Neurology: cardiac involvement in myotonic dystrophy: practice question

A General Medicine 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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General Medicine · Neurology: cardiac involvement in myotonic dystrophy

Sample item

A 30-year-old man has difficulty releasing his grip, frontal balding, bilateral ptosis, cataracts and testicular atrophy. His father had similar symptoms with onset at a later age. Which cardiac problem is most likely to cause sudden death in him?

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

The answer and the reasoning

D. Progressive conduction block and arrhythmia

Myotonic dystrophy type 1 is an autosomal dominant CTG expansion in the DMPK gene, with anticipation. Cardiac conduction defects and arrhythmias are the main cardiac risk, so regular ECG surveillance is needed, and pacemaker or defibrillator implantation may be required.

Option by option

  • A. Constrictive pericarditis

    Constrictive pericarditis is not a recognised feature of this disorder.

  • B. Obstructive hypertrophic cardiomyopathy

    Hypertrophic cardiomyopathy is associated with Friedreich ataxia rather than with myotonic dystrophy.

  • C. Dilatation of the aortic root

    Aortic root dilatation is characteristic of Marfan syndrome and not of myotonic dystrophy.

  • D. Progressive conduction block and arrhythmia (correct)

    Correct. Conduction disease and arrhythmia are the leading cardiac cause of death in myotonic dystrophy.

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 General Medicine question

What is the answer to this General Medicine question?

The best answer is D, Progressive conduction block and arrhythmia. Myotonic dystrophy type 1 is an autosomal dominant CTG expansion in the DMPK gene, with anticipation.

Why is "Constrictive pericarditis" not the answer?

Constrictive pericarditis is not a recognised feature of this disorder.

Why is "Obstructive hypertrophic cardiomyopathy" not the answer?

Hypertrophic cardiomyopathy is associated with Friedreich ataxia rather than with myotonic dystrophy.

Why is "Dilatation of the aortic root" not the answer?

Aortic root dilatation is characteristic of Marfan syndrome and not of myotonic dystrophy.

What concept does this question test?

It tests Neurology: cardiac involvement in myotonic dystrophy in General Medicine. Cardiac conduction defects and arrhythmias are the main cardiac risk, so regular ECG surveillance is needed, and pacemaker or defibrillator implantation may be required.

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: cardiac involvement in myotonic dystrophy?

For each disease know the classic presentation, the best test, and the first-line treatment. Then use the flashcards and the other General Medicine 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.