Table 1.
| sCJD (n=17) | RPD (n=13)1 | OR | 95% CI | p | ||
|---|---|---|---|---|---|---|
| Male, n (%) | 8 (47) | 7 (54) | - | - | 0.99 | |
| Age at diagnosis, years (SEM) | 63.5 (1.8) | 68.2 (3.6) | - | - | 0.23 | |
| Age Range, years | 52.3-76 | 40-90.5 | - | - | - | |
| Onset to diagnosis, days (SEM) | 255 (83) | 144 (24) | - | - | 0.25 | |
| Onset to DTI scan, days (SEM) | 115 (21) | 128 (27) | - | - | 0.70 | |
| Clinical Data n (%) | ||||||
| Dementia | 17 (100) | 13 (100) | - | - | ||
| Cerebellar | 15 (88) | 2 (15) | 41 | 5.5 -311 | ||
| Pyr/EPyr | 14 (82) | 9 (69) | 2.1 | 0.4 – 11 | ||
| Akinetic Mutism | 9 (53) | 3 (23) | 3.8 | 0.8 – 17 | ||
| Visual | 3 (18) | 2 (15) | 1.2 | 0.2 -7 | ||
| Myoclonus | 13 (76) | 5 (38) | 5.2 | 1.1 - 24 | ||
| High Cortical | 8 (47) | 3 (23) | 3.0 | 0.6 -14 | ||
| Diagnostic Testing | Sensitivity (95% CI) | Specificity (95% CI) | ||||
| EEG PSWC (%) | 7/17 (41) | 6/13 (46) | 0.8 | 0.2 – 3.4 | 41 (27 - 56) | 54 (35 - 73) |
| CSF 14-3-3 “+” (%) | 10/15 (67) | 3/12 (25) | 6 | 1.2 - 31 | 67 (50 -79) | 75 (54 -90) |
| CSF T-tau “+” (%) | 12/15 (80) | 0/12 (0) | Inf | 9.2 - Inf | 80 (66 -80) | 100 (83 -100) |
| MRI according to Vitali and colleagues (2011) and Zerr and colleagues (2009) (%) | 12/14 (86) | 1/13 (8) | 72 | 4 - 2903 | 86 (65 – 93) | 92 (70 -100) |
1
Please see the Methods section for diagnoses
Abbreviations – SEM = standard error of the mean, Pyr/EPyr = pyramidal/extrapyramidal, T-tau = total tau, “+” = positive value per National Prion Disease Pathology Surveillance Center Report, 95 % CI = 95% Confidence Interval, Inf = Infinity, PSWC = periodic sharp wave complexes