Table 1.
Demographics and frequency of meeting core diagnostic features across the overall autopsied cohort
| All cases | Primary neuropathological diagnosis | FTLD major molecular class | |||||
|---|---|---|---|---|---|---|---|
| n = 117 | FTLD | AD | ALS | FTLD–Tau | FTLD–TDP | FTLD–FUS | |
| n = 98 | n = 15 | n = 4 | n = 34 | n = 55 | n = 8 | ||
| Male (%) | 83 (71) | 69 (70) | 10 (66.7) | 4 (100) | 26 (76.5) | 35 (63.6) | 7 (87.5) |
| Age of onset, mean (range) | 55 (18–80) | 55.0 (18–73) | 57.3 (34–80) | 55.0 (45–68) | 57.6$ (18–73) | 55.4$ (18–73) | 41.1#,† (26–54) |
| Age at presentation, mean (range) | 60 (29–83) | 60.8 (29–78) | 62.8 (43–83) | 58.3 (47–73) | 64.4$ (53–78) | 60.1$ (44–78) | 45.0#,† (29–55) |
| Age at death, mean (range) | 64.6 (35–83) | 64.2 (35–83) | 68.1 (49–90) | 60.8 (52–74) | 67.8$ (57–83) | 64.5$ (47–80) | 48.3#,† (35–57) |
| Met possible bvFTD criteria | 78, 86 | 84*, 92* | 53*, 60* | 25, 25 | 79, 85 | 86, 95 | 88, 100 |
| Met probable bvFTD criteria | 54, 66 | 64, 74 | 33, 47 | 0, 0 | 58, 64 | 66, 79 | 75, 75 |
| Met definite bvFTD criteria | 21 | 26* | 0* | 0 | 9† | 40# | 0 |
| Early disinhibition | 77, 85 | 83*, 90* | 47*, 60* | 50, 50 | 83, 85 | 82, 91 | 88, 100 |
| Inappropriate | 52, 63 | 55, 65 | 40, 53 | 25, 50 | 56, 65 | 51, 62 | 75, 88 |
| Loss of manners | 52, 64 | 58*, 70* | 27*, 40* | 0, 0 | 56, 65 | 56, 71 | 75, 88 |
| Impulsive | 48, 61 | 54*, 68* | 13*, 20* | 25, 25 | 50, 65 | 51, 67 | 88, 88 |
| Early apathy | 79, 81 | 80, 82 | 67, 73 | 100, 100 | 77, 77 | 80, 84 | 88, 88 |
| Apathy | 79, 85 | 80, 86 | 67, 73 | 100, 100 | 77, 77 | 80, 91 | 88, 88 |
| Inertia | 17, 24 | 17, 25 | 13, 20 | 25, 25 | 9, 12 | 22, 29 | 25, 38 |
| Early loss of sympathy/empathy | 45, 50 | 48, 54 | 40, 40 | 0, 0 | 44, 47 | 47, 56 | 63, 63 |
| Lacking empathy | 33, 39 | 35, 40 | 33, 40 | 0, 0 | 32, 35 | 31, 38 | 63, 65 |
| Lacking warmth | 35, 39 | 40*, 44* | 13*, 13* | 0, 0 | 34, 35 | 42, 47 | 63, 63 |
| Early compulsive behaviour | 63, 74 | 70*, 80* | 33*, 53* | 0, 0 | 68, 79 | 69, 76 | 88, 100 |
| Simple repetitive behaviour | 26, 37 | 30, 42* | 7, 13* | 0, 0 | 21, 35 | 31, 42 | 50, 63 |
| Complex compulsions | 55, 64 | 60, 69 | 33, 53 | 0, 0 | 62, 77 | 55, 60 | 88, 88 |
| Stereotypic speech | 16, 24 | 19, 27 | 0, 13 | 0, 0 | 15, 27 | 18, 22 | 50, 63 |
| Eating behaviour | 63, 80 | 69*, 87* | 40*, 53* | 0, 25 | 65, 82 | 71, 89 | 75, 88 |
| Change in food preference | 50, 68 | 56*, 74* | 27*, 47* | 0, 25 | 50, 68 | 58, 78 | 63, 63 |
| Binge eating | 44, 61 | 48, 65 | 33, 47 | 0, 0 | 53, 74 | 42, 60 | 63, 63 |
| Oral exploration | 8, 13 | 8, 13 | 7, 13 | 0, 0 | 6, 12$ | 7, 9$ | 25, 50#,† |
| Neuropsych pattern | 28, 29 | 29, 30 | 20, 20 | 25, 25 | 37, 37 | 27, 29 | 17, 17 |
| Executive impairment | 86, 89 | 85, 89 | 100, 100 | 50, 50 | 93, 97 | 80, 84 | 86, 86 |
| Memory preserved | 41, 41 | 44, 44 | 20, 20 | 50, 50 | 53, 53 | 41, 41 | 29, 29 |
| Visuospatial preserved | 73, 74 | 75, 76 | 53, 53 | 100, 100 | 73, 73 | 74, 76 | 83, 83 |
Unless otherwise specified, results are formatted as per cent with feature at first evaluation, per cent with feature at any point in disease course. AD = Alzheimer’s disease.
Comparison of primary diagnoses (ALS not included): *significant difference between FTLD and Alzheimers disease.
Comparison of FTLD major molecular classes (FTLD–UPS not included): #significantly different from FTLD–Tau; †significantly different from FTLD–TDP; $significantly different from FTLD–FUS.