Kuru plaques are seen in which of the following neurodegenerative disorders?
## **Core Concept**
Kuru plaques are a characteristic neuropathological feature associated with prion diseases, which are a group of infectious, fatal brain diseases. Prion diseases lead to the degeneration of brain tissue, resulting in a range of neurological symptoms. The presence of kuru plaques, also known as amyloid plaques, is particularly noted in the brains of individuals affected by these diseases.
## **Why the Correct Answer is Right**
The correct answer, Creutzfeldt-Jakob disease (CJD), is a prion disease that leads to rapid cognitive decline and is characterized neuropathologically by the presence of kuru plaques, also known as amyloid plaques. These plaques are composed of abnormally folded prion protein (PrP) and are a hallmark of prion diseases, which include CJD, bovine spongiform encephalopathy (BSE or mad cow disease), and kuru. The presence of these plaques disrupts normal brain function, leading to the clinical manifestations of the disease.
## **Why Each Wrong Option is Incorrect**
- **Option A:** Alzheimer's disease is characterized by the presence of amyloid-beta plaques and neurofibrillary tangles, not kuru plaques. While amyloid plaques are present in Alzheimer's, they are distinct from kuru plaques in composition and context.
- **Option B:** Parkinson's disease is primarily characterized by the loss of dopaminergic neurons in the substantia nigra and the presence of Lewy bodies, not kuru plaques.
- **Option C:** Huntington's disease is marked by neuronal loss in the basal ganglia and the presence of huntingtin protein aggregates, not kuru plaques.
## **Clinical Pearl / High-Yield Fact**
A key point to remember is that prion diseases, including CJD, are infectious and can be transmitted through contaminated tissue or medical procedures. The presence of kuru plaques is a diagnostic feature that can help differentiate CJD from other neurodegenerative disorders. It's also crucial to note that CJD has a very rapid progression compared to other neurodegenerative diseases.
## **Correct Answer:** . Creutzfeldt-Jakob disease