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Physiology · Neurophysiology and special senses: Huntington disease pathway
Sample itemA 42-year-old man has choreiform movements and cognitive decline, with atrophy of the caudate nucleus on imaging. Which basal ganglia change underlies the chorea?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
A. Early loss of indirect pathway neurons, which disinhibits the thalamus
In Huntington disease, the GABAergic medium spiny neurons of the indirect pathway die first. This reduces inhibition of the thalamus through the globus pallidus externa and subthalamic nucleus, and excess motor activity (chorea) results. In Parkinson disease nigrostriatal dopamine loss reduces movement.
Option by option
A. Early loss of indirect pathway neurons, which disinhibits the thalamus (correct)
Correct. Indirect pathway loss disinhibits the thalamus and causes chorea.
B. Loss of nigrostriatal dopamine, which reduces direct pathway output
Nigrostriatal dopamine loss describes Parkinson disease, with rigidity and bradykinesia.
C. Loss of dentate nucleus output from the cerebellum
Dentate nucleus lesions cause intention tremor and ataxia.
D. Destruction of the subthalamic nucleus
Subthalamic nucleus destruction produces hemiballismus on the opposite side, not chorea with caudate atrophy.
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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