NR AOTE
AU Glatzel,M.; Abela,E.; Maissen,M.; Aguzzi,A.
TI Extraneural pathological prion protein in sporadic Creutzfeldt-Jakob disease
QU International Conference - Prion diseases: from basic research to intervention concepts - TSE-Forum, 08.10.-10.10.2003, Gasteig, München - Poster session - DG-24
PT Konferenz-Poster
AB
Precise knowledge of the distribution of disease-associated prion protein (PrPsc) in Creutzfeldt-Jakob disease is fundamental for classification and diagnosis of Creutzfeldt-Jakob disease, and for instructing preventive measures against iatrogenic incidents.
We used a highly sensitive detection method to investigate the occurrence of PrPsc in extraneural organs of 36 patients who developed sporadic Creutzfeldt-Jakob disease between 1996 and 2002. Concentration of PrPsc by differential precipitation with sodium phosphotungstate increased the sensitivity of Western blot analysis by up to three orders of magnitude.
PrPsc was present in brain tissue of all patients. Additionally, we found PrPsc in 10/28 spleens and in 8/32 skeletal muscle samples. Three patients had PrPsc in both spleen and muscle. Patients with extraneural PrPsc had significantly longer disease duration; uncommon molecular variants of sporadic Creutzfeldt-Jakob disease were overrepresented.
In sporadic Creutzfeldt-Jakob disease, PrPsc was documented only in the central nervous system and its appendages. Thanks to sensitive techniques, we found extraneural deposition of PrPsc in spleen and muscle of one third of patients. Extraneural PrPsc appears to correlate with long disease duration. PrPsc detection was absolutely specific for Creutzfeldt-Jakob disease, suggesting that muscle biopsy may represent a practical method for obtaining definite ante mortem diagnosis of Creutzfeldt-Jakob disease.
AD Markus Glatzel, Eugenio Abela, Manuela Maissen, Adriano Aguzzi, National Reference Center for Prion Diseases, Switzerland
SP englisch
PO Deutschland