CNS: Creutzfeldt-Jakob disease: practice question

A Pathology question in the style of the NEET PG paper. Answer it first, then read why the right option is right and why each of the others is wrong.

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Pathology · CNS: Creutzfeldt-Jakob disease

Sample item

A 62-year-old woman has had rapidly progressive dementia over four months, with myoclonic jerks and periodic sharp wave complexes on EEG. What would brain histology show?

Original practice item, not a recalled exam question. Nothing here is recorded.

The answer and the reasoning

C. Spongiform vacuolation of the cortex with neuronal loss, gliosis and no inflammation

Creutzfeldt-Jakob disease is a prion disorder in which the normal cellular prion protein is converted to a beta-sheet rich, protease-resistant isoform that accumulates. The brain shows spongiform change, neuronal loss and astrocytosis without an inflammatory response. Disease is rapidly fatal and cannot be cured, and tissue is handled with special decontamination.

Option by option

  • A. Pick bodies in atrophic frontal and temporal lobes

    Pick bodies indicate frontotemporal dementia with early personality change.

  • B. Perivascular lymphocytic cuffs with microglial nodules

    Perivascular cuffing with microglial nodules indicates viral encephalitis, which is febrile and inflammatory.

  • C. Spongiform vacuolation of the cortex with neuronal loss, gliosis and no inflammation (correct)

    Correct. Spongiform change without inflammation fits prion disease with this clinical course.

  • D. Neurofibrillary tangles and senile plaques with hippocampal atrophy

    Tangles and plaques indicate Alzheimer disease, which progresses over years.

Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.

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Questions about this Pathology question

What is the answer to this Pathology question?

The best answer is C, Spongiform vacuolation of the cortex with neuronal loss, gliosis and no inflammation. Creutzfeldt-Jakob disease is a prion disorder in which the normal cellular prion protein is converted to a beta-sheet rich, protease-resistant isoform that accumulates.

Why is "Pick bodies in atrophic frontal and temporal lobes" not the answer?

Pick bodies indicate frontotemporal dementia with early personality change.

Why is "Perivascular lymphocytic cuffs with microglial nodules" not the answer?

Perivascular cuffing with microglial nodules indicates viral encephalitis, which is febrile and inflammatory.

Why is "Neurofibrillary tangles and senile plaques with hippocampal atrophy" not the answer?

Tangles and plaques indicate Alzheimer disease, which progresses over years.

What concept does this question test?

It tests CNS: Creutzfeldt-Jakob disease in Pathology. The brain shows spongiform change, neuronal loss and astrocytosis without an inflammatory response. Disease is rapidly fatal and cannot be cured, and tissue is handled with special decontamination.

How hard is this question?

We tag it as a multi-step question: it asks you to combine more than one idea. The tag is our own editorial judgement, not an official difficulty rating, so read the reason for every option, not only the answer.

How should I study CNS: Creutzfeldt-Jakob disease?

Learn each disease as a bundle: cause, key histology, markers, and the clinical hallmark. Then use the flashcards and the other Pathology questions on this site to test what stuck.

Is this a real NEET PG question?

No. It is an original practice item (editorial status: sample, version 1). NextPrep does not publish recalled or leaked questions, and independent faculty review of this item has not been completed yet, so confirm important facts in a standard textbook.