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Transthyretin amyloidosis is a relatively uncommon hereditary cause of cerebral amyloid angiopathy (CAA) characterized by amyloidogenic transthyretin deposition in cerebral and leptomeningeal blood vessels. Transthyretin is synthesized mainly by the liver but also by the choroid plexus. To evaluate the effectiveness of liver transplantation on CAA associated with amyloidogenic transthyretin, researchers assessed the clinical courses of eight patients who had the amyloidogenic transthyretin Tyr114Cys mutation and related CNS symptoms. Three patients underwent living-donor liver transplantation, and five (cousins of the first three) did not.
Compared with the no-transplant group, the transplant group had significantly lower mortality. All of t…