What is the human-inherited neurodegenerative disease caused by prions that is characterized by rapid cognitive decline?

• A. Gerstmann-Straüssler-Scheinker disease
• B. Familial fatal insomnia
• C. Kuru
• D. Creutzfeldt-Jakob disease

Answer: (D)

The human-inherited neurodegenerative disease caused by prions that is characterized by rapid cognitive decline is Creutzfeldt-Jakob disease. This disease is a form of transmissible spongiform encephalopathy, which is a group of progressive neurodegenerative disorders caused by infectious agents known as prions. These prions lead to the formation of abnormal protein aggregates in the brain, causing neuronal damage and eventually resulting in severe cognitive impairment and other neurological symptoms.

Creutzfeldt-Jakob disease can present in various forms, including sporadic, hereditary, and acquired forms. The rapid progression of symptoms, including severe mental decline, motor dysfunction, and ultimately leading to coma and death, is a hallmark of this condition. The cognitive decline can occur over a matter of months, distinguishing it from other neurodegenerative diseases that may have a more gradual onset.

While the other disorders mentioned are also associated with prions and neurodegeneration, they often have different characteristics or slower progression rates. For example, Gerstmann-Straüssler-Scheinker disease is usually more about ataxia and cognitive decline over a longer period, familial fatal insomnia primarily leads to sleep disturbances, and kuru is associated more with prion disease in specific populations due