IMPROVEMENT IN THE POLYNEUROPATHY ASSOCIATED WITH FAMILIAL AMYLOID POLYNEUROPATHY AFTER LIVER-TRANSPLANTATION

Objective: To study, following liver transplantation, the neurologic p rogression or regression of the polyneuropathy in a cohort of patients with familial amyloidotic polyneuropathy (FAP), Background: FAP is ch aracterized by the relentless progression of neurologic and cardiac im pairment, leading to death within 7 to 15 years after disease onset. N o effective treatment to slow or halt the progression of this disease has been found to date. Design/Methods: Over the past 3 years, our FAP patients were offered liver transplantation as treatment, We report o n nine patients who were followed longitudinally with serial neurologi c examinations since transplantation. Results: Clinically, all patient s evaluated for neurologic progression reported significant improvemen t in general well being. No patient showed any progression in neurolog ic disease since receiving a liver transplant. Improvements are docume nted in symptomatic, autonomic, and sensorimotor neurologic disease in all patients. Conclusion: Our experience suggests that liver transpla ntation may offer hope for arrest of progression and neurologic improv ement in patients with FAP.