No. | Form | Age (yr) | PRNP | EEGa | 14.3.3b | Durationc | MRI Delayedd |
---|---|---|---|---|---|---|---|
1 | FFIe | 54 | MM D178N-129 mol/L | NS | – | 6 | 5 |
2 | gCJDe | 69 | MM E200K | + | + | 4 | 6 |
3 | gCJDe | 58 | MM E200K | + | NA | 6 | 3 |
4 | gCJDe | 49 | MV D178N-129V | – | – | 10+ | 4 |
5 | gCJDe | 70 | MV V203I | NS | + | 11 | 10 |
6 | gCJDe | 67 | MM E200K | NA | NA | 5 | 3 |
7 | iCJD | 18 | MV | NA | – | 16 | 6 |
8 | iCJD | 34 | MV | + | + | 23 | 8 |
9 | iCJDe | 25 | MM | – | – | 12 | 8 |
10 | GSSe | 47 | MV P102 L | – | – | 54 | 27 |
11 | vCJD | 43 | MM | + | – | 15 | 12 |
12 | vCJD | 52 | MM | NS | – | 8 | 7 |
13 | sCJD | 66 | NA | NA | NA | NA | 3 |
14 | sCJDe | 54 | MV | + | + | 10 | 6 |
15 | sCJDe | 62 | MM | + | + | 19 | 7 |
16 | sCJDe | 66 | VV | + | + | 4 | 2 |
17 | sCJDe | 51 | MM | + | + | 4 | 2 |
18 | sCJD | 56 | MV | – | + | 13 | 8 |
19 | sCJD | 74 | MM | NA | NA | 3 | 2 |
20 | sCJD | 52 | MM | + | + | 3 | 2 |
21 | sCJDe | 81 | MM | + | + | 4 | 4 |
22 | sCJD | 53 | MV | NS | + | 21 | 1 |
23 | sCJD | 55 | MV | + | – | 60+ | 23 |
24 | sCJD | 80 | MV | + | – | 9 | 8 |
25 | sCJD | 77 | MV | + | – | 24+ | 10 |
26 | sCJD | 40 | NA | NA | NA | 10+ | 9 |
27 | sCJD | 72 | MM | + | + | 3 | 2 |
28 | sCJDe | 64 | MM | + | + | 4 | 1 |
29 | sCJD | 55 | NA | – | + | 26 | 20 |
30 | sCJD | 84 | NA | NA | NA | 5 | 3 |
31 | sCJD | 80 | NA | NS | – | 2 | 1 |
Note:— – indicates not present.
a + Indicates periodic sharp wave complexes.
b Detection of 14.3.3 protein in the CSF.
c Duration of the disease in months.
d Time in months between first symptoms and MR imaging examination.
e The diagnosis was confirmed by postmortem examination and/or mutation was present in the PRNP. PRNP: genotype at codon 129 (MM, VV, MV) and mutation when present.