Table 3 Autonomic Symptom Profile Scores, score > 0, n (%).
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 | 36 (75) | 13 (72) | 23 (77) | 0.74 | 24 (50) | 0.02 |
Vasomotor impairment | 15 (31) | 4 (22) | 11 (37) | 0.35 | 6 (13) | 0.047 |
Secretomotor disorder | 28 (58) | 3 (17) | 25 (83) | 0.00 | 25 (52) | 0.68 |
Gastroparesis | 15 (31) | 4 (22) | 11 (37) | 0.35 | 6 (13) | 0.047 |
Diarrhoea | 13 (27) | 6 (33) | 7 (23) | 0.51 | 15 (31) | 0.82 |
Constipation | 10 (21) | 4 (22) | 6 (20) | 1.00 | 14 (29) | 0.48 |
Bladder disorder | 17 (35) | 5 (28) | 12 (40) | 0.54 | 18 (38) | 1.00 |
Pupillomotor impairment | 29 (60) | 12 (67) | 17 (57) | 0.55 | 19 (40) | 0.07 |
Sleep disorder | 28 (58) | 5 (28) | 23 (77) | 0.00 | 23 (48) | 0.41 |
Syncope | 3 (6) | 2 (11) | 1 (3) | 0.55 | 1 (2) | 0.62 |
Sum score | 46 (96) | 17 (94) | 29 (97) | 1.00 | 46 (96) | 1.00 |
| Â | Male patients n = 15 | non-ERT n = 2 | ERT n = 13 | p-value | Male controls n = 15 | p-value |
Male sexual failure | 6 (40) | 1 (50) | 5 (38) | 1.00 | 3 (20) | 0.43 |