Table 2 Autonomic Symptom Profile scores, median (range).
Domain | All patients n = 48 | non-ERT n = 18 | ERT n = 30 | non-ERT versus ERT, p-value | Healthy controls n = 48 | Patients versus controls, p-value |
|---|---|---|---|---|---|---|
Orthostatic intolerance | 13 (0-35) | 14 (0-28) | 13 (0-35) | 0.96 | 1.3 (0-25) | 0.004 |
Vasomotor impairment | 0 (0-8.2) | 0 (0-7.6) | 0 (0-8.2) | 0.33 | 0 (0-6.3) | 0.017 |
Secretomotor disorder | 1.5 (0-14) | 0 (0-7.5) | 3 (0-14) | 0.000 | 1.5 (0-7.5) | 0.23 |
Gastroparesis | 0 (0-8.4) | 0 (0-8.4) | 0 (0-5) | 0.36 | 0 (0-3.3) | 0.017 |
Diarrhoea | 0 (0-16) | 0 (0-8) | 0 (0-16) | 0.57 | 0 (0-8) | 1.00 |
Constipation | 0 (0-11) | 0 (0-6) | 0 (0-11) | 0.95 | 0 (0-9) | 0.63 |
Bladder disorder | 0 (0-12) | 0 (0-10) | 0 (0-12) | 0.38 | 0 (0-4) | 0.74 |
Pupillomotor impairment | 0.5 (0-4.5) | 0.5 (0-2.5) | 0.5 (0-4.5) | 0.78 | 0 (0-3) | 0.13 |
Sleep disorder | 1.5 (0-7.5) | 0 (0-6) | 1.5 (0-7.5) | 0.002 | 0 (0-6) | 0.06 |
Syncope | 0 (0-4) | 0 (0-4) | 0 (0-4) | 0.29 | 0 (0-4) | 0.31 |
Sum score | 22 (0-78) | 21 (0-49) | 24 (0-78) | 0.30 | 12 (0-35) | 0.005 |
| Â | Male patients n = 15 | non-ERT n = 2 | ERT n = 13 | p-value | Male controls n = 15 | p-value |
Male sexual failure | 0 (0-17) | 0.8 (0-1.5) | 0 (0-17) | 0.93 | 0 (0-7.5) | 0.29 |