True about prion protein diseases is all, except -
Most patients (~90%) with Cruetzfeldt-Jacob Disease (CJD) exhibit myoclonus that appears at various times throughout the illness.
Unlike other involuntary movements, myoclonus persists during sleep. Startle myoclonus elicited by loud sounds or bright lights is frequent
Myoclonus is seen in 10% of the patients
Caused by infectious protein
Brain biopsy is diagnostic
Commonly manifests as dementia