Table 1:

MR imaging lesion patterns on DWI* and patient characteristics

Patient CharacteristicsAll Patients (n = 55)Cortex and Basal Ganglia (n = 32)Only Cortex (n = 18)P Value
Male/female28/2717/159/9.83
Median age (yr)636362.20
    Range (yr)29–7941–7946–74
Median duration (mo)6512.05
    Range (mo)1–411–413–32
Codon 129 genotype of the PRNPMM (64%)MM (62%)MM (67%).09
MV (25%)MV (24%)MV (27%)
VV (12%)VV (14%)VV (7%)
MR m.a.o.3.02.23.6.23
    Range0.3–22.00.3–19.81.0–22.0
MR m.b.d.3.01.88.0.06
    Range0.3–23.10.3–23.10.4–22.8
CSF85%81%94%.40
    14-3-3+46/5426/3217/18
EEG31%26%36%.49
    PSWC+15/498/315/14
  • 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.