Table 1 The clinical findings of probable CJD patients in Korea with codons 178, 200-129, and 232 mutations.
Patient | Case 1 | Case 2 | Case 3 |
|---|---|---|---|
Age of onset | 67 | 58 | 65 |
Mutation | D178N-129M | E200K-129MV | M232R |
Sex | M | M | M |
Symptoms of onset | Progressive gait disturbance Dysarthria Extrapyramidal sign | Gait disturbance Confused mentality Dysarthria Agitation Myoclonus | Memory decline Gait disturbance |
Total clinical duration | Alive (27 months)* | 3 months | 16 months |
MRI imaging (DWI or FLAIR) | High signal intensities in both parietal and occipital gyri | High signal intensity on bilateral frontotemporoparietal area and caudate nucleus | High signal intensities from the cortex of the parieto-occipital and the temporal lobes |
EEG finding | Normal | Sharp or spike and slow waves | Diffuse theta to delta range slow waves |
14-3-3 protein in CSF | Positive | Positive | Positive |