Final answer:
Major or Mild Neurocognitive Disorder due to Prion disease is primarily characterized by rapid cognitive decline, which includes memory loss and a variety of neurological symptoms due to brain tissue damage caused by abnormal prion proteins.
Step-by-step explanation:
Major or Mild Neurocognitive Disorder due to Prion disease is characterized by rapid cognitive decline. The symptoms associated with Prion diseases can include disruptive memory loss, confusion about time or place, difficulty with planning and executing tasks, poor judgment, personality changes, slowed movements, balance and posture problems, rigid muscles, speech changes, and psychological symptoms such as dementia. Creutzfeldt-Jakob disease (CJD), a type of Prion disease, leads to degeneration of neural tissue, causing the brain to exhibit sponge-like lesions visible on scans, which result from abnormal formations of prion protein.
Prions, the causative agents of variant CJD and other diseases, are misfolded proteins that can induce normal proteins to also misfold, leading to the accumulation of prions within nerve cells. This aggregation and subsequent neuronal damage give the brain a spongy appearance and result in various neurological symptoms. These symptoms continue to worsen over time, ultimately leading to coma and death. Humans can contract variant CJD through consumption of infected meat or exposure to nervous tissue containing prions. The gold standard for diagnosing these conditions involves histological examination of brain biopsies, but new assays like RT-QuIC are providing hope for earlier detection.