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Table 2 Staging and amyloid presence in Aβ CP from iCJD associated with Aβ pathology along with cases of sCJD and AD used as controls

From: Iatrogenic Creutzfeldt-Jakob disease with Amyloid-β pathology: an international study

Case number Age (y) Disease durat. (mo) Codon 129 genotype PrPSc type Phase 1 Phase 2 Phase 3-4 Phase 5
Neocortex Thiofl Hippoc. Thiofl Subcortical Thiofl Cerebellum Thiofl
iCJD
 1 (1)a 26 5 na na +++b,c +d ++ - +e - + -
 2 (19) 29 6 MV 1 +++f + + + - nt - nt
 3 (8) 44 18 MV i+2 ++++ + ++ - +++ - - -
 4 (9) 51 14 MM i - - - - - nt + +
 5 (17) 62 2 na na ++++ +g na na na na - -
mean±SD 42±15 9±7    78%h   62.5%   40%   40%  
sCJD
 1 (57)i 63 3 MV 1 +++f + ++ + ++ nt - nt
 2 (59) 71 9 MV 2 +f + - - - nt - nt
 3 (65) 71 10 VV 2 + + - nt - nt - nt
 4 (67) 79 6 VV 2 +++ + - nt - nt - nt
mean±SD 71±6.5 7±3    57%   25%   17%   0%  
P valuej      NS   NS   NS   NS  
AD
 1 56 60 na na ++++ + ++ + +++ + + +
 2 56 60 na na ++++ + ++ + +++ + + +
 3 59 na na na ++++ + ++ + +++ + + +
 4 60 130 na na ++++ + ++ + +++ + + -
 5 70 66 na na ++++ + ++ + +++ + + +
 6 72 111 na na ++++ + ++ + +++ - + +
 7 74 22 na na ++++ + ++ + +++ + + +
mean±SD 64±8 75±39    100%   100%   100%   100%  
P valuek      < 0.02   < 0.04   < 0.0004   < 0.05  
  1. Phases 1 to 5 refer to Aβ plaques deposition in various brain regions reflecting disease progression according to Thal et al [63]; a,iNumbers in parenthesis in the first column refer to numerals used in aTable 1 and iAdditional file 1: Table S2; bMinus and plus signs indicate absence (-) or presence (+) of Aβ pathology affecting one (+), two (++), three (+++) or four (++++) of the brain regions that characterize each phase; Phase 1: frontal, temporal, parietal and occipital cortical regions; Phase 2: hippocampus and entorhinal cortex; Phase 3-4: striatum, thalamus and midbrain; Phase 5: cerebellum; c,eRefers to one iCJD with one or two missing sections from the cneocortex or esubcortical regions; dThe (+) sign under Thiofl (Thioflavin S) indicates presence of amyloid plaques in at least one brain region; the (-) sign indicates that all regions tested had Thioflavin S negative staining; fRefers to one iCJD and two sCJD cases with missing parietal cortex; gPositive staining with Congo red [58]; hPercentage of brain regions with CP; j,kFisher’s exact test comparing iCJD to jsCJD or kAD; Hippoc. hippocampal formation (hippocampus and entorhinal cortex), y years, durat. duration, mo months, na not available, nt not tested, NS not significant, SD standard deviation