Table 1.
Main clinical and demographic characteristics.
| Baseline | 1.5 years | 4.5 years | ||||
|---|---|---|---|---|---|---|
| HSP (n = 26) |
SPG4 (n = 15) |
HSP (n = 18) |
SPG4 (n = 11) |
HSP (n = 13) |
SPG4 (n = 10) |
|
| Female | 14/26 (65%) | 9/15 (40%) | 10/18 (55%) | 6/11 (60%) | 6/13 (50%) | 5/10 (50%) |
| Age—years | 46 (12) | 48.6 (3.2) | 50 (12) | 52 (3.4) | 50 (12) | 53.4 (3.5) |
| Leg length—cm | 88 (6.5) | 88.7 (0.9) | 88 (6.5) | 88 (6.5) | 88 (6.5) | 88 (6.5) |
| SPRS | 18 (1.8) | 17 (2.5) | 19 (2.4) | 19 (3.1) | 17 (2.6) | 17 (3.1) |
| mSPRS | 17 (1.5) | 16 (2.3) | 16 (2.3) | 16 (2.8) | 16 (2.4) | 14 (2.9) |
| Disease duration—years | 17 (10) | 16 (2.3) | 17 (10) | 16.5 (2.9) | 17 (10) | 18.8 (3.3) |
| Age at onset—years | 30 (17) | 34.5 (4.9) | 30 (4.2) | 35 (5.5) | 32 (5.1) | 35 (6.1) |
| Walking-aid assistance | 15/26 (58%) | 10/15 (67%) | 10/18 (56%) | 7/11 (64%) | 7/13 (54%) | 6/10 (60%) |
| Clinical form | 24/26 pure (92%) | 100% pure | 16/18 pure (88%) | 100% pure | 100% pure | 100% pure |
| Disease stage | 1–2 (8%) | 1–1 (6%) | 1–2 (11%) | 1–1 (9%) | 1–0 (0%) | 1–0 (0%) |
| 2–3 (12%) | 2–3 (20%) | 2–3 (17%) | 2–3 (27%) | 2–1 (7%) | 2–1 (10%) | |
| 3–8 (30%) | 3–4 (27%) | 3–4 (22%) | 3–1 (18%) | 3–5 (39%) | 3–3 (30%) | |
| 4–13 (50%) | 4–7 (47%) | 4–9 (50%) | 4–6 (46%) | 4–7 (54%) | 4–6 (60%) | |
| Symptomatic treatment | ||||||
| Botulinum toxin | 2/26 (8%) | 1/15 (7%) | 1/18 (6%) | 1/11 (9%) | 1/13 (8%) | 1/10 (10%) |
| Baclofen/Tizanidine | 4/26 (15%) | 1/15 (7%) | 6/18 (33%) | 4/11 (36%) | 5/13 (39%) | 2/13 (15%) |
| Physical therapy | 13/26 (50%) | 6/15 (40%) | 12/18 (67%) | 6/11 (55%) | 8/13 (62%) | 4/10 (40%) |
Data are shown in frequency (percentage) and mean (standard deviation). cm, centimeters; HSP, hereditary spastic paraplegias; SPRS, Spastic Paraplegia Rating Scale; mSPRS, Motor Spastic Paraplegia Rating Scale.