Final diagnosis | n | Updated WHO criteria | 2017 EU criteria | ||||||
sCJD probable | sCJD possible | Non-CJD | Sensitivity (95% CI) | sCJD probable | sCJD possible | Non-CJD | Sensitivity (95% CI) | ||
Definite sCJD | 297 | 224 | 10 | 63 | 75.4* (70.2 to 80.0) | 291 | 1 | 5 | 98.0 (95.7 to 99.1) |
gCJD | 151 | 120 | 7 | 24 | 79.5* (72.3 to 85.1) | 147 | 2 | 2 | 97.3 (93.4 to 99.0) |
Definite CJD | 448 | 344 | 17 | 87 |
76.8*
(72.7 to 80.5) | 438 | 3 | 7 |
97.8
(95.9 to 98.8) |
Probable sCJD | 402 | 292 | 16 | 94 | 72.6* (68.1 to 76.8) | 402 | 0 | 0 | 100 (99.0 to 100) |
Probable or definite CJD | 850 | 636 | 33 | 181 |
74.8*
(71.8 to 77.6) | 840 | 3 | 7 |
98.8
(97.8 to 99.4) |
sCJD probable | sCJD possible | Non-CJD | Specificity (95% CI) | sCJD probable | sCJD possible | Non-CJD | Specificity (95% CI) | ||
Definite non- CJD | 61 | 11 | 8 | 42 | 82.0† (70.5 to 89.6) | 11 | 8 | 42 | 82.0 (70.5 to 89.6) |
Probable non-CJD | 339 | 13 | 61 | 265 | 96.2† (93.5 to 97.7) | 14 | 61 | 264 | 95.9 (93.2 to 97.5) |
Total non-CJD | 400 | 24 | 69 | 307 |
94.0†
(91.2 to 95.9) | 25 | 69 | 306 |
93.7
(90.9 to 95.7) |
Data referring to total definite CJD, total (probable+definite) CJD, and total non-CJD are shown in bold.
*Compared with the 2017 EU criteria, p<0.001 (McNemar’s test).
†Compared with the 2017 EU criteria, p>0.99 (McNemar’s test).
CJD, Creutzfeldt-Jakob disease; EU, European Union; gCJD, genetic CJD; sCJD, sporadic CJD.