Author/Editor     Bentes, C; Conceicao, I; de Carvalho, M; Alves, M; Sales-Luis, ML
Title     Liver transplantation in familial amyloidotic polyneurophaty (FAP) type I. Clinical and electromyographic evaluation
Type     članek
Source     In: Stalberg EV, de Weerd AW, Zidar J, editors. ECCN 98. 9th European congress of clinical neurophysiology; 1998 Jun 4-7; Ljubljana. Bologna: Monduzzi editore,
Publication year     1998
Volume     str. 675-8
Language     eng
Abstract     Liver transplantation is the only treatment that have shown some efficacy on FAP patients. However, the evolution of clinical and EMG parameters after surgery is still scarcely reported. We studied 25 patients submitted to liver transplantation between 1992-97, with the aim of periodically evaluating clinical and EMG scores after the procedure, comparing with previous ones. Patients (14 females and 11 males), mean age 41.9 years (30 to 53), with a mean time of symptomatic disease of 6.6 years (1 to 15) and a mean follow-up time of 1.7 years (0.5-4.8) have had clinical and EMG evaluations each 6 months. The mortality rate was 31% Patients who died had higher clinical and EMG scores before surgery than those who survive. Clinical scores showed early improvement than EMG scores which improved only after the second year post surgery. This last evidence can be interpreted as the natural evolution of a chronic, axonal polyneuropathy which recovery depends on reinervation.
Descriptors     LIVER TRANSPLANTATION
AMYLOID NEUROPATHIES
ELECTROMYOGRAPHY
CAUSE OF DEATH