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
| 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 | ||||||||
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