The national surveillance network for Creutzfeldt-Jakob disease (CJD) and related diseases (Gertsmann-Straussler-Scheinker syndrome and fatal familial insomnia) aims to detect all cases of human subacute transmissible spongiform encephalopathies (TSEs), and more specifically cases of variant CJD (v-CJD) linked to bovine spongiform encephalopathy (BSE), classify them by etiology, describe their epidemiological characteristics, and identify potential risk factors. In 1998, the surveillance network received 459 reports of suspected CJD, 590 in 1999, and 823 in 2000. Since 1992, when the Inserm Unit 360 began monitoring CJD, the ratio of diagnosed CJD cases to reported cases has changed significantly, dropping from 85% in 1993 to 16% in 2000. This epidemiological surveillance shows that, following an increase between 1993 and 1996, the trend in CJD mortality appears to have stabilized. The surveillance system has likely achieved the highest possible level of comprehensiveness. (adapted from the text)