Major or Mild Neurocognitive Disorder due to Prion Disease

Human prion disease is a rare but devastating cause of neurocognitive disorder (NCD). There are three forms of human prion disease, as categorized by neurologist Michael Geschwind: (1) spontaneous (sporadic), (2) genetic (familial), and (3) acquired (infectious/transmitted). They constitute a group of neurodegenerative diseases with shared neuropathological characteristics, and the acquired category is often referred to as transmissible spongiform encephalopathies.

Prion disease affects multiple brain regions, and all three categories of human prion disease may have multiple possible clinical presentations. For this reason, human prion disease has been known as the “great imitator.” The clinical diagnosis of human prion disease is often challenging due to the multiplicity of clinical presentations and the rarity of prion disease. Those with prion disease typically have cognitive impairment, and most ...