Table 1.
Demographic and clinical characteristics of participants
| Characteristic | n | All participants, n = 98 | Clinical subgroups | P-value | |||
|---|---|---|---|---|---|---|---|
| nfvPPA, n = 75 | PPAOS, n = 12 | Non-fluent dysarthric, n = 5 | PAA, n = 6 | ||||
| Age at diagnosis, years | 98 | 68.0 (7.1) | 68.7 (7.0) | 64.2 (8.7) | 68.9 (4.8) | 66.2 (5.7) | 0.5 |
| Age at estimated symptom onset, years | 83 | 63.1 (7.1) | 63.7 (7.1) | 58.1 (7.8) | 65.6 (4.7) | 61.7 (6.0) | 0.5 |
| Time from estimated symptom onset to diagnosis, years | 83 | 4.3 (2.0) | 4.4 (1.9) | 3.3 (1.9) | 3.3 (1.7) | 4.5 (2.5) | 0.5 |
| Biological sex | 98 | – | – | – | – | – | 0.9 |
| Female | – | 63/98 (64%) | 49/75 (65%) | 8/12 (67%) | 3/5 (60%) | 3/6 (50%) | – |
| Male | – | 35/98 (36%) | 26/75 (35%) | 4/12 (33%) | 2/5 (40%) | 3/6 (50%) | – |
| Years of education | 98 | 16.2 (3.2) | 16.3 (3.1) | 15.2 (2.8) | 15.0 (2.0) | 18.0 (5.2) | 0.7 |
| Handness | 98 | – | – | – | – | 0.8 | |
| Right-handed | – | 86/98 (88%) | 64/75 (85%) | 12/12 (100%) | 5/5 (100%) | 5/6 (83%) | – |
| Left-handed | – | 11/98 (11%) | 10/75 (13%) | 0/12 (0%) | 0/5 (0%) | 1/6 (17%) | – |
| Ambidextrous | – | 1/98 (1.0%) | 1/75 (1.3%) | 0/12 (0%) | 0/5 (0%) | 0/6 (0%) | – |
| MMSE/30 | 92 | 25.6 (4.1) | 25.3 (4.0) | 29.0 (0.9) | 27.0 (2.3) | 22.8 (6.4) | 0.02 |
| CDR® Sum of Boxes | 95 | 1.9 (1.8) | 1.9 (1.9) | 1.0 (1.1) | 2.7 (1.5) | 2.5 (1.9) | 0.5 |
| CDR® plus NACC FTLD-SB | 84 | 3.6 (2.4) | 3.6 (2.6) | 2.6 (1.3) | 4.5 (2.0) | 4.5 (2.0) | 0.5 |
| Global score CDR® plus NACC FTLD | 84 | – | – | – | – | 0.9 | |
| 0.5 | – | 40/84 (48%) | 32/64 (50%) | 5/10 (50%) | 1/4 (25%) | 2/6 (33%) | – |
| 1 | – | 38/84 (45%) | 26/64 (41%) | 5/10 (50%) | 3/4 (75%) | 4/6 (67%) | – |
| 2 | – | 6/84 (7.1%) | 6/64 (9.4%) | 0/10 (0%) | 0/4 (0%) | 0/6 (0%) | – |
| NPI total score | 88 | 14.9 (13.0) | 14.9 (13.3) | 10.7 (10.6) | 22.2 (10.3) | 17.0 (16.9) | 0.5 |
| UPDRS-III, total score | 47 | 14.7 (11.3) | 14.4 (11.3) | 2.0 (1.4) | 18.0 (3.0) | 19.8 (13.8) | 0.5 |
| PSPRS, total score | 57 | 12.4 (10.1) | 12.8 (10.2) | 6.9 (5.8) | 22.0 (18.4) | 14.0 (10.2) | 0.5 |
| Mutation | 98 | – | – | – | – | 0.9 | |
| No mutation | – | 79/98 (81%) | 59/75 (79%) | 9/12 (75%) | 5/5 (100%) | 6/6 (100%) | – |
| Not screened | – | 16/98 (16%) | 13/75 (17%) | 3/12 (25%) | 0/5 (0%) | 0/6 (0%) | – |
| GRN | – | 3/98 (3.1%) | 3/75 (4.0%) | 0/12 (0%) | 0/5 (0%) | 0/6 (0%) | – |
| Neuropathological diagnosis | 43 | – | – | – | – | 0.7 | |
| Corticobasal degeneration | – | 17/43 (40%) | 14/33 (42%) | 1/3 (33%) | 0/3 (0%) | 2/4 (50%) | – |
| Progressive supranuclear palsy | – | 11/43 (26%) | 6/33 (18%) | 1/3 (33%) | 3/3 (100%) | 1/4 (25%) | – |
| Pick’s disease | – | 7/43 (16%) | 5/33 (15%) | 1/3 (33%) | 0/3 (0%) | 1/4 (25%) | – |
| FTLD-TDP type A | – | 4/43 (9.3%) | 4/33 (12%) | 0/3 (0%) | 0/3 (0%) | 0/4 (0%) | – |
| Other pathologies | – | 4/43 (9.3%) | 4/33 (12%) | 0/3 (0%) | 0/3 (0%) | 0/4 (0%) | – |
Unless otherwise indicated, results are expressed as mean (standard deviation, SD). The P-values are adjusted for multiple comparisons (false discovery rate, FDR) and statistically significant P-values are highlighted in bold. The ‘non-fluent dysarthric’ subgroup included five participants with reduced speech fluency, executive dysfunction and dysarthria but without apraxia of speech or quantitative evidence of expressive agrammatism. ‘Other pathologies’ included one participant with an unclassifiable tauopathy, one participant with Alzheimer’s disease, one with mixed Alzheimer’s disease and corticobasal degeneration, and one with FTLD-TDP type B. CDR = Clinical Dementia Rating; FTLD = frontotemporal lobar degeneration; MMSE = Mini-Mental State Examination; nfvPPA = non-fluent/agrammatic variant primary progressive aphasia; PPAOS = primary progressive apraxia of speech; PAA = primary agrammatic aphasia; NPI = neuropsychiatric inventory; PSPRS = progressive supranuclear palsy rating scale; UPDRS-III = Unified Parkinson’s Disease Rating Scale part III.