Table 1.
Demographic and clinical findings in FTLD patient groups and healthy controls
| MND‐pm | MND‐plus | bvFTD | svPPA | nfvPPA | PSPs | HC | P | |
|---|---|---|---|---|---|---|---|---|
| Age at MRI (years) | 58.9 ± 10.9 (35–77) | 64.1 ± 8.5 (44–76) | 61.6 ± 8.2 (46–75) | 65.5 ± 8.5 (51–82)* | 68.1 ± 6.0 (59–80)*× | 69.5 ± 7.5 (52–85)°*× | 64.1 ± 5.6 (58–75)* | 0.01 |
| Gender (women/men) | 10/11 | 6/8 | 5/9 | 7/5 | 7/4 | 9/12 | 18/10 | 0.51 |
| Education (years) | 11.1 ± 3.7 (3–17)° | 9.6 ± 4.2 (4–18)° | 10.8 ± 5.1 (3–18)° | 11.0 ± 5.5 (3–18)° | 10.0 ± 6.2 (5–22)° | 8.9 ± 4.1 (5–17)° | 14.4 ± 4.7 (5–24) | 0.02 |
| Age at onset (years) | 57.7 ± 11.8 (32–77) | 62.9 ± 8.7 (42–77) | 58.7 ± 7.2 (44–69) | 63.2 ± 8.9 (50–81) | 66.4 ± 5.9 (58–79)*× | 67.2 ± 8.1 (50–81)*× | – | 0.02 |
| Disease duration (months) | 27.0 ± 23.8 (6–97) | 26.0 ± 22.5 (3–70) | 38.4 ± 26.0 (8–97) | 44.0 ± 18.5 (23–84) | 36.1 ± 17.6 (15–70) | 47.4 ± 33.1 (3–166)*# | – | 0.02 |
| CDR‐SB | – | – | 4.5 ± 2.6 (2–9)^ | 2.4 ± 2.3 (0.5–8) | 2.6 ± 1.0 (1.5–4) | – | – | 0.06 |
| UPDRS III | – | – | – | – | – | 60.6 ± 24.9 (0–92) | – | – |
| ALSFRS‐R | 37.9 ± 5.7 (23–46) | 34.0 ± 9.1 (22–45) | – | – | – | – | – | – |
| Disease progression rate | 0.6 ± 0.6 (0.1–2.5) | 0.8 ± 0.7 (0.1–3.0) | – | – | – | – | – | – |
| UMN score | 11.0 ± 3.7 (1–14) | 9.9 ± 5.9 (1–16) | – | – | – | – | – | – |
| WMH load (mm3) | 0.8 ± 1.0 (0.0–3.4) | 1.3 ± 1.2 (0–3.2) | 1.2 ± 1.4 (0.04–4.2) | 1.4 ± 1.5 (0.1–4.3) | 1.9 ± 1.5 (0.3–4.9)°* | 1.2 ± 1.4 (0.0–4.3) | 1.1 ± 1.1 (0.0–4.6) | 0.24 |
Values are means ± standard deviations (range) or number. P values refer to the Kruskal–Wallis or Fisher exact tests among groups as appropriate. Symbols refer to between group comparisons assessed using ANOVA models followed by pairwise comparisons: °P < 0.05 versus HC; *P < 0.05 versus MND‐pm; # P < 0.05 versus MND‐plus; × P < 0.05 versus bvFTD; ^P < 0.05 versus svPPA. Abbreviations: ALSFRS‐R, ALS functional rating scale revised; (Cedarbaum et al., 1999) bvFTD, behavioral variant of FTD; CDR‐SB, clinical dementia rating scale Sum of Boxes; (Morris, 1993) HC, healthy controls; MND, motor neuron disease; MND‐pm, pure‐motor MND; MND‐plus, MND with cognitive/behavioral impairment; nfvPPA, nonfluent variant of primary progressive aphasia; PSPs, progressive supranuclear palsy syndrome; svPPA, semantic variant of primary progressive aphasia; UPDRS III, Unified Parkinson's disease rating scale III; (Goetz et al., 2008) UMN score, upper motor neuron score;(Turner et al., 2004) WMH = white matter hyperintensity.