Table 3.
Comparison between mixed and pure pathology of FTLD
| Mixed FTLD-TDP Type A with FTLD-Tau | Pure FTLD-TDP, Type A (n=10) | Mixed FTLD-TDP, type B with FTLD-Tau | Pure FTLD-TDP, type B (n=16) | Mixed FTLD-TDP, type C with FTLD-Tau | Pure FTLD-TDP, type C (n=14) | Mixed FTLD-Tau, CBD with FTLD-TDP (n=4) | Pure FTLD-Tau, CBD (n=17) | |||
|---|---|---|---|---|---|---|---|---|---|---|
| Case 1 | Case 2 | Case 3 | Case 4 | Case 5 | ||||||
| Age at death SD (years) | 70 | 71.0±5.7 | 77 | 69 | 60.6±8.0 | 76 | 85 | 69.3±4.2 | 67.0±5.9 | 66.1± 5.2 |
| Onset age SD (years) | 62 | 62.5±5.0 | 57 | 65 | 51.8±11.5 | 71 | 77 | 54.8±6.8 | 59.0±5.7 | 58.6± 5.9 |
| Disease duration SD (years) | 8 | 8.5±1.9 | 20 | 4 | 8.8±7.0 | 5 | 8 | 14.5±3.9 | 7.0±2.8 | 7.5± 4.2 |
| Sex ratio (Male/Female) | Male | 4:6 | Male | Female | 8:8 | Male | Male | 9:5 | 2:2 | 7:10 |
| Education (years) | 17 | 16.4±2.8 | 14 | 18 | 16.2±2.9 | 20 | 16 | 16.1±3.6 | 17.0±0.7 | 16.2 ± 2.5 |
| APOE ε4 allele frequency* | ε3/ε3 | 0% (0/8) | ε3/ε3 | ε3/ε4 | 29% (4/14) | - | ε3/ε3 | 7% (1/14) | 67% (2/3) | 13% (2/15) |
| Clinical syndrome | bvFTD | 3 bvFTD 4 CBS 2 AD dementia 1 AD dementia, PSP | bvFTD-MND | svPPA | 6 bvFTD 2 ALS 6 bvFTD-MND 1 AD dementia 1 nfvPPA | svPPA | svPPA | 14 svPPA | 1 CBS 2 nfvPPA 1 bvFTD | 7 CBS 2 PSPS 3 bvFTD 5 nfvPPA |
Abbreviations. AD: Alzheimer’s disease, ALS: amyotrophic lateral sclerosis, APOE: apolipoprotein E, bvFTD: behavioral variant of frontotemporal dementia, CBD: corticobasal degeneration, CBS: corticobasal syndrome, FTLD: frontotemporal lobar degeneration, MND: motor neuron disease, nfvPPA: nonfluent variant of primary progressive aphasia, PSPS: progressive supranuclear palsy syndrome, SD: standard deviation, svPPA: semantic variant of primary progressive aphasia, TDP: TAR-DNA binding protein 43
*
the number of subjects who bore at least one copy of the APOE ε4