TABLE 1.
Participants’ demographic and clinical measures.
| ALS | PD | HD | HCs | P | |
| N | 99 | 73 | 25 | 101 | – |
| Sex (M/F) | 51/48 | 51/22 | 16/9 | 39/62 | HCs: F > M; PD: M > Fa |
| Age (years) | 63.99 ± 10.37(28–84) | 68.59 ± 9.02 (47–90) | 55.88 ± 16.07 (27–78) | 63.39 ± 11.10 (24–86) | HD < HCs & ALS < PDb |
| Education (years) | 12.52 ± 4.51 (5–19) | 13.73 ± 3.68 (5–18) | 11.76 ± 3.18 (8–17) | 13.34 ± 3.80 (5–22) | n.s.b |
| Disease duration (months) | 17.25 ± 17.87 (2–108) | 82.63 ± 64.85 (6–288) | 111.36 ± 164.41 (12–852) | – | PD & HD > ALSc |
| ALSFRS-R | 39.50 ± 6.08 (22–48) | – | – | – | – |
| UPDRS-II | – | 6.89 ± 5.44 (0–28) | – | – | – |
| UPDRS-III | – | 13.05 ± 9.20 (0–39) | – | – | – |
| UHDRS-I | – | – | 32.75 ± 13.53 (12–73) | – | – |
| UHDRS-IV | – | – | 18.84 ± 4.50 (8–25) | – | – |
| UHDRS-V | – | – | 80.80 ± 14.48 (50–100) | – | – |
| UHDRS-VI | – | – | 9.24 ± 3.00 (3–13) | – | – |
| HTT triplets (N) | – | – | 43.61 ± 4.51 (39–59) | – | – |
| Genetics (N) | – | ||||
| C9orf72 | 3 | – | – | – | – |
| TARDBP | 3 | – | – | – | – |
| SOD1 | 1 | – | – | – | – |
| King’s | |||||
| Stage 0 | 4.9% | – | – | – | – |
| Stage 1 | 39% | – | – | – | – |
| Stage 2 | 25.6% | – | – | – | – |
| Stage 3 | 26.8% | – | – | – | – |
| Stage 4 | 3.7% | – | – | – | – |
| Modified Hoehn-Yahr | |||||
| Stage 1 | – | 15.3% | – | – | – |
| Stage 1.5 | – | 18.1% | – | – | – |
| Stage 2 | – | 34.7% | – | – | – |
| Stage 2.5 | – | 20.8% | – | – | – |
| Stage 3 | – | 11.1% | |||
| Shoulson-Fahn | |||||
| Stage 1 | – | – | 36% | – | – |
| Stage 2 | – | – | 44% | – | – |
| Stage 3 | – | – | 20% | – | – |
| ECAS | 100.73 ± 16.98 (43–127) | 103.38 ± 14.03 (58–124) | 81.20 ± 23.43 (34–116) | – | ALS & PD > HDc |
| STAI-Y1 | 53.74 ± 11.06 (33–87) | 46.01 ± 10.33 (20–78) | 51.60 ± 9.37 (37–76) | – | ALS > PDb |
| STAI-Y2 | 49.54 ± 9.32 (33–71) | 48.26 ± 9.43 (34–74) | 49.52 ± 8.22 (35–63) | – | n.s.b |
| BDI | 13.33 ± 8.62 (0–41) | 9.21 ± 3.38 (0–38) | 10.24 ± 7.17 (0–25) | – | ALS > PDb |
| DAS | |||||
| Total | 21.52 ± 7.51 (3–40) | 24.19 ± 7.15 (11–41) | 29.20 ± 9.09 (11–48) | 20.69 ± 7.42 (1–55) | PD & HD > HCsb |
| Initiation | 8.40 ± 4.51 (0–20) | 8.73 ± 4.16 (1–23) | 10.32 ± 5.62 (2–23) | 7.36 ± 3.38 (0–17) | HD > HCsb |
| Executive | 6.63 ± 4.21 (0–18) | 7.44 ± 4.29 (0–19) | 12.00 ± 5.01 (6–24) | 6.43 ± 4.11 (0–26) | HD > HCsb |
| Emotional | 6.48 ± 3.50 (0–15) | 8.03 ± 3.21 (0–15) | 6.88 ± 3.13 (2–14) | 6.86 ± 3.48 (0–21) | n.s. |
ALS, amyotrophic laterals sclerosis; ALSFRS-R, amyotrophic lateral sclerosis functional rating scale-revised; BDI, Beck depression inventory; DAS, dimensional apathy scale; ECAS, Edinburgh cognitive and behavioral ALS screen; F, female; HCs, healthy controls; HD, Huntington’s disease; HTT, huntingtin; M, male; PD, Parkinson’s disease; STAI-Y1, state and trait anxiety inventory–form Y–state anxiety; STAI-Y2, state and trait anxiety inventory–form Y–trait anxiety; UHDRS, unified Huntington’s disease rating scale; UPDRS, unified Parkinson’s disease rating scale. aAdjusted standardized residuals (χ2-statistics). bBonferroni-corrected post-hoc comparisons (F-statistics). cDwass-Steel-Critchlow-Flinger post-hoc comparisons (Kruskall-Wallis H-statistics).