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

• Published in: Acta neuropathologica 2000-04, Vol.99 (4), p.358-364
• Main Authors: VAN EVERBROECK, B, PALS, P, DZIEDZIC, T, DOM, R, GODFRAIND, C, SCIOT, R, BRUCHER, J. M, MARTIN, J. J, CRAS, P
• Format: Article
• Language: English
• Subjects: Alzheimer's disease; Amyloid beta-Peptides - analysis; Astrocytes; Belgium; Biological and medical sciences; Biopsy; Bovine spongiform encephalopathy; Brain - pathology; Cerebellum; Cortex (frontal); Creutzfeldt-Jakob disease; Creutzfeldt-Jakob Syndrome - pathology; Gliosis; Humans; Immunohistochemistry; Malformations of the nervous system; Medical sciences; Monoclonal antibodies; Nerve Tissue Proteins - analysis; Neurodegenerative diseases; Neurofibrillary Tangles - pathology; Neurology; Neuropathology; Occipital lobe; Prion protein; PrPC Proteins - analysis; Retrospective Studies; Synucleins; Ubiquitins - analysis; Vigilance
• ISSN: 0001-6322; 1432-0533
• DOI: 10.1007/s004010051136

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. Therefore, vigilance concerning the disease's incidence has been increased. We conducted a comprehensive, nation-wide and retrospective study. In 79 Belgian autopsies, we found the characteristic triad of spongiosis, neuronal loss and reactive gliosis. The occipital cortex was most affected, while the cerebellum was mostly spared. Immunohistochemistry was performed using hydrated autoclave pretreatment and several monoclonal antibodies directed against the prion protein. We identified prion-immunoreactive patterns and locations reflecting the important heterogeneity, independently of the antibody that was used. Granular prion immunoreactivity was observed in astrocytes. We studied the regional intensity of the prion immunostaining and determined that the frontal cortex with 95% positive immunoreactivity was best suited 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 prospective neuropathological registry will be able to function.