Overview
Definition
Rapidly progressive, invariably fatal prion disease.
Epidemiology
Varies globally depending on genetics, environment, or exposure.
Etiology & Risk Factors
- Misfolded prion protein (PrPSc).
Clinical Symptoms
- Rapidly progressive dementia
- myoclonus
- ataxia.
Clinical Approach
Diagnosis
- MRI (cortical ribboning)
- CSF 14-3-3 protein
- EEG (periodic sharp waves).
Management
- Supportive care only
- invariably fatal within 1 year.
Complications
- Death.