Spine imaging: Hirayama disease: 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 · Spine imaging: Hirayama disease

Sample item

A 20-year-old man has progressive asymmetric weakness and wasting of one hand and forearm with sparing of brachioradialis, worsened by cold. Neutral cervical MRI is normal, but MRI in neck flexion shows forward displacement of the posterior dural sac with an enhancing epidural crescent. What is the diagnosis?

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

The answer and the reasoning

C. Hirayama disease

Hirayama disease, a juvenile asymmetric distal upper limb amyotrophy, is caused by forward shift of the dural sac during neck flexion that compresses the lower cervical cord. It is more common in young Asian men and the flexion MRI is diagnostic. Spondylosis occurs later, syringomyelia shows a cavity, and ALS has upper motor neuron signs.

Option by option

  • A. Amyotrophic lateral sclerosis

    ALS has upper motor neuron signs and no flexion-related cord displacement.

  • B. Cervical spondylotic myelopathy

    Cervical spondylotic myelopathy occurs in older patients with degenerative canal narrowing.

  • C. Hirayama disease (correct)

    Correct. Flexion MRI showing forward dural displacement is diagnostic of Hirayama disease.

  • D. Syringomyelia

    Syringomyelia shows a fluid cavity in the cord with sensory dissociation.

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 C, Hirayama disease. Hirayama disease, a juvenile asymmetric distal upper limb amyotrophy, is caused by forward shift of the dural sac during neck flexion that compresses the lower cervical cord.

Why is "Amyotrophic lateral sclerosis" not the answer?

ALS has upper motor neuron signs and no flexion-related cord displacement.

Why is "Cervical spondylotic myelopathy" not the answer?

Cervical spondylotic myelopathy occurs in older patients with degenerative canal narrowing.

Why is "Syringomyelia" not the answer?

Syringomyelia shows a fluid cavity in the cord with sensory dissociation.

What concept does this question test?

It tests Spine imaging: Hirayama disease in Radiology. It is more common in young Asian men and the flexion MRI is diagnostic. Spondylosis occurs later, syringomyelia shows a cavity, and ALS has upper motor neuron signs.

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 Spine imaging: Hirayama disease?

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.