Table 1.
Demographics, clinical scores, severity of linguistic symptoms, cognitive task data and regional brain volumes for the bvFTD groups and healthy controls
| Controls | bvFTD | |||
|---|---|---|---|---|
| C9orf72 | GRN | MAPT | ||
| Number of participants | 100 | 24 | 9 | 10 |
| % Male | 45 | 67 | 44 | 80 |
| % Right-handed | 95 | 96 | 100 | 70 |
| Age (years) | 60.2 (7.1) | 62.3 (7.9) | 67.2 (7.4) | 59.1 (7.7) |
| Education (years) | 13.5 (3.1) | 14.5 (3.6) | 12.0 (3.2) | 13.4 (3.5) |
| MMSE | 29.1 (1.2) | 26.0 (3.4) | 23.9 (5.1) | 24.4 (5.0) |
| CDR® plus NACC FTLD Global score | 0.1 (0.2) | 1.6 (0.5) | 1.7 (0.5) | 1.8 (0.4) |
| CDR® plus NACC FTLD Sum of Boxes | 0.3 (0.6) | 8.5 (3.9) | 8.0 (3.5) | 9.0 (3.1) |
| Progressive Aphasia Severity Scale Sum of Boxes | 0.1 (0.4) | 2.7 (2.6) | 2.2 (2.5) | 3.3 (2.6) |
| Linguistic symptoms | ||||
| Impaired articulation | 0.07 (0.18) | 0.27 (0.53) | 0.06 (0.17) | 0.10 (0.21) |
| Decreased fluency | 0.00 (0.00) | 0.44 (0.61) | 0.39 (0.36) | 0.55 (0.50) |
| Impaired grammar/syntax | 0.00 (0.00) | 0.06 (0.17) | 0.11 (0.33) | 0.15 (0.34) |
| Impaired word retrieval | 0.08 (0.21) | 0.48 (0.56) | 0.44 (0.68) | 0.80 (0.59) |
| Impaired speech repetition | 0.01 (0.05) | 0.04 (0.14) | 0.06 (0.17) | 0.00 (0.00) |
| Impaired sentence comprehension | 0.00 (0.00) | 0.38 (0.61) | 0.22 (0.36) | 0.45 (0.69) |
| Impaired single word comprehension | 0.01 (0.05) | 0.06 (0.17) | 0.00 (0.00) | 0.40 (0.66)ab |
| Dyslexia | 0.02 (0.12) | 0.08 (0.41) | 0.11 (0.33) | 0.15 (0.24) |
| Dysgraphia | 0.01 (0.05) | 0.17 (0.32) | 0.22 (0.44) | 0.15 (0.34) |
| Impaired functional communication | 0.03 (0.13) | 0.73 (0.83) | 0.56 (0.73) | 0.55 (0.44) |
| Cognitive tasks | ||||
| Boston Naming Test (/30) | 27.8 (2.0) | 23.9 (4.0) | 24.3 (4.9) | 17.0 (7.7)ab |
| Modified Camel and Cactus Test (/32) | 30.0 (1.5) | 24.7 (5.2) | 25.1 (4.3) | 24.5 (5.9) |
| Category Fluency (max in 60 s) | 23.3 (5.6) | 13.0 (6.2) | 12.4 (6.6) | 12.7 (5.2) |
| Regional left hemisphere brain volumes (as a % of TIV) | ||||
| Inferior frontal gyrus | 0.56 (0.06) | 0.47 (0.09)c | 0.47 (0.08) | 0.55 (0.06) |
| Insula | 0.36 (0.03) | 0.28 (0.04) | 0.30 (0.02) | 0.26 (0.05)b |
| Motor cortex | 1.36 (0.12) | 1.21 (0.13)c | 1.22 (0.07) | 1.33 (0.12) |
| Temporal pole | 0.50 (0.06) | 0.43 (0.08) | 0.46 (0.07) | 0.35 (0.09)ab |
| Superior temporal gyrus | 0.48 (0.05) | 0.41 (0.04) | 0.42 (0.05) | 0.41 (0.04) |
| Supratemporal region | 0.40 (0.04) | 0.37 (0.04) | 0.35 (0.05) | 0.37 (0.04) |
| Angular gyrus | 0.50 (0.06) | 0.43 (0.05)c | 0.49 (0.08) | 0.50 (0.08) |
Data are shown as mean (standard deviation). Bold items are significantly different to controls
bvFTD behavioural variant fronto-temporal dementia; TIV total intracranial volume
asignificantly impaired compared to C9orf72 mutation carriers
bsignificantly impaired compared to GRN mutation carriers
csignificantly impaired compared to MAPT mutation carriers