Demographic and clinical data from genotype-specific patients with HSP included in this studya
| Parameter | SPG3A | SPG4 | SPG7 | SPG11 |
|---|---|---|---|---|
| Sample (No.) | 7 | 12 | 10 | 8 |
| Sex (M/F) | 2:5 | 6:6 | 5:5 | 4:4 |
| Age (yr) | 37.0 (12.0) | 57.0 (12.5) | 63.0 (10.0) | 29.0 (6.0) |
| Age at onset (yr) | 0 (3.5) | 37.5 (8.5) | 35.0 (10.0) | 17.5 (3.5) |
| Disease duration (yr) | 33.0 (10.2) | 21.0 (13.0) | 27.0 (24.0) | 10.0 (6.0) |
| SPRS-BR | 21.0 (18.0) | 18.5 (9.0) | 22.5 (23.0) | 24.0 (9.5) |
| Leg spasticity | 1 (0–2) | 1 (0–2) | 1 (0–2) | 1 (0–2) |
| Leg muscle strength | 4 (3–5) | 4 (4–5) | 5 (4–5) | 4 (4–5) |
a
Parameters are expressed using median (interquartile range). Muscle spasticity and strength were assessed in the following lower limb muscles: thigh adductors and abductors, quadriceps, hamstrings, ankle plantar flexors and dorsal flexors using the Ashworth scale and the Medical Research Council Scale for Muscle Strength, respectively. Results shown in the table represent an average value from all muscles tested in each patient.