| Pathology . | n (%) . | Gender, % male . | Age at clinical onset, mean (SD) (years) . | Age at death, mean (SD) (years) . | Duration, mean (SD) (years) . | Clinical presentation . | |||||
|---|---|---|---|---|---|---|---|---|---|---|---|
| . | . | . | . | . | . | Behavioural variant FTD . | FTD/MND . | Semantic dementia . | PNFA . | Corticobasal syndrome . | PSPS . |
| Tau | 42 (44) | ||||||||||
| Pick’s disease | 13 (14) | 69.2 | 55.4 (5.8) | 69.2 (3.5)a | 12.0 (4.3)a | 6 | 0 | 4 | 3 | 0 | 0 |
| MAPT mutations | 14 (15) | 64.3 | 50.4 (8.6) | 62.6 (10.4) | 12.1 (4.3) | 12 | 0 | 0 | 0 | 2 | 0 |
| Corticobasal degeneration | 9 (9) | 55.6 | 58.2 (9.0) | 67.8 (7.8)b | 8.3 (2.2)b | 3 | 0 | 0 | 4 | 1 | 1 |
| Progressive supranuclear palsy | 6 (6) | 28.6 | 59.0 (5.2) | 69.8 (36) | 10.0 (1.9) | 0 | 0 | 0 | 0 | 1 | 5 |
| TDP-43 | 48 (51) | ||||||||||
| A | 25 (26) (9 GRN) | 60.0 | 58.3 (6.9) | 65.3 (8.2) | 6.4 (2.5) | 20 | 1 | 0 | 3 | 1 | 0 |
| B | 3 (3) | 33.3 | 55.7 (6.7) | 60.8 (5.7) | 5.2 (1.0) | 0 | 3 | 0 | 0 | 0 | 0 |
| C | 19 (20) | 78.9 | 59.3 (6.6) | 72.2 (5.9) | 12.9 (2.7) | 0 | 0 | 19 | 0 | 0 | 0 |
| D | 1 (1) | 100.0 | 52.0 | 62.0 | 10.0 | 1 | 0 | 0 | 0 | 0 | 0 |
| FUS | 5 (5) | ||||||||||
| Atypical FTLDU | 4 (4) | 100.0 | 45.5 (4.7) | 53.9 (4.4) | 8.4 (2.5) | 4 | 0 | 0 | 0 | 0 | 0 |
| NIFID | 1 (1) | 0.0 | 27.0 | NA | NA | 1 | 0 | 0 | 0 | 0 | 0 |
| TOTAL | 95 | 47 | 4 | 23 | 10 | 5 | 6 | ||||
| Pathology . | n (%) . | Gender, % male . | Age at clinical onset, mean (SD) (years) . | Age at death, mean (SD) (years) . | Duration, mean (SD) (years) . | Clinical presentation . | |||||
|---|---|---|---|---|---|---|---|---|---|---|---|
| . | . | . | . | . | . | Behavioural variant FTD . | FTD/MND . | Semantic dementia . | PNFA . | Corticobasal syndrome . | PSPS . |
| Tau | 42 (44) | ||||||||||
| Pick’s disease | 13 (14) | 69.2 | 55.4 (5.8) | 69.2 (3.5)a | 12.0 (4.3)a | 6 | 0 | 4 | 3 | 0 | 0 |
| MAPT mutations | 14 (15) | 64.3 | 50.4 (8.6) | 62.6 (10.4) | 12.1 (4.3) | 12 | 0 | 0 | 0 | 2 | 0 |
| Corticobasal degeneration | 9 (9) | 55.6 | 58.2 (9.0) | 67.8 (7.8)b | 8.3 (2.2)b | 3 | 0 | 0 | 4 | 1 | 1 |
| Progressive supranuclear palsy | 6 (6) | 28.6 | 59.0 (5.2) | 69.8 (36) | 10.0 (1.9) | 0 | 0 | 0 | 0 | 1 | 5 |
| TDP-43 | 48 (51) | ||||||||||
| A | 25 (26) (9 GRN) | 60.0 | 58.3 (6.9) | 65.3 (8.2) | 6.4 (2.5) | 20 | 1 | 0 | 3 | 1 | 0 |
| B | 3 (3) | 33.3 | 55.7 (6.7) | 60.8 (5.7) | 5.2 (1.0) | 0 | 3 | 0 | 0 | 0 | 0 |
| C | 19 (20) | 78.9 | 59.3 (6.6) | 72.2 (5.9) | 12.9 (2.7) | 0 | 0 | 19 | 0 | 0 | 0 |
| D | 1 (1) | 100.0 | 52.0 | 62.0 | 10.0 | 1 | 0 | 0 | 0 | 0 | 0 |
| FUS | 5 (5) | ||||||||||
| Atypical FTLDU | 4 (4) | 100.0 | 45.5 (4.7) | 53.9 (4.4) | 8.4 (2.5) | 4 | 0 | 0 | 0 | 0 | 0 |
| NIFID | 1 (1) | 0.0 | 27.0 | NA | NA | 1 | 0 | 0 | 0 | 0 | 0 |
| TOTAL | 95 | 47 | 4 | 23 | 10 | 5 | 6 | ||||
a Data from 10 patients.
b Data from 8 patients.
NA = not available; NIFID = neuronal intermediate filament inclusion disease; PSPS = progressive supranuclear palsy syndrome.
| Pathology . | n (%) . | Gender, % male . | Age at clinical onset, mean (SD) (years) . | Age at death, mean (SD) (years) . | Duration, mean (SD) (years) . | Clinical presentation . | |||||
|---|---|---|---|---|---|---|---|---|---|---|---|
| . | . | . | . | . | . | Behavioural variant FTD . | FTD/MND . | Semantic dementia . | PNFA . | Corticobasal syndrome . | PSPS . |
| Tau | 42 (44) | ||||||||||
| Pick’s disease | 13 (14) | 69.2 | 55.4 (5.8) | 69.2 (3.5)a | 12.0 (4.3)a | 6 | 0 | 4 | 3 | 0 | 0 |
| MAPT mutations | 14 (15) | 64.3 | 50.4 (8.6) | 62.6 (10.4) | 12.1 (4.3) | 12 | 0 | 0 | 0 | 2 | 0 |
| Corticobasal degeneration | 9 (9) | 55.6 | 58.2 (9.0) | 67.8 (7.8)b | 8.3 (2.2)b | 3 | 0 | 0 | 4 | 1 | 1 |
| Progressive supranuclear palsy | 6 (6) | 28.6 | 59.0 (5.2) | 69.8 (36) | 10.0 (1.9) | 0 | 0 | 0 | 0 | 1 | 5 |
| TDP-43 | 48 (51) | ||||||||||
| A | 25 (26) (9 GRN) | 60.0 | 58.3 (6.9) | 65.3 (8.2) | 6.4 (2.5) | 20 | 1 | 0 | 3 | 1 | 0 |
| B | 3 (3) | 33.3 | 55.7 (6.7) | 60.8 (5.7) | 5.2 (1.0) | 0 | 3 | 0 | 0 | 0 | 0 |
| C | 19 (20) | 78.9 | 59.3 (6.6) | 72.2 (5.9) | 12.9 (2.7) | 0 | 0 | 19 | 0 | 0 | 0 |
| D | 1 (1) | 100.0 | 52.0 | 62.0 | 10.0 | 1 | 0 | 0 | 0 | 0 | 0 |
| FUS | 5 (5) | ||||||||||
| Atypical FTLDU | 4 (4) | 100.0 | 45.5 (4.7) | 53.9 (4.4) | 8.4 (2.5) | 4 | 0 | 0 | 0 | 0 | 0 |
| NIFID | 1 (1) | 0.0 | 27.0 | NA | NA | 1 | 0 | 0 | 0 | 0 | 0 |
| TOTAL | 95 | 47 | 4 | 23 | 10 | 5 | 6 | ||||
| Pathology . | n (%) . | Gender, % male . | Age at clinical onset, mean (SD) (years) . | Age at death, mean (SD) (years) . | Duration, mean (SD) (years) . | Clinical presentation . | |||||
|---|---|---|---|---|---|---|---|---|---|---|---|
| . | . | . | . | . | . | Behavioural variant FTD . | FTD/MND . | Semantic dementia . | PNFA . | Corticobasal syndrome . | PSPS . |
| Tau | 42 (44) | ||||||||||
| Pick’s disease | 13 (14) | 69.2 | 55.4 (5.8) | 69.2 (3.5)a | 12.0 (4.3)a | 6 | 0 | 4 | 3 | 0 | 0 |
| MAPT mutations | 14 (15) | 64.3 | 50.4 (8.6) | 62.6 (10.4) | 12.1 (4.3) | 12 | 0 | 0 | 0 | 2 | 0 |
| Corticobasal degeneration | 9 (9) | 55.6 | 58.2 (9.0) | 67.8 (7.8)b | 8.3 (2.2)b | 3 | 0 | 0 | 4 | 1 | 1 |
| Progressive supranuclear palsy | 6 (6) | 28.6 | 59.0 (5.2) | 69.8 (36) | 10.0 (1.9) | 0 | 0 | 0 | 0 | 1 | 5 |
| TDP-43 | 48 (51) | ||||||||||
| A | 25 (26) (9 GRN) | 60.0 | 58.3 (6.9) | 65.3 (8.2) | 6.4 (2.5) | 20 | 1 | 0 | 3 | 1 | 0 |
| B | 3 (3) | 33.3 | 55.7 (6.7) | 60.8 (5.7) | 5.2 (1.0) | 0 | 3 | 0 | 0 | 0 | 0 |
| C | 19 (20) | 78.9 | 59.3 (6.6) | 72.2 (5.9) | 12.9 (2.7) | 0 | 0 | 19 | 0 | 0 | 0 |
| D | 1 (1) | 100.0 | 52.0 | 62.0 | 10.0 | 1 | 0 | 0 | 0 | 0 | 0 |
| FUS | 5 (5) | ||||||||||
| Atypical FTLDU | 4 (4) | 100.0 | 45.5 (4.7) | 53.9 (4.4) | 8.4 (2.5) | 4 | 0 | 0 | 0 | 0 | 0 |
| NIFID | 1 (1) | 0.0 | 27.0 | NA | NA | 1 | 0 | 0 | 0 | 0 | 0 |
| TOTAL | 95 | 47 | 4 | 23 | 10 | 5 | 6 | ||||
a Data from 10 patients.
b Data from 8 patients.
NA = not available; NIFID = neuronal intermediate filament inclusion disease; PSPS = progressive supranuclear palsy syndrome.
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