title: Quantitative study of spongiform change in putamen of 24 cases of Creutzfeldt-Jakob disease.
abstract: 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.
authors.affiliation: 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.
authors.forename: Lydie
authors.initials: L
authors.lastname: Truchot
authors.forename: Anna
authors.initials: A
authors.lastname: Bencsik
authors.forename: Armand
authors.initials: A
authors.lastname: Perret-Liaudet
authors.forename: Anne-Gaëlle
authors.initials: AG
authors.lastname: Biacabe
authors.forename: Marlène
authors.initials: M
authors.lastname: Richard
authors.forename: James
authors.initials: J
authors.lastname: Ironside
authors.forename: Nicolas
authors.initials: N
authors.lastname: Kopp
authors.forename: Nathalie
authors.initials: N
authors.lastname: Streichenberger
mesh_terms: 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
chemical_list: D018620:PrPSc Proteins