This article outlines the objectives of the national surveillance network for Creutzfeldt-Jakob disease and related diseases: to detect all cases of human subacute transmissible spongiform encephalopathies (TSEs), and more specifically cases of variant CJD linked to bovine spongiform encephalopathy (BSE), classify them by etiology, describe their epidemiological characteristics, and identify potential risk factors. The authors present the epidemiological characteristics of suspected CJD cases reported in 2001, as well as the main characteristics of CJD cases that resulted in death in 2001.