transplantation in two Swedish patients with familial amyloidotic polyneuropathy (FAP-met30). Clin Genet 40:242–246
Bergethon PR, Sabin TD, Lewis D, Simms RW, Cohen AS, Skinner M (1996) Improvement in the polyneuropathy associated with familial amyloid polyneuropathy after liver transplantation. Neurology 47:944–951
Adams D, Samuel D, Goulon-Goeau C, Nakazato M, Costa PM, Feray C, Planté V, Ducot B, Ichai P, Lacroix C, Metral S, Bismuth H, Said G (2000) The course and prognostic factors of familial amyloid polyneuropathy after liver transplantation. Brain 123:1495–1504
Ericzon BG, Larsson M, Herlenius G, Wilczek HE; Familial Amyloidotic Polyneuropathy World Transplant Registry (2003) Report from the Familial Amyloidotic Polyneuropathy World Transplant Registry (FAPWTR) and the Domino Liver Transplant Registry (DLTR). Amyloid 10(Suppl 1):67–76
Parrilla P, Ramirez P, Andreu LF, Bueno SF, Robles R, Miras M, Acosta F, Viader-Farre D, de Mingo P, Bermejo J, Garrido SF, Munar-Ques M (1997) Long-term results of liver transplantation in familial amyloidotic polyneuropathy type I. Transplantation 64:646–649
Monteiro E, Perdigoto R, Furtado AL (1998) Liver transplantation for familial amyloid polyneuropathy. Hepatogastroenterology 45:1375–1380
de Carvalho M, Conceicao I, Bentes C, Luis ML (2002) Long-term quantitative evaluation of liver transplantation in familial amyloid polyneuropathy (Portuguese V30M). Amyloid 9:126–133
Tashima K, Ando Y, Terazaki H, Yoshimatsu S, Suhr OB, Obayashi K, Yamashita T, Ando E, Uchino M, Ando M (1999) Outcome of liver transplantation for transthyretin amyloidosis: follow-up of Japanese familial amyloidotic polyneuropathy patients. J Neurol Sci 171:19–23
Kobayashi S, Morita H, Asawa T, Takei Y, Hashimoto T, Ikegami T, Hashikura Y, Kawasaki S, Ikeda S (2003) Peripheral nerve function in patients with familial amyloid polyneuropathy after liver transplantation. Amyloid 10:17–24
Takei Y, Ikeda S, Hashikura Y, Ikegami T, Kawasaki S (1999) Partial-liver transplantation to treat familial amyloid polyneuropathy: follow-up study of 11 patients. Ann Intern Med 131:592–595
Ikeda S, Nakano T, Yanagisawa N, Nakazato M, Tsukagoshi H (1992) Asymptomatic homozygous gene carrier in a family with type I familial amyloid polyneuropathy. Eur Neurol 32:308
Oh SJ (2007) Neuropathies of the foot. Clin Neurophysiol 118:954–980
Buchthal F, Rosenfalck A (1966) Evoked action potentials and conduction velocity in human sensory nerves. Brain Res 3:1–122
Denys EH (1991) AAEM minimonograph #14: The influence of temperature in clinical neurophysiology. Muscle Nerve 14:795–811
Stangou AJ, Hawkins PN (2004) Liver transplantation in transthyretin-related familial amyloid polyneuropathy. Curr Opin Neurol 17:615–620
Matsunami H, Makuuchi M, Kawasaki S, Hashikura Y, Ikegami T, Nakazawa Y, Miyagawa S, Takei Y, Ikeda S, Yanagisawa N (1995) A case of familial amyloid polyneuropathy treated with partial liver transplantation using a graft from a living related donor. Transplantation 60:301–303
Suhr OB, Ericzon B-G, Friman S (2002) Long-term follow-up of survival of liver transplant recipients with familial amyloid polyneuropathy (Portuguese type). Liver Transpl 8:787–794
Takei Y, Ikeda S, Ikegami T, Hashikura Y, Miyagawa S, Ando Y, Japanese Liver Transplantation Society (2005) Ten years of experience with liver transplantation for familial amyloid polyneuropathy in Japan: outcomes of living donor liver transplantation. Intern Med 44:1151–1156
Suhr OB, Friman S, Ericzon B-G (2005) Early liver transplantation improves familial amyloidotic polyneuropathy patients’ survival. Amyloid 12:233–238
Ikeda S, Yanagisawa N (1987) Clinical findings in familial amyloid polyneuropathy. In: Ohashi M ed. Amyloidosis. Nagoya: Nagoya University Press, pp 199–219
Suhr OB, Wiklund U, Ando Y, Ando E, Olofsson B-O (1997) Impact of liver transplantation on autonomic neuropathy in familial amyloidotic polyneuropathy: an evaluation by spectral analysis of heart rate variability. J Intern Med 242:225–229
Suhr OB, Anan I, Ahlstrom KR, Rydh A (2003) Gastric emptying before and after liver transplantation for familial amyloidotic polyneuropathy, Portuguese type (Val30Met). Amyloid 10:121–126
Holmgren G, Ericzon BG, Groth CG, Steen L, Suhr O, Andersen O, Wallin BG, Seymour A, Richardson S, Hawkins PN, Pepys M (1993) Clinical improvement and amyloid regression after liver transplantation in hereditary transthyretin amyloidosis. Lancet 341:1113–1116
Suhr OB, Holmgren G, Steen L, Wikström L, Norden G, Friman S, Duraj FF, Groth CG, Ericzon BG (1995) Liver transplantation in familial amyloidotic polyneuropathy. Follow-up of the first 20 Swedish patients. Transplantation 60:933–938
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Shimojima, Y., Morita, H., Kobayashi, S. et al. Ten-year follow-up of peripheral nerve function in patients with familial amyloid polyneuropathy after liver transplantation. J Neurol 255, 1220–1225 (2008). https://doi.org/10.1007/s00415-008-0884-9
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00415-008-0884-9