Table 2.
Neuropathological evaluation and semi-quantitative assessment of tau pathology
| Case # | Estimated Braak stage | amyloid beta | TDP-43 | alpha-synuclein | CP13 (p-tau, S202) | MAB359 (ac-tau, K274) | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| ACC | IFG | ERC/Hip | ACC | IFG | ERC/Hip | ACC | IFG | ERC/Hip | ACC | IFG | ERC/Hip | ACC | IFG | ERC/Hip | |||
| TSC | TS 1 | 0 | - | - | - | - | - | - | - | - | - | - | - | - | - | - | - |
| TS 2 | 0 | - | - | - | - | - | - | - | - | - | - | ++ | - | - | - | - | |
| TS 3 | 0 | - | N/A | - | - | N/A | - | - | N/A | - | - | N/A | - | - | N/A | - | |
| TS 4 | 0 | - | - | - | - | - | - | - | - | - | - | - | - | - | - | - | |
| TS 5 | 0 | - | - | - | - | - | - | - | - | - | - | + | - | - | - | - | |
| TS 6 | 0 | - | - | - | - | - | - | - | - | - | - | - | - | + | +++ | +++ | |
| TS 7 | 0 | - | - | - | - | - | - | - | - | - | + | ++ | - | + | ++ | - | |
| TS 8 | 0 | - | - | - | - | - | - | - | - | - | + | - | - | - | - | - | |
| TS 9 | 2 | - | - | - | - | - | - | - | - | - | - | + | + | - | + | + | |
| TS 10 | 2* | - | - | - | - | - | - | - | - | - | + | +++ | +++ | ++ | +++ | +++ | |
| TS 11 | 1 | - | - | - | - | - | - | - | - | - | - | - | + | - | - | + | |
| Epilepsy (non-TSC) | EP 1 | 0 | - | - | - | - | - | - | - | - | - | + | - | - | - | - | - |
| EP 2 | 0 | - | - | - | - | - | - | - | - | - | - | - | - | - | - | - | |
| EP 3 | 0 | - | - | - | - | - | - | - | - | - | - | - | - | - | - | - | |
| EP 4 | 0 | - | - | - | - | - | - | - | - | - | - | - | - | - | - | - | |
| EP 5 | 0 | - | - | - | - | - | - | - | - | - | + | - | - | - | - | - | |
| EP 6 | 0 | - | - | - | - | - | - | - | - | - | - | - | - | - | - | - | |
| EP 7 | 0 | - | - | - | - | - | - | - | - | - | - | - | - | - | - | - | |
| EP 8 | 1 | - | - | - | - | - | - | - | - | - | - | - | ++ | - | - | ++ | |
| EP 9 | 0 | - | - | - | - | - | - | - | - | - | - | - | - | - | - | - | |
| Control | Control 1 | 0 | - | - | - | - | - | - | - | - | - | - | - | - | - | - | - |
| Control 2 | 0 | - | - | - | - | - | - | - | - | - | - | - | - | - | - | - | |
| Control 3 | 0 | - | - | - | - | - | - | - | - | - | - | - | + | - | - | - | |
| Control 4 | 0 | - | - | - | - | - | - | - | - | - | - | - | - | - | - | - | |
| Control 5 | 0 | - | - | - | - | - | - | - | - | - | + | - | - | + | - | - | |
| Control 6 | 0 | - | - | - | - | - | - | - | - | - | - | - | - | - | - | - | |
| Control 7 | 0 | - | - | - | - | - | - | - | - | - | - | - | - | - | - | - | |
| Control 8 | 0 | - | - | - | - | - | - | - | - | - | - | - | - | - | - | - | |
| Control 9 | 1 | - | - | - | - | - | - | - | - | - | - | - | + | - | - | - | |
| Control 10 | 2 | - | - | - | - | - | - | - | - | - | - | + | ++ | - | - | - | |
Semi-quantitative ratings of immunoreactivity were observed using a 10 objective across the entire region on a single section and designated as - (absent), + (1–5 tangles), ++ (5–15 tangles), or +++ (> 15 tangles). ACC, anterior cingulate cortex; ERC, entorhinal cortex; Hip, Hippocampus; IFG, inferior frontal gyrus; N/A, not assessed.
*
Braak stage assessment is tentative given the well-developed TSC tauopathy in this case; the NFTs seen throughout the limbic system and basal temporal areas were not accompanied by prominent neuropil threads, as would be expected in AD-type neurofibrillary tauopathy; therefore, the true Braak stage may be lower.