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From the Neurological Unit (Drs. Bergethon and Sabin) and the Arthritis Center (Drs. Simms, Cohen, and Skinner), Boston City Hospital, and the Departments of Neurology (Drs. Bergethon and Sabin) and Medicine (Drs. Simms, Cohen, and Skinner), Boston University School of Medicine, Boston, MA; and the Department of Surgery (Dr. Lewis), The New England Deaconess Hospital, Boston, MA.
Supported by grants from the National Institutes of Health (AR40414, AR20613, RR533) and the Boston City Hospital Fund for Excellence.
Received February 22, 1995. Accepted in final form December 8, 1995.
Address correspondence to Dr Bergethon, The Neurological Unit, Boston City Hospital, 818 Harrison Avenue, Boston, MA 02118.
Objective: To study, following liver transplantation, the neurologic progression or regression of the polyneuropathy in a cohort of patients with familial amyloidotic polyneuropathy (FAP). Background: FAP is characterized by the relentless progression of neurologic and cardiac impairment, leading to death within 7 to 15 years after disease onset. No 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 on nine patients who were followed longitudinally with serial neurologic examinations since transplantation. Results: Clinically, all patients evaluated for neurologic progression reported significant improvement in general well being. No patient showed any progression in neurologic disease since receiving a liver transplant. Improvements are documented in symptomatic, autonomic, and sensorimotor neurologic disease in all patients. Conclusion: Our experience suggests that liver transplantation may offer hope for arrest of progression and neurologic improvement in patients with FAP.
NEUROLOGY 1996;47: 944-951
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