Table 3. Characteristics of 43 cases of genetic prion disease identified in Canada, 1998 to 2013.
| Subtype1 | Mutation identified2 | Mutation not identified3 | Definite (%)4 | Probable (%)5 | Total |
|---|---|---|---|---|---|
| CJD | 21 | 0 | 16 (76) | 5 (24) | 21 |
| GSS | 11 | 9 | 16 (80) | 4 (20) | 20 |
| FFI | 2 | 0 | 1 (50) | 1 (50) | 2 |
| All | 34 | 9 | 33 (79) | 10 (21) | 43 |
1CJD—Creutzfeldt-Jakob disease; GSS—Gerstmann-Sträussler-Scheinker disease; FFI—fatal familial insomnia. 2Causative DNA change identified. 3Causative DNA change not identified (no genetic analysis performed). 4Diagnosis confirmed by neuropathology. 5Diagnosis supported by non-neuropathological criteria.