Sudden death in elderly women with Fabry's disease

We report two cases of elderly women who died unexpectedly. In both cases, light- and electron-microscopical examination proved a diagnosis of Fabry's disease. The major pathological changes were myocardial mottling, foamy vacuolation of myocytes and glomerular endothelial cells, and characteristic lamellar inclusions. Neither woman had either a personal or family history of Fabry's disease. Fabry's disease presenting as sudden death is very rare. We present the findings in two cases together with a review of literature particularly relevant to the cardiovascular system.