Patient Characteristics | All Patients (n = 55) | Cortex and Basal Ganglia (n = 32) | Only Cortex (n = 18) | P Value |
---|---|---|---|---|
Male/female | 28/27 | 17/15 | 9/9 | .83 |
Median age (yr) | 63 | 63 | 62 | .20 |
Range (yr) | 29–79 | 41–79 | 46–74 | |
Median duration (mo) | 6 | 5 | 12 | .05 |
Range (mo) | 1–41 | 1–41 | 3–32 | |
Codon 129 genotype of the PRNP | MM (64%) | MM (62%) | MM (67%) | .09 |
MV (25%) | MV (24%) | MV (27%) | ||
VV (12%) | VV (14%) | VV (7%) | ||
MR m.a.o. | 3.0 | 2.2 | 3.6 | .23 |
Range | 0.3–22.0 | 0.3–19.8 | 1.0–22.0 | |
MR m.b.d. | 3.0 | 1.8 | 8.0 | .06 |
Range | 0.3–23.1 | 0.3–23.1 | 0.4–22.8 | |
CSF | 85% | 81% | 94% | .40 |
14-3-3+ | 46/54 | 26/32 | 17/18 | |
EEG | 31% | 26% | 36% | .49 |
PSWC+ | 15/49 | 8/31 | 5/14 |
Note:—m.a.o. indicates months after onset; m.b.d., months before death; DWI, diffusion-weighted imaging; EEG, electroencephalography; PSWC, periodic short-wave complex.
* In 1 patient, only basal ganglia signal increase was found; 4 patients did not show signal alterations on the MR Imaging.