Fabry''s disease, a rare X-linked disorder of glycosphingolipid metabolism, can present as an insidious dementia in middle or later life. This genetic disorder produces a deficiency of α-galactosidase A which results in the deposition of glycosphingolipids in blood vessel walls in the brain as well as in the kidney, heart, peripheral nerves, and other organs. Among the cerebrovascular manifestations of this disorder is a vascular dementia from involvement of multiple small penetrating blood vessels. Fabry''s disease is a consideration in the work-up of an otherwise unexplained vascular dementia, particularly in males less than 65 years of age.

and shocks) post replacement. A total of 1,483 citations were screened and 16 nonrandomized studies were included. Descriptive analysis revealed major complications as reoperation due to infection (median 1.7%; range 0.7%-2.5%; n¼5 studies), reoperation due to hematoma (median 0.6%; range 0.0%-1.5%; n¼8); and reoperation for other reasons such as lead damage, pain, and pocket erosion (median 1.6%; range 0.1%-2.8%; n¼10). Minor complications were incisional infection (median 0.9%; range 0.01%-1.8%; n¼6) and pocket hematoma (median 0.9%; range 0.4%-3.5%; n¼5). Within 30AE24 months of replacement, 27% of patients received appropriate ICD therapies (range 11%-38%; n¼5) and 8% of patients received inappropriate therapies (n¼1). There is a small but significant risk of complications with ICD replacement. The competing risks and potential life-saving benefit should be addressed with patients when presenting the option of ICD replacement to achieve an informed high quality decision based on personal preferences.