Table 3 Frequency distribution of different symptoms

SymptomsMRI+MRI−p ValueMRI+MRI−p Value
Stage 1*Stage 1*Stage 1*TotalTotalTotal
Values are percentages.
* Onset of symptoms until MRI.
† Whole time interval from onset to death.
MRI+ = typical hyperintense basal ganglia; MRI− = no hyperintense basal ganglia.
Main symptoms
    Dementia80570.00390930.576
    Myoclonus40380.86484841.000
    Ataxia73700.71080880.372
    Pyramidal signs39391.00072750.850
    Extrapyramidal signs32430.22170820.123
    Akinetic mutism1240.08539550.064
Psychiatric symptoms
    Depression33520.02635550.018
    Aggressiveness17140.82020300.166
    Paranoia991.00010130.791
    Hallucinations31180.08837361.000
    Sleep disturbance35410.49136450.308
Visual disturbances
    Cortical blindness13131.00016180.821
    Gaze palsy1091.00016130.812
    Other visual disturbances31270.71334.0360.861
Other symptoms
    Gait disturbances81770.53490950.376
    Dysarthria35341.0004550.00.617
    Vigilance disorder20180.83439460.400
    Disorder of the frontal brain14270.08638460.394
    Aphasia25210.69637300.481
    Speech reduction20250.54132520.017
    Apraxia24250.84731321.000
    Sensory abnormalities14320.01317340.017
    Hyperhidrosis13180.48718210.669
    Seizures690.53211210.105