Eating and sleep disorders: fatal familial insomnia: practice question

A Psychiatry 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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Psychiatry · Eating and sleep disorders: fatal familial insomnia

Sample item

A 50-year-old man develops progressive, untreatable insomnia with sweating, tachycardia, hypertension and myoclonus, followed by dementia within a year. His father and an uncle died of a similar illness. Autopsy would most likely show degeneration of which structure?

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

The answer and the reasoning

B. Thalamus

Fatal familial insomnia is an autosomal dominant prion disease with a mutation in the prion protein gene. It causes untreatable insomnia, autonomic overactivity, motor signs and dementia, with prominent atrophy of the anterior ventral and mediodorsal thalamic nuclei.

Option by option

  • A. Cerebellar vermis

    Cerebellar vermis atrophy occurs in alcoholic cerebellar degeneration.

  • B. Thalamus (correct)

    Correct. Thalamic degeneration is the pathological hallmark.

  • C. Substantia nigra

    Substantia nigra degeneration causes Parkinson disease.

  • D. Hippocampus

    Hippocampal atrophy is characteristic of Alzheimer disease and not of this prion disease.

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 Psychiatry question

What is the answer to this Psychiatry question?

The best answer is B, Thalamus. Fatal familial insomnia is an autosomal dominant prion disease with a mutation in the prion protein gene.

Why is "Cerebellar vermis" not the answer?

Cerebellar vermis atrophy occurs in alcoholic cerebellar degeneration.

Why is "Substantia nigra" not the answer?

Substantia nigra degeneration causes Parkinson disease.

Why is "Hippocampus" not the answer?

Hippocampal atrophy is characteristic of Alzheimer disease and not of this prion disease.

What concept does this question test?

It tests Eating and sleep disorders: fatal familial insomnia in Psychiatry. It causes untreatable insomnia, autonomic overactivity, motor signs and dementia, with prominent atrophy of the anterior ventral and mediodorsal thalamic nuclei.

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 Eating and sleep disorders: fatal familial insomnia?

Learn each diagnosis by its core features and its first-line treatment. Then use the flashcards and the other Psychiatry 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.