Question 1

Q: A 65-year-old man with no significant medical history begins to have memory loss and personality changes. Rapidly, over the next few months his symptoms increase in severity. He experiences a rapid mental deterioration associated with sudden, jerking movements, particularly in response to being startled. He has gait disturbances as well. Eventually, he lapses into a coma and dies approximately ten months after the onset of symptoms. Which of the following would most likely be seen on autopsy of the brain in this patient?

Spongiform changes. ***Spongiform changes*** - This patient's **rapidly progressive dementia**, associated with **myoclonus (jerking movements)** and **gait disturbances**, culminating in death within months, is highly suggestive of **Creutzfeldt-Jakob disease (CJD)**. - CJD is characterized by the accumulation of abnormally folded prion proteins (PrPSc), leading to **spongiform degeneration** (vacuolation) of neurons and neuropil, **neuronal loss**, and **astrogliosis** in the brain. *Neurofibrillary tangles* - **Neurofibrillary tangles**, composed primarily of hyperphosphorylated **tau protein**, are a hallmark of **Alzheimer's disease**. - Alzheimer's disease typically has a much **slower progression** over several years, unlike the rapid deterioration seen in this patient. *Amyloid plaques* - **Amyloid plaques**, formed by the extracellular deposition of **beta-amyloid protein**, are also characteristic features of **Alzheimer's disease**. - Like neurofibrillary tangles, amyloid plaques are associated with a chronic, progressive course of dementia, not the **rapidly fatal trajectory** described. *Vascular lesions* - **Vascular lesions**, such as infarcts or hemorrhages, are the underlying pathology in **vascular dementia**. - While vascular dementia can cause cognitive decline, its presentation often involves **step-wise deterioration** and may be associated with focal neurological deficits, which are not the primary features here. *Lewy bodies* - **Lewy bodies**, which are intracellular cytoplasmic inclusions of **alpha-synuclein protein**, are characteristic of **Lewy body dementia** and **Parkinson's disease**. - Lewy body dementia presents with fluctuating cognition, visual hallucinations, and parkinsonism, but typically does not show the **rapid progression** and prominent myoclonus seen in CJD.

Question

A 65-year-old man with no significant medical history begins to have memory loss and personality changes. Rapidly, over the next few months his symptoms increase in severity. He experiences a rapid mental deterioration associated with sudden, jerking movements, particularly in response to being startled. He has gait disturbances as well. Eventually, he lapses into a coma and dies approximately ten months after the onset of symptoms. Which of the following would most likely be seen on autopsy of the brain in this patient?

Accepted Answer

Spongiform changes

Answer

Neurofibrillary tangles

Answer

Amyloid plaques

Answer

Vascular lesions

Answer

Lewy bodies

General Pathology — MCQs

General Pathology — MCQs

On this page

Practice by Chapter

Want unlimited practice?