Neurodegenerative proteinopathy - the best known of the human prion diseases
Aetiology
- Sporadic - patients 60 years
- Variant - patients 20 years, due to BSE exposure
- Iatrogenic - patients 30 years
- Genetic - affects any age group
Clinical presentation
- Clinical findings include myoclonus, visual disturbances and cerebellar, pyramidal and extrapyramidal signs in addition to rapidly progressive cognitive and functional impairment
Management
- Supportive
Made with Bullet