Neurodegenerative Disorders · Pathology

Huntington disease is caused by an expanded trinucleotide repeat (CAG) in the HTT gene. Which specific brain structure undergoes the most profound atrophy in this condition?

  1. Hippocampus
  2. Substantia nigra
  3. Caudate nucleus
  4. Cerebellum
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Correct answer: Caudate nucleus

The striatum, particularly the caudate nucleus and to a lesser extent the putamen, undergoes severe atrophy in Huntington disease. This loss of inhibitory GABAergic neurons leads to the characteristic choreiform movements.

Difficulty: Medium Question 5 of 20

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