Huntington disease neuroanatomy — MRCPsych Paper A MCQ

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EasyNeuroanatomyHuntington disease neuroanatomyMRCPsych Paper A

A 43-year-old man develops chorea, irritability and progressive executive dysfunction. His father died after experiencing a similar illness. Which brain structure is characteristically affected early and prominently in this disorder?

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Correct answer: A — Caudate nucleus

The correct answer is **D, caudate nucleus**. The combination of midlife-onset chorea, behavioural change, executive dysfunction and an affected parent is characteristic of autosomal dominant Huntington disease. Neurodegeneration particularly affects medium spiny neurons of the striatum, including the caudate nucleus and putamen; caudate atrophy is an early and characteristic finding. Hippocampal pathology is more strongly associated with disorders causing prominent episodic-memory impairment. Inferior olivary lesions produce cerebellar or palatal movement abnormalities rather than this syndrome. The locus coeruleus is a noradrenergic brainstem nucleus and is not the principal site of Huntington pathology. Optic chiasmal lesions characteristically produce visual-field defects.

Reference: Han et al. Gray matter alterations in Huntington's disease: A meta-analysis of VBM neuroimaging studies. 2024. https://pubmed.ncbi.nlm.nih.gov/38953592/