Autoantibodies against ADAMTS13 as detected in most patients with acquired thrombotic thrombocytopenic purpura (TTP), or mutations in the ADAMTS13 gene as found in patients with hereditary TTP are associated with an increased risk of thrombotic occlusion of micro vessels in different organs, including the brain (43). Here, ADAMTS13 is linked to thrombotic thrombocytopenic purpura.