A retrospective study of Creutzfeldt-Jakob disease in Belgium

Using data from Belgian neuropathological archives, completed with the resu lts of a comprehensive study of available medical records, we found 100 pat ients who fulfilled diagnostic criteria for probable or definite Creutzfeld t-Jakob disease (CJD). Mean age at death was 63 years. The median disease d uration was 9 months. Progressive mental deterioration was present in all c ases, whereas signs of cerebellar dysfunction and myoclonus were found in a pproximately 80% of the patients. In 50% of the population, the EEG reveale d characteristic abnormalities. Ninety-six patients suffered from the spora dic type of CJD, while 4 suffered from a hereditary form. In our series, we could find no evidence for the new variant, neither for an iatrogenic caus e.