Case No./Patient Sex/Age (y) | Dementia | Myoclonus | PSD | Prion Protein Gene | CDJ Type | Diagnostic Level | Diffusion-weighted Imaging | FLAIR Imaging | ADC Analysis |
---|---|---|---|---|---|---|---|---|---|
1/F/55 | Yes | Yes | Yes | M232R | F | Definite | E(1), L(2) | E(1), L(2) | E(1), L(2) |
2/M/78 | Yes | No | No | V180I | F | Definite | L(2) | L(2) | L(2) |
3/M/70 | No | Yes | Yes | NA | S? | Probable | E(4), L(1) | E(1) | NA |
4/M/66 | Yes | Yes | Yes | Wild type | S | Probable | E(1), L(3) | E(1), L(3) | E(1), L(3) |
5/M/60 | Yes | No | Yes | Wild type | S | Probable | E(1), L(1) | E(1), L(1) | E(1), L(1) |
6/M/58 | Yes | Yes | Yes | E200K | F | Definite | L(3) | NA | NA |
7/F/75* | Yes | No | No | Wild type† | S | Definite | L(1) | L(1) | L(1) |
8/M/68 | Yes | Yes | Yes | Wild type | S | Probable | E(2) | E(1) | NA |
9/F/74 | Yes | Yes | Yes | Wild type | S | Probable | L(1) | L(1) | L(1) |
10/F/76 | Yes | Yes | Yes | NA | S? | Probable | E(1) | E(1) | E(1) |
11/M/78 | Yes | Yes | Yes | NA | S? | Probable | E(1) | E(1) | E(1) |
12/F/76 | Yes | Yes | Yes | NA | S? | Probable | E(2) | E(2) | E(1) |
13/F/82 | Yes | No | No | V180I | F | Definite | E(1) | E(1) | E(1) |
Note.—E200K indicates the change of codon 200 from glutamate to lysine; M232R, change of codon 232 from methionine to arginine; V180I, change of codon 180 from valine to isoleucine; NA, not available; F, familial; S, sporadic; S?, possibly sporadic; E, the early-stage study; L, late-stage study. The number in the parenthesis indicates the number of MR studies performed. Total numbers of examinations were as follows: diffusion-weighted imaging, 14 early stage and 14 late stage; FLAIR imaging, 10 early stage and 10 late stage; ADC imaging, seven early stage and 10 late stage.
* The diagnosis of CJD was confirmed at histopathologic analysis.
† Wild-type prion protein gene with valine homozygosity at codon 129.