Comparison of clinical features and number of patients and time (years) to reach disease milestones and autonomic symptoms between PSP phenotype groups
| RS (n=53) | PSP-P (n=23) | PAGF (n=5) | Cognitive (n=22) | Total (n=103) | P value | |
| Number of females (%) | 22 (42) | 5 (22) | 4 (80) | 8 (36) | 39 (38) | NS* |
| Age at onset | 66.8 (63.9–72.8) | 66.3 (56.6–72.8) | 69.1 (61.9–74.0) | 65.9 (61.3–72.6) | 66.8 (62.1–72.5) | NS |
| Age at death | 74.3 (70.8–81.0) | 76.5 (67.3–82.9) | 82.7 (75.8–84.6) | 74.7 (68.0–81.1) | 74.6 (70.4–81.4) | NS |
| Disease duration | 7.0 (5.5–8.4) | 9.0 (7.0–13.2) | 13.6 (8.3–16.3) | 7.6 (5.6–9.6) | 7.5 (5.7–10.0) | p=0.002†, p=0.003‡, p=0.05§, p=0.02¶ |
| Time to diagnosis | 2.3 (1.5–3.8) | 5.2 (2.5–7.0) | 3.0 (2.4–7.8) | 3.3 (2.0–5.3) | 3.1 (1.8–5.0) | p<0.001†, p=0.03** |
| Time to falls | n=53 (100%) | n=23 (100%) | n=5 (100%) | n=22 (100%) | n=103 (100%) | – |
| 0.4 (0–1.8) | 3.0 (1.0–5.0) | 2.0 (0.2–5.7) | 2.3 (0.5–4.0) | 1.0 (0–3.3) | p<0.001†, p<0.001** | |
| Time to first milestone | n=53 (100%) | n=22 (96%) | n=5 (100%) | n=22 (100%) | n=102 (99%) | NS* |
| 4.1 (2.6–5.0) | 5.3 (3.5–9.1) | 5.6 (5.3–12.5) | 3.3 (2.3–5.1) | 4.2 (2.8–5.7) | p=0.01†, p=0.004‡, p=0.01§, p=0.003¶ | |
| Time to wheelchair | n=47 (89%) | n=19 (83%) | n=5 (100%) | n=21 (95%) | n=92 (89%) | NS* |
| 4.7 (4.0–5.7) | 7.0 (4.1–9.6) | 6.1 (5.3–12.5) | 5.0 (3.6–8.8) | 5.0 (4.0–7.1) | p=0.02†, p=0.01‡, p=0.04¶ | |
| Time to dysarthria | n=40 (75%) | n=16 (70%) | n=3 (60%) | n=17 (81%) | n=76 (74%) | NS* |
| 5.7 (4.0–6.6) | 7.3 (5.7–12.2) | 8.5 (5.5–15.0) | 6.0 (4.3–7.4) | 6.0 (4.3–7.4) | p=0.003†, p=0.04‡, p=0.03§ | |
| Time to dysphagia | n=38 (73%) | n=16 (70%) | n=2 (40%) | n=14 (64%) | n=70 (69%) | NS* |
| 6.2 (4.7–7.6) | 8.1 (5.8–11.8) | 15.9 (15.1–16.8) | 6.5 (4.9–8.4) | 6.9 (5.1–8.6) | p=0.008†, p=0.004‡, p=0.01¶ | |
| Time to dementia | n=18 (34%) | n=13 (57%) | n=2 (40%) | n=16 (73%) | n=49 (48%) | p=0.01* |
| 3.4 (1.8–4.4) | 6.0 (3.5–11.3) | 11.6 (9.2–13.9) | 3.2 (2.2–5.1) | 3.7 (2.3–6.8) | p=0.01, p=0.01‡, p=0.02§, p=0.01¶ | |
| Time to care | n=46 (87%) | n=20 (87%) | n=3 (60%) | n=20 (91%) | n=89 (86%) | NS* |
| 5.0 (3.3–6.6) | 6.8 (4.0–10.1) | 13.7 (13.0–15.0) | 5.6 (3.7–8.8) | 5.6 (3.9–7.8) | p=0.04, p=0.001‡, p=0.005¶, p=0.01†† | |
| Time to constipation | n=38 (72%) | n=14 (61%) | n=5 (100%) | n=16 (73%) | n=73 (71%) | NS* |
| 4.1 (2.9–6.2) | 4.8 (3.6–6.1) | 11.7 (6.9–12.5) | 4.7 (2.7–6.8) | 4.8 (3.4–6.7) | p=0.001‡, p=0.006¶, p=0.009†† | |
| Time to urinary symptoms | n=43 (81%) | n=18 (78%) | n=3 (60%) | n=19 (86%) | n=83 (81%) | NS* |
| 2.7 (1.7–5.0) | 5.4 (2.2–9.8) | 4.0 (3.0–11.7) | 4.3 (3.2–6.3) | 4.0 (2.0–6.1) | p=0.006†, p=0.03** | |
| Time to erectile dysfunction (n=64) | n=10 (32%) | n=7 (39%) | n=1 (100%) | n=2 (14%) | n=20 (31%) | NS* |
| −2.0 (-7.4–0.8) | 2.5 (0.6–5.8) | 1.0 | 0.1 (-0.4–0.6) | 0.4 (-3.0–2.6) | NS | |
| Time to orthostatic hypotension | n=2 (4%) | n=5 (22%) | n=0 | n=2 (9%) | n=9 (9%) | NS* |
| 2.5 (2.0–3.1) | 4.0 (0.5–5.7) | – | −0.1 (-2.0–1.8) | 2.0 (0.5–4.0) | NS |
Data are expressed as number (frequency) or median (IQR). Cognitive group is a composite of PSP-Speech/Language, PSP-Frontal/Behavioural and PSP-Corticobasal Syndrome groups. Fisher’s exact test or Kruskall-Wallis comparison between groups and Dunn multiple comparisons test.
*Fisher’s exact test.
†RS versus PSP-P.
‡RS versus PAGF.
§Cognitive versus PSP-P.
¶Cognitive versus PAGF.
**RS versus cognitive.
††PSP-P versus PAGF.
NS, non-significant; PAGF, pure akinesia with gait freezing; PSP, progressive supranuclear palsy; PSP-P, progressive supranuclear palsy-parkinsonism; RS, Richardson syndrome.