Creutzfeldt-Jakob Disease (CJD):
Prions are infectious agents that is a small proteinaceous particle with no nucleus. Most common is CJD. Annual incidence of about 1/1 000 000.
Develop a spongiform encephalopathy; cerebral atrophy; neuronal loss.
Clinical features – gradual onset of dementia; weakness and stiffness of limbs (extrapyramidal lesion signs); myoclonus; also can get anxiety, fatigue, headache, dizziness.
Management – 90% fatal within 1 year of diagnosis