Neuroimaging: adrenoleukodystrophy: practice question

A Radiology 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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Radiology · Neuroimaging: adrenoleukodystrophy

Sample item

A 7-year-old boy has deteriorating school performance, visual problems and skin pigmentation. MRI shows symmetric T2 hyperintensity in the parieto-occipital white matter with a peripheral rim of contrast enhancement. What is the diagnosis?

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

The answer and the reasoning

A. X-linked adrenoleukodystrophy

Classic X-linked ALD begins in the posterior white matter and spreads forward, with an enhancing leading edge of active demyelination. Adrenal insufficiency explains the pigmentation, and very long chain fatty acids are raised.

Option by option

  • A. X-linked adrenoleukodystrophy (correct)

    Correct. Posterior predominant demyelination in a boy with adrenal signs is X-ALD.

  • B. Krabbe disease

    Krabbe disease presents in infants with thalamic density.

  • C. Canavan disease

    Canavan disease causes macrocephaly with diffuse white matter changes.

  • D. Metachromatic leukodystrophy

    Metachromatic leukodystrophy shows a tigroid pattern with frontal predominance and no enhancement.

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 Radiology question

What is the answer to this Radiology question?

The best answer is A, X-linked adrenoleukodystrophy. Classic X-linked ALD begins in the posterior white matter and spreads forward, with an enhancing leading edge of active demyelination.

Why is "Krabbe disease" not the answer?

Krabbe disease presents in infants with thalamic density.

Why is "Canavan disease" not the answer?

Canavan disease causes macrocephaly with diffuse white matter changes.

Why is "Metachromatic leukodystrophy" not the answer?

Metachromatic leukodystrophy shows a tigroid pattern with frontal predominance and no enhancement.

What concept does this question test?

It tests Neuroimaging: adrenoleukodystrophy in Radiology. Adrenal insufficiency explains the pigmentation, and very long chain fatty acids are raised.

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 Neuroimaging: adrenoleukodystrophy?

Learn signs with the image, and the disease they point to. Then use the flashcards and the other Radiology 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.