Neurology: Duchenne muscular dystrophy: practice question

A Pediatrics 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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Pediatrics · Neurology: Duchenne muscular dystrophy

Sample item

A 5-year-old boy uses his hands to climb up his thighs to stand, has calf pseudohypertrophy and a CK of 20,000 IU/L. What is the finding called and the likely deficiency?

ATrendelenburg sign; dysferlinBGower sign; acid maltaseCBabinski sign; dystrophinDGower sign; dystrophin deficiency

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

The answer and the reasoning

D. Gower sign; dystrophin deficiency

Duchenne muscular dystrophy is an X-linked frameshift mutation producing absent dystrophin. Proximal weakness gives the Gower manoeuvre, and CK is markedly raised. Glucocorticoids prolong ambulation, and cardiomyopathy and respiratory failure determine survival.

Option by option

A. Trendelenburg sign; dysferlin
Trendelenburg sign relates to hip abductor weakness and dysferlin is related to other limb-girdle disease.
B. Gower sign; acid maltase
Acid maltase deficiency is Pompe disease.
C. Babinski sign; dystrophin
Babinski sign is a pyramidal sign.
D. Gower sign; dystrophin deficiency (correct)
Correct. Gower sign with dystrophin deficiency.

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.

Questions about this Pediatrics question

What is the answer to this Pediatrics question?

The best answer is D, Gower sign; dystrophin deficiency. Duchenne muscular dystrophy is an X-linked frameshift mutation producing absent dystrophin.

Why is "Trendelenburg sign; dysferlin" not the answer?

Trendelenburg sign relates to hip abductor weakness and dysferlin is related to other limb-girdle disease.

Why is "Gower sign; acid maltase" not the answer?

Acid maltase deficiency is Pompe disease.

Why is "Babinski sign; dystrophin" not the answer?

Babinski sign is a pyramidal sign.

What concept does this question test?

It tests Neurology: Duchenne muscular dystrophy in Pediatrics. Proximal weakness gives the Gower manoeuvre, and CK is markedly raised. Glucocorticoids prolong ambulation, and cardiomyopathy and respiratory failure determine survival.

How hard is this question?

We tag it as an application question: it asks you to use a fact on a patient or a finding. 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 Neurology: Duchenne muscular dystrophy?

Memorise milestones and the vaccine schedule as tables and revise them often. Then use the flashcards and the other Pediatrics 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.