Epidemiological studies on Creutzfeldt-Jakob disease in Poland

The epidemiological, clinical and neuropathological study of Creutzfeldt-Jakob disease (CJD) in Poland was established in 1996. It was preceded by wide, repeated informative action among neurologists and psychiatrists in the whole country. The investigations were partially sponsored by the European Commission (E.C.) as part of the programme Biomed 1. The results obtained by us during the first three years of the study are presented in this paper. In 1996-1998 over 60 probable or possible cases of CJD (or information about them) were referred to our Institute. Neuropathologically typical changes for spongiform encephalopathy were found in 28 cases (among them four cases in laboratories of Medical Schools in Szczecin and Poznań). Neuropathological evaluation was based on paraffin slices stained by H-E, PAS, Bielschowsky, Kanzler-Arendt and Klüver-Barrera methods. In certain cases antibody 3F4 was used. In three patients only clinically probable CJD were diagnosed, since neuropathology was not done. In twenty-five persons, a detailed inquiry form was filled in after the model indicated by the E.C. As the result of processing the whole material, we diagnosed in twenty-four patients a sporadic form of CJD. The remaining case belonged to a family with Gerstmann-Sträussler-Scheinker syndrome. In sporadic CJD cases examined and observed by us no exogenic risk factors for the disease could be detected.