- Transmissible Spongiform Encephalopathy (TSE)
- Infectious variant (uncommon) can be spread by contact w/infected brain matter (via prions)
- Most common is sporadic form, which is non-infectious
- Also a hereditary form
- Avg age onset in 60s
- Though can affect old & young (<50)
- Rapidly progressive decline in memory, cognition, thinking
- Other manifestations include aphasia, disorientation, apraxia, visual-spatial impairment
- Onset to death w/in 1y
- Coordination problems and sudden jerking movements (myoclonus) may come first
- Coordination problems and myoclonus
- Sometimes ataxia, nystagmus
- Sometimes hyperreflexia