Table 1.
Subject demographics
| Case no. | Clinical Dx | Primary Neuropathological Dx | MAPT variant | Age/Sex | Symptom duration (yr) | PMI (hr) | Thal phase | Braak stage | CERAD score | ADNC | CDR score |
|---|---|---|---|---|---|---|---|---|---|---|---|
| FTLD-tau/MAPT | |||||||||||
| 1 | bvFTD/PSP-RS | FTLD-tau/MAPT | IVS10 + 16 C > T | 60/M | 8 | 12.5 | ND | 1 | absent | Low | 2a |
| 2 | bvFTD | FTLD-tau/MAPT | IVS10 + 16 C > T | 60/M | 13 | 15.8 | 1 | 0 | absent | Low | 3b |
| 3 | bvFTD | FTLD-tau/MAPT | IVS10 + 16 C > T | 63/F | 17 | 19.7 | 1 | 0 | absent | Low | 3 |
| 4 | bvFTD | FTLD-tau/MAPT | P301L | 59/F | 4 | 24.2 | 1 | 1 | absent | Low | 2 |
| 5 | bvFTD | FTLD-tau/MAPT | P301L | 75/M | 9 | 8.6 | 1 | 2 | absent | Low | 3 |
| 6 | bvFTD | FTLD-tau/MAPT | V337 M | 68/F | 13 | 18.0 | 0 | 1 | absent | Not | 3 |
| 7 | PSP-RS | PSP | A152T | 56/F | 6 | 5.7 | 2 | 2 | absent | Low | 3 |
| 8 | nfvPPA | CBD | A152T | 63/M | 10 | > 48 h | 1 | 3 | absent | Low | 1 |
| Average | 63 | 10 | 15 | 1 | 1 | 2.5 | |||||
| Sporadic bvFTD-PiD | |||||||||||
| 1 | bvFTD | PiD | None | 60/F | 6 | 15.1 | 2–3 | 1 | moderate | Low | 3c |
| 2 | bvFTD | PiD | None | 57/M | 6 | 21.7 | 0 | 0 | absent | Not | 2d |
| 3 | bvFTD | PiD | None | 70/M | 10 | 10.2 | 2 | 0 | moderate | Low | n/a |
| 4 | bvFTD | PiD | None | 72/M | 9 | 24.3 | 0 | 0 | absent | Not | 3 |
| 5 | bvFTD | PiD | None | 69/F | 13 | 6.3 | 0 | 2 | absent | Not | 3 |
| 6 | bvFTD | PiD | None | 76/F | 13 | 25.5 | 0 | 0 | absent | Not | 3 |
| 7 | bvFTD | PiD | None | 57/M | 13 | 12.4 | 1 | 1 | absent | Low | 3 |
| Average | 66 | 10 | 17 | 1 | 1 | 2.8 | |||||
aantemortem, 3 months prior to death, bantemortem, 20 months prior to death, cantemortem, 18 months prior to death, dantemortem, 7 months prior to death, diagnosis (Dx), behvaioral variant FTD (bvFTD), progressive supranuclear palsy-Richardson syndrome (PSP-RS), nonfluent variant primary progressive aphasia (nfvPPA), corticobasal degeneration (CBD), Pick’s disease (PiD), Postmortem interval (PMI)