Table 2. Age at onset, duration of illness, and sense of smell, REM behavior disorder, urinary/GI symptoms, plasma norepinephrine, and heart rate at entry visit in patients who retained a pure autonomic failure phenotype at their last follow-up visit.
| # Age at onset /sex | Duration of illness2 yrs | UPSIT score | RBD | Urinary Sx3 | GI Sx4 | NE pg/ml | HR bpm | ΔHR bpm |
|---|---|---|---|---|---|---|---|---|
| 1. 52/M | 5 | NA | - | + + | + + | NA | 75 | +15 |
| 2. 63/M | 4 | NA | - | + | + | NA | 63 | +18 |
| 3. 53/M | 8 | NA | - | + | + | NA | 73 | 0 |
| 4. 64/F | 3 | 37 | - | + | + | 110 | 69 | +3 |
| 5. 54/M | 2 | 38 | - | + | + | 61 | 67 | +5 |
| 6. 43/M | 14 | 36 | - | - | + | 32 | 69 | +2 |
| 7. 70/F | 5 | 30 | - | - | + | 78 | 76 | +8 |
| 8. 77/F | 1 | 30 | - | - | + | 66 | 68 | +3 |
| 9. 60/F | 6 | 30 | - | + | - | 101 | 79 | +1 |
| 10. 52/M | 10 | 36 | - | + | - | NA | 62 | +10 |
| 11. 64/M | 12 | 33 | - | + | + + | 38 | 68 | +1 |
| 12. 54/M | 8 | 33 | - | + + | + + | 23 | 64 | 0 |
|
| ||||||||
| 57 (12) | 6 (5) | 33 (6) | 0% | + | + | 63 (37) | 69 (6) | +3 (6) |
Age at onset was defined as age at first symptom of orthostatic hypotension.
Duration of illness was defined as onset to final follow-up visit.
Urinary symptoms severity was based on the UMSARS-I score, where ‘-’ = no symptoms; ‘+’ = mild/no medication required; and ‘+ +’ = moderate/requiring medications.
GI symptom severity wasrated as constipation score in UMSARS-I, where ‘-’ = no symptoms, + = mild/occasional, no medication required, ‘++’ = moderate/frequent medication required, and ‘+++’ = severe/chronic use of laxatives, enemas, inability to have a spontaneous bowel movement).
UPSIT = University of Pennsylvania Smell Identification Test; RBD=rapid eye movement sleep behavior disorder; GI = gastrointestinal; NE=norepinephrine; HR=heart rate, NA = not available. All men had erectile dysfunction. No patient with PAF had subtle motor signs at the final visit.