Neuroimaging: Leigh syndrome: 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: Leigh syndrome

Sample item

An infant has developmental regression and lactic acidosis. MRI shows symmetrical T2-hyperintense lesions in the putamina and periaqueductal brainstem, and spectroscopy shows a lactate peak. What is the diagnosis?

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

The answer and the reasoning

B. Leigh syndrome

Leigh syndrome is a mitochondrial subacute necrotising encephalopathy with symmetric basal ganglia and brainstem lesions and elevated lactate. Wernicke encephalopathy has a similar distribution but arises from thiamine deficiency in adults.

Option by option

  • A. Hypoxic-ischaemic encephalopathy

    Hypoxic-ischaemic injury has a history of a perinatal or acute event rather than progressive regression.

  • B. Leigh syndrome (correct)

    Correct. Symmetric basal ganglia and brainstem lesions with lactate in an infant indicate Leigh syndrome.

  • C. Wilson disease

    Wilson disease causes copper-related changes of the putamen and thalamus in adolescents, not lactic acidosis in infancy.

  • D. Wernicke encephalopathy

    Wernicke encephalopathy affects the mamillary bodies and medial thalami in alcohol-related or malnourished adults.

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 B, Leigh syndrome. Leigh syndrome is a mitochondrial subacute necrotising encephalopathy with symmetric basal ganglia and brainstem lesions and elevated lactate.

Why is "Hypoxic-ischaemic encephalopathy" not the answer?

Hypoxic-ischaemic injury has a history of a perinatal or acute event rather than progressive regression.

Why is "Wilson disease" not the answer?

Wilson disease causes copper-related changes of the putamen and thalamus in adolescents, not lactic acidosis in infancy.

Why is "Wernicke encephalopathy" not the answer?

Wernicke encephalopathy affects the mamillary bodies and medial thalami in alcohol-related or malnourished adults.

What concept does this question test?

It tests Neuroimaging: Leigh syndrome in Radiology. Wernicke encephalopathy has a similar distribution but arises from thiamine deficiency in adults.

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: Leigh syndrome?

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.