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INTRODUCTION. The epidemiological surveillance of human transmissible spongiform encephalopathies, is heavily dependent on diagnostic quality, requires therefore a dynamic health care system able to incorporate new diagnostic tools, and rests on activities within three major observation fields: study of age-specific incidences of the disorder, identification of possible changes in clinico-pathological profile, particularly, of variant Creutzfeldt-Jakob disease, and analysis of incidences among the young population with codon 129VV. Problems for evaluating potentially emerging disorders are determined by increasing awareness of diagnosis, changes in autopsy rates, appropriate use of the 14-3-3 protein test in CSF test and international variations in expected data. DEVELOPMENT. The situation in Spain with increasing incidences among the elderly might reflect the impact of recent changes in use of new diagnostic tests, increasing diagnostic awareness of the clinicians among the elderly and a drop in autopsy rates since 1996. Systematic use of 14-33- test in suspected cases, genotyping, and post-mortem study practice, as well as international collaboration are recommended for optimal surveillance. The importance of compulsory notification by law is debated. Generous notification of suspected cases to Instituto de Salud Carlos III, Centro Nacional de Epidemiologia is kindly requested
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