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Table 3 Staging of Aβ CAA in iCJD and control cases of sCJD and AD

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

Case number Age (y) Disease duration (mo) Codon 129 genotype PrPSc
type
Phase 1 Phase 2 Phase 3 Vstl.a score CAAb type
Neocortex Thiofl Hippoc. Thiofl Crbl Thiofl Midb. Subcort. Thiofl   
iCJD
1 (1)c 26 5 na na +++d,e +f ++ + + + na -e - 3 1
2 (19) 29 6 MV 1 +++g + + + + nt + + nt 2 2
3 (16) 32 3.5 MV na +h na - na - na - - na 1 2
4 (8) 44 18 MV i+2 ++++ + ++ - + + - - - 3 2
5 (21) 50 14 MM na +++g + - nt - nt + + nt 1 2
6 (9) 51 14 MM i ++ + - - + + - - - 2 2
7 (10) 54 2 MM 1 + + - - - - - - nt 1 2
8 (17) 62 2 na na ++++ +i na na + +i na na na na na
9 (22) 62 4 VV na ++g - - nt + - + + nt 1 2
10 (23) 71 4 MV na - nt + - + - - - nt 1 2
11 (27) 75 3 VV 2 ++++ - + - + - - - nt 1 2
mean±SD 50.5±17 7±6    69%j   35%   73%   33% 16%    
sCJD
1 (55)k 49 4 MM 1 +g - - - - nt - - nt 1 2
2 (56) 63 4 MM 1 +g - ++ - + - + ++ nt 1 2
3 (57) 63 3 MV 1 - - - - + nt - + nt 1 2
4 (44) 64 1 MM 1 ++ + - - - nt - - - 1 2
5 (58) 71 8 VV 2 +++g - ++ - + - - - nt 1 2
6 (66) 74 4.5 VV 2 + - - nt - nt - - nt 1 2
mean±SD 64±9 4±2    38%   33%   50%   17% 25%    
P valuel      < 0.03   NS   NS   NS NS   = 0.05* NS*
AD
1 56 60 na na ++++ + - - + + + + - 2 2
2 56 60 na na ++++ + - - + + - - - 3 2
3 59 na na na ++++ - - - + + - - - 3 2
4 60 130 na na ++++ - -n - + - - + + 2 2
5 70 66 na na ++++ + + - + + - - - 2 2
6 72 111 na na ++++ + + + + + + + + 3 1
7 74 22 na na +++ - - - + + - + - 3 2
mean±SD 64±8 75±39    96%   15%   100%   29% 29%    
P valuem      < 0.006   NS   NS   NS NS   < 0.02* NS*
  1. Phases 1 to 3 refer to Aβ CAA deposition in various brain regions, reflecting disease progression according to Thal et al [61]; aVonsattel (Vstl) 0-3 score of Aβ CAA severity to the highest score in one or more brain locations [66]; bPresence (type 1) or absence (type 2) of Aβ deposits in the cortical capillaries [60]; c,kNumbers in parenthesis in the first column refer to numerals used in cTable 1 and kAdditional file 1: Table S2; dMinus and plus signs indicate absence (-) or presence (+) of Aβ CAA affecting one (+), two (++), three (+++) or four (++++) brain regions in each phase; Phase 1: frontal, temporal, parietal and occipital cortical regions; Phase 2: hippocampus, entorhinal cortex, cerebellum and midbrain; Phase 3: striatum, thalamus; eRefers to one iCJD patient with one missing section from neocortex and subcortical regions; fThe (+) sign under Thiofl (Thioflavin S) indicates presence of positive staining of at least one brain region; the (-) sign indicates that all regions examined had Thioflavin S negative staining; gRefers to 3 iCJD and 3 sCJD cases with missing parietal cortex; hRefers to one iCJD with missing frontal cortex; iPositive staining with Congo red [58]; jPercentage of brain regions with Aβ CAA; l,mFisher’s exact test comparing iCJD to lsCJD or mAD; nRefers to one AD patient with missing entorhinal cortex; y years, mo months Hippoc. hippocampal formation (hippocampus and entorhinal cortex), Crbl cerebellum, Midb. midbrain, Subcort. subcortical regions (striatum and thalamus), na not available, nt not tested, NS not significant, SD standard deviation; *Student’s t-test