Quantitative study of spongiform change in putamen of 24 cases of Creutzfeldt-Jakob disease.
Creutzfeldt-Jakob disease (CJD) is characterized by 4 main neuropathological lesions: spongiform change, neuronal loss, astrocytic gliosis, and accumulation of pathological prion protein (PrPsc), which is partially protease-resistant (PrPres). This study focused on spongiform change (SC) in the putamen. Because SC varies from case to case, we investigated whether its quantification could provide relevant criteria to discriminate types of PrPres in CJD. SC was quantified in 24 CJD cases, 12 with PrPres type 1 (CJD-PrP1) and 12 with PrPres type 2 (CJD-PrP2), compared to 25 control cases. The study was performed by direct microscopy examination (DME) and by semiautomatic quantification (SAQ) using shape and size criteria previously described. These criteria were suitable for SC quantification in putamen in the majority of cases, except for those with microspongiosis. The results obtained by DME and SAQ methods were correlated and SC scores were compared to the types of PrPres. Sporadic CJD cases with PrPres type 2 were more affected by SC than type 1, suggesting that putamen could be a preferential site to distinguish type 1 from type 2 histologically. The origin of the difference in SC intensity according to the type of PrPres is discussed in terms of host and strain factors.
Truchot
Lydie
L
Centre de Diagnostic de Traitement et de Prévention des Maladies à Prions, Hôpital Neurologique Pierre Wertheimer, Laboratoire de Biochimie et Laboratoire de Neuropathologie, Lyon, France.
Bencsik
Anna
A
Perret-Liaudet
Armand
A
Biacabe
Anne-Gaëlle
AG
Richard
Marlène
M
Ironside
James
J
Kopp
Nicolas
N
Streichenberger
Nathalie
N
D007562:Creutzfeldt-Jakob Syndrome / Q000473:pathology*; D006801:Humans; D007091:Image Processing, Computer-Assisted; D009474:Neurons / Q000473:pathology; D018620:PrPSc Proteins / Q000235:genetics / Q000378:metabolism; D017096:Prion Diseases / Q000473:pathology; D011699:Putamen / Q000473:pathology*; D012189:Retrospective Studies; D014617:Vacuoles / Q000473:pathology
D018620:PrPSc Proteins