Table 1. Baseline Characteristics.
| Characteristic | Patients With MRC Scale <5 at Enrollment | Patients With MRC Scale ≥5 at Enrollment | ||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Stratum 1 | Stratum 2 | Stratum 3 | ||||||||||
| Included in Model | Excluded From Model | |||||||||||
| sCJD | vCJD | iCJD | Fast IPD | Slow IPD | sCJD | Fast IPD | vCJD | iCJD | Slow IPD | Asymptomatic | Control | |
| Enrolled | 217 | 6 | 4 | 15 | 8 | 147 | 15 | 20 | 12 | 59 | 60 | 35 |
| Age at enrollment, median (range), y | 68 (46-88) | 54 (26-56) | 48 (27-51) | 65 (54 -78) | 47 (40-65) | 65 (39-83) | 55 (38-86) | 25 (14-50) | 46 (37-51) | 46 (26-69) | 39 (20-88) | 47 (23-75) |
| Sex | ||||||||||||
| Male | 78 | 2 | 3 | 7 | 3 | 81 | 8 | 15 | 11 | 26 | 22 | 17 |
| Female | 139 | 4 | 1 | 8 | 5 | 66 | 7 | 5 | 1 | 33 | 38 | 18 |
| Time between enrollment and first symptoms, median (IQR), mo | 3 (2-5) | 9 (7-22) | 5 (3-12) | 3 (2-4) | 36 (16-51) | 6 (3-9) | 7 (4-12) | 6 (5-11) | 8 (5-12) | 30 (14-62) | NA | NA |
| MRC Scale at enrollment [0-20] | ||||||||||||
| No. assessed | 215 | 5 | 4 | 15 | 8 | 147 | 15 | 20 | 12 | 59 | 60 | 35 |
| Median (IQR) | 1 (0-2) | 3 (2-4) | 3 (2-4) | 1 (0-2) | 2 (1-2) | 10 (7-14) | 14 (10-19) | 15 (11-18) | 16 (12-18) | 18 (12-19) | 20 (20-20) | 20 (20-20) |
| MMSE score at enrollment [0-30] | ||||||||||||
| No. assessed | 164 | 1 | 3 | 13 | 5 | 124 | 14 | 19 | 12 | 54 | 56 | 35 |
| Median (IQR) | 0 | 0 | 0 | 0 | 0 | 14 (6-20) | 18 (14-27) | 19 (14-23) | 21 (17-28) | 20 (15-28) | 30 (29-30) | 30 (30-30) |
| Rankin score at enrollment [0-5] | ||||||||||||
| No. assessed | 216 | 5 | 4 | 15 | 8 | 146 | 15 | 19 | 12 | 58 | 58 | 35 |
| Asymptomatic (0) | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 0 | 3 | 56 | 35 |
| No or slight symptoms (1/2) | 0 | 0 | 0 | 0 | 0 | 10 | 3 | 2 | 4 | 18 | 1 | 0 |
| Moderate disability (3) | 0 | 0 | 0 | 0 | 0 | 31 | 6 | 6 | 1 | 23 | 0 | 0 |
| Moderate to severe disability (4) | 7 | 2 | 0 | 2 | 1 | 73 | 4 | 10 | 6 | 11 | 0 | 0 |
| Severe disability (5) | 209 | 3 | 4 | 13 | 7 | 32 | 2 | 1 | 1 | 3 | 1 | 0 |
| Median (IQR) | 5 | 5 (4-5) | 5 | 5 | 5 | 4 (3-4) | 3 (3-4) | 4 (3-4) | 4 (2-4) | 3 (2-3) | 0 | 0 |
| Codon 129 | ||||||||||||
| No. assessed | 170 | 6 | 4 | 14 | 8 | 139 | 15 | 20 | 12 | 57 | 33 | 0 |
| MM | 113 | 6 | 1 | 13 | 4 | 46 | 9 | 19 | 2 | 32 | 21 | … |
| MV | 25 | 0 | 2 | 1 | 3 | 56 | 6 | 1 | 10 | 22 | 11 | … |
| VV | 32 | 0 | 1 | 0 | 1 | 37 | 0 | 0 | 0 | 3 | 1 | … |
Abbreviations: ellipses, no data available for this group; iCJD, iatrogenic Creutzfeldt-Jakob disease; IPD, inherited prion disease; IQR, interquartile range; MMSE, Mini-Mental State Examination; MRC, Medical Research Council; NA, not applicable; sCJD, sporadic Creutzfeldt-Jakob disease; vCJD, variant Creutzfeldt-Jakob disease.