Table 1.
Physical performance assessments in patients with SBMA.
| SBMA mean ± SD (range) | Percentage of reference values† | |
|---|---|---|
| Quantitative muscle assessment (kg) | ||
| Upper extremity composite | 66 ± 25 (18–140) | 42% |
| Lower extremity composite | 98 ± 41 (28–231) | 65% |
| Total force | 164 ± 63 (63–372) | 55% |
| Adult Myopathy Assessment Tool | ||
| Endurance score (range = 0–24, 24 = max score) | 14.5 ± 5.3 (4–24) | 60% |
| Functional score (range = 0–21, 21 = max score) | 14.7 ± 5.4 (2–21) | 70% |
| Total score (range = 0–45, 45 = max score) | 29.2 ± 10.3 (9–45) | 65% |
| Timed 2-minute walk (m) | ||
| Distance walked | 109 ± 50 (15–208) | 51% |
†Normative QMA values obtained from published reference values [22, 23]; AMAT results expressed as a percentage of the maximum attainable score; timed 2 minute walk results compared with published age and gender matched normal reference values [24].
Abbreviations: SBMA: spinal bulbar muscular atrophy; kg: kilograms; m: meters.