Table 1.
Comparison of typical VV1 characteristics and our case*.
| Characteristic | Our case | Typical VV1 |
|---|---|---|
| Subject demographic, disease course | ||
| Sex | Female | Male predominance |
| Age of onset (years) | 61 | 44 (19–55) |
| Disease duration (months) | 32 | 21 (17–49) |
| Clinical features | ||
| Cognitive problemsa | Yes | Yes |
| Aphasia | Yes | Yes |
| Apraxia | Yes | Occasionally |
| Visual disturbancesb | Yes, early | Occasionally |
| Limb or gait ataxia | No | Yes |
| Myoclonus | Yes | Yes |
| Pyramidal symptomsc | Yes, rigidity | Yes |
| Psychiatric symptomsd | Yes, early, agitation, aggressions | Yes, regression, fear, aggressions |
| EEG | ||
| PSWCse | No | No |
| MRI | ||
| Widespread cortical signal involvement | Yes, right predominance | Yes |
| Basal ganglia signal increase | Yes, caudate and putamen | Sometimes |
| Laboratory findings | ||
| 14-3-3 positive | Yes | Yes |
| Tau | Yes, significantly elevated | Yes, significantly elevated |
| RT-QuIC | Negative | Unknown -mostly negative |
| Neuropathological features | Spongiform degeneration affecting cortical and subcortical regions | Severe spongiform changes in the cerebral cortex and striatum |
*Typical characteristics based on published findings from Meissner et al. (12).
aMemory loss, dementia, and confusion.
bVisual loss, field defect, blindness, and distortion.
cRigidity or spasticity.
dAnxiety, depression, aggression, psychosis, personality changes, and regression.
ePSWCs – periodic sharp wave complexes on electroencephalography.