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Effect of liver transplantation on transthyretin Tyr114Cys-related cerebral amyloid angiopathy

From the Departments of Neurology (T.Y., M. Ueda, S.O., T.H., A.U., Y.M., M. Uchion), Diagnostic Medicine (Y.A., K.O.), Pediatric Surgery and Transplantation (M.E.Z., H.I.), and Diagnostic Radiology (T.H.), Graduate School of Medical Sciences, Kumamoto University; and National Institute for Minamata Research Center (M.N.), Minamata, Japan.

Address correspondence and reprint requests to Dr. Taro Yamashita, Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, 1-1-1 Honjo, Kumamoto 860-8556, Japan taro-yamashita{at}fc.kuh.kumamoto-u.ac.jp

Objective: Patients with amyloidogenic transthyretin (ATTR) Tyr114Cys develop amyloid deposits in cerebral blood vessels, cerebral hemorrhage, and rapidly progressive dementia that presents with hereditary cerebral amyloid angiopathy (CAA). However, no treatment has been identified for CAA. Although liver transplantation has become an acceptable treatment of TTR-related amyloidosis, liver transplantation may not successfully treat CNS manifestations of the disorder. In this study, we examined the effect of liver transplantation on these manifestations of TTR-related CAA.

Methods: We compared clinical courses of three patients with CAA associated with ATTR Tyr114Cys who underwent liver transplantation with those of five patients with the disorder who did not undergo liver transplantation.

Results: The mortality and occurrence of cerebral hemorrhage and dementia in patients having transplantations were reduced compared with those in patients not having transplantations. The two groups did not differ with regard to the frequency of episodes of fluctuating consciousness and TIAs. The group undergoing transplantations had significantly smaller volumes of intracranial hemorrhage than did the no-transplantation group.

Conclusion: Liver transplantation was effective for CNS manifestations of cerebral amyloid angiopathy associated with amyloidogenic transthyretin Tyr114Cys.

Abbreviations: ATTR = amyloidogenic transthyretin; CAA = cerebral amyloid angiopathy; FAP = familial amyloidotic polyneuropathy; TTR = transthyretin.

Disclosure: The authors report no conflicts of interest.

Received February 20, 2007. Accepted in final form June 25, 2007.

This article has been cited by other articles:

				Can Liver Transplantation Treat Transthyretin-Related Cerebral Amyloid Angiopathy? Journal Watch Neurology, February 19, 2008; 2008(219): 1 - 1. [Full Text]