Table 1.
Age at onset, duration of PAF phase, and entry visit smell, REM behaviour disorder, urinary/GI symptoms, plasma norepinephrine, and heart rate in patients later diagnosed with a manifest synucleinopathy.
| # Age at onset1 /sex | PAF phase2 yrs | UPSIT score | RBD | Urinary Sx3 | GI Sx4 | NE pg/ml | HR bpm | ΔHR bpm |
|---|---|---|---|---|---|---|---|---|
| 1. 66/F | 5 | 14 | + | + | + | 134 | 65 | +5 |
|
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| 2. 73/M | 7 | 9 | + | + | + | 226 | 60 | +3 |
| 3. 58/M | 15 | NA | - | - | - | NA | 47 | +2 |
| 4. 49/M | 25 | NA | + | + | + | NA | 60 | 0 |
| 5. 52/M | 25 | NA | + | + + | - | NA | 64 | +10 |
| 6. 64/M | 11 | 12 | + | - | - | 162 | 66 | -1 |
|
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| PD: 60 (9) | 14 (9) | 11 (3) | 83% | + | + | 174 (47) | 60 (7) | 3 (4) |
|
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| 7. 75/M | 5 | 23 | + | + | + | 97 | 59 | +6 |
| 8. 65/F | 14 | 11 | + | - | + | 32 | 64 | 0 |
| 9. 60/F | 21 | NA | + | + | - | NA | 72 | -2 |
| 10. 72/F | 5 | NA | + | + | + | 99 | 69 | +9 |
| 11. 77/M | 6 | 11 | + | + | + | 218 | 62 | +8 |
| 12. 72/F | 8 | 23 | + | - | - | 118 | 54 | +9 |
| 13. 63/M | 8 | 11 | + | + | - | 85 | 67 | +6 |
| 14. 75/F | 5 | 23 | + | + | + + | 166 | 75 | +10 |
| 15. 54/M | 13 | 23 | + | - | - | NA | 47 | +3 |
| 16. 64/M | 13 | 29 | + | + + | - | NA | 62 | +5 |
| 17. 63/M | 13 | 23 | + | + | + + | 114 | 78 | 0 |
| 18. 71/M | 7 | 26 | + | + | + | NA | 60 | +11 |
| 19. 54/M | 10 | NA | + | + | + | NA | 83 | +10 |
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| DLB: 66 (7) | 10 (5) | 20 (7) | 100% | + | + | 116 (56) | 66 (10) | 6 (4) |
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| 20. 45/M | 4 | 37 | + | + + | + + + | 133 | 71 | +15 |
| 21. 64/M | 4 | 37 | + | + + | + + + | 379 | 71 | +11 |
| 22. 46/F | 3 | 33 | + | + + | + + + | 464 | 76 | +10 |
| 23. 57/M | 6 | NA | + | + + | + + | NA | 73 | +11 |
| 24. 47/M | 8 | NA | + | + + | + + + | NA | 65 | +15 |
| 25. 58/M | 8 | 30 | + | + + | + + | 271 | 69 | +13 |
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| MSA: 53 (8) | 6 (2) | 34 (3) | 100% | + + | + + + | 312 (143) | 71 (4) | 13 (2) |
Onset was defined as age at first symptom of orthostatic hypotension.
Duration of PAF phase was defined as time from onset of orthostatic symptoms to diagnosis of manifest CNS synucleinopathy.
Urinary symptoms severity was based on the UMSARS-I score, where ‘-’ = no symptoms; ‘+’ = mild/no medication required; and ‘+ +’ = moderate/requiring medications.
GI symptom severity was rated 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; NE=norepinephrine; HR=heart rate, NA = not available. All men had erectile dysfunction. Cases 3, 8, and 12 did not have subtle motor signs on entry.