Retrospective study of Creutzfeldt-Jakob disease in Belgium: neuropathological findings

Creutzfeldt-Jakob disease (CJD) is a spongiform encephalopathy that affects about 1 in 10(6) inhabitants in most countries. Recently, a new variant of CJD has been linked to the epidemic of bovine spongiform encephalopathy. T herefore, vigilance concerning the disease's incidence has been increased. We conducted a comprehensive, nation-wide and retrospective study. In 79 Be lgian autopsies, we found the characteristic tried of spongiosis, neuronal loss and reactive gliosis. The occipital cortex was most affected, while th e cerebellum was mostly spared. Immunohistochemistry was performed using hy drated autoclave pretreatment and several monoclonal antibodies directed ag ainst the prion protein. We identified prion-immunoreactive patterns and lo cations reflecting the important heterogeneity, independently of the antibo dy that was used. Granular prion immunoreactivity was observed in astrocyte s. We studied the regioned intensity of the prion immunostaining and determ ined that the frontal cortex with 95% positive immunoreactivity was best su ited for a biopsy. We studied the disease duration in sporadic CJD patients who showed neuropathological lesions of other neurodegenerative disorders (such as Alzheimer's disease). The study shapes the framework in which a pr ospective neuropathological registry will be able to function.