- prions are glycoproteins which codes for a membrane protein
- definitive diagnosis is post mortem neuropathological examination
- tonsillar biopsy and CSF 14-3-3 (marker of rapid neuronal destruction) helps in diagnosis
New variant CJD
- young adults (mean age 29) presents with painful sensory symptoms, psychiatric symptoms, ataxia, dementia, myoclonus, upgaze paresis and chorea
- MRI : high signal on T2 weighted image in pulvinar (posterior thalamus)
- EEG normal
Sporadic CJD
- older age, shorter disease duration
- rapidly progressive dementia, cerebellar ataxia, pyramidal and extrapyramidal signs and myoclonus
- EEG : triphasic waves
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