VARIANT CREUTZ FELDT JACOB DISEASE
- young patients <30 years*
Rapid mental deterioration and dementia
myoclonus
psychological symptoms- anxiety, withdrawal
akinetic mutism
MRI- HOCKEY STICK SIGN- posterior thalamic changes (bilateral pulvinar high intensity signals) picture
Tonsil biopsy- Prion proteins (picture)
Px. Poor. Death in 13 months
Treatment- none
Symptomatic. Palliative.