| MICROBIOLOGY AND IMMUNOLOGY ON-LINE |
Classical CJD and Variant CJD: Similar names, different conditions
from Health Canada
| Classical CJD | Variant CJD (vCJD) | |
| Age Range | onset 45 - 75 years of age | onset 12 - 74 years of age |
| Average Age | 60 years of age | 28 years of age |
| Average Duration | 4 - 6 months | 8 - 38 months |
| Symptoms (in order of progression) |
1 - rapid progressive dementia 2 - involuntary movements (myoclonus, chorea, dystonia) 3 - akinetic mutism 4 - extrapyramidal symptoms 5 - ataxia 6 - pyramidal signs 7 - cortical blindness |
1 - psychological symptoms (anxiety,
depression, withdrawal) 2 - ataxia (with persistent dysesthesia) 3 - involuntary movements (myoclonus, chorea, dystonia) 4 - dementia 5 - akinetic mutism |
| CT | cerebral/cerebellar atrophy | usually normal |
| MRI | putamen and caudate hyperintensity | bilateral pulvinar signal (77%) (see pop-up box on main slow virus page) |
| CSF | normal | normal (can have high protein) |
| 14-3-3 (CSF) | positive (>80%) | positive (50%) |
| EEG | pseudoperiodic sharp waves (50-70%) | normal (or non-specific low waves) |
| DNA (at codon 129) | met/met 71% met/val 13% val/val 16% |
met/met 100% |
| Pathology - brain |
Both classical and variant CJD: Spongiform changes, neuronal loss,
astrocytosis, protease resistant PrP
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| PrP amyloid plaques NOT seen | amyloid plaques | |
| Pathology - tonsil | normal | positive immuno-cytochemistry for PrPsc |