Mollenhauer B, Zerr I, Ruge D, Krause G, Mehnert WH, Kretzschmar HA, Poser S (2002): Epidemiologie und klinische Symptomatik der Creutzfeldt-Jakob-Krankheit. [Epidemiology and clinical symptomatology of Creutzfeldt-Jakob disease]
Dtsch. Med. Wochenschr. 127: 312-317.

Epidemiology and clinical symptomatic of Creutzfeldt-Jakob disease. OBJECTIVE: Analogous to prospective studies in other countries, prevalance and symptoms of sporadic Creutzfeldt-Jakob disease (CJD) were recorded in order to assess irregularities in the incidence of the disease in Germany since the onset of bovine spongiform encephalopathy (BSE). PATIENTS AND METHODS: Since 1993 all suspected case of CJD reported in the Federal Republic of Germany have been analysed by a unified schema and classified by standardised criteria. In addition to voluntary reporting two other systems were accessed: (1) compulsory reporting to the Robert Koch-Institut via the appropriate Health Department and (2) cause of death statistics of the Federal Office of Statistics. RESULTS: Between June 1993 and May 2001, a total of 1247 patients with suspected CJD, obtained by the "Study of the epidemiology and early diagnosis of human spongiform encephalopathies" at Göttingen University, were examined. The suspected disease was confirmed by autopsy in 404 cases, the diagnosis of probable CJD was made in 369 cases on the basis of clinical data and additional investigation. At the beginning of the Göttingen Study in 1993 the incidence in Germany was 0.7 per mill. population, while in the year 2000 it had risen to 1.3 per mill. population. Corresponding increases in the number of cases since 1993 have been noted also by the Robert Koch-Institut and the Federal Office of Statistics. CONCLUSIONS: The increased incidence can be explained primarily by a decrease in previously unknown cases. Concerted action as part of the Göttingen Study has increased the cooperation of associated clinics. In addition to sporadic cases of CJD, genetic and, more rarely, iatrogenic forms have been seen in Germany. But no cases of new variant CJD have been reported so far.