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Progressive supranuclear palsy — MRCPsych Paper A MCQ

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HardNeuropathologyProgressive supranuclear palsyMRCPsych Paper A

A 70-year-old man develops recurrent unprovoked falls within the first year of illness, slowing followed by palsy of vertical gaze, symmetrical axial rigidity and frontal executive dysfunction. At post-mortem examination, which neuropathological finding would be most characteristic?

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Correct answer: C — Four-repeat tau-positive globose tangles and tufted astrocytes, predominantly in basal ganglia and brainstem

The presentation is classic progressive supranuclear palsy–Richardson syndrome: early falls, vertical supranuclear gaze palsy, symmetrical axial rigidity and frontal executive dysfunction. PSP is a four-repeat tauopathy characterised by neuronal globose neurofibrillary tangles, oligodendroglial coiled bodies and tufted astrocytes, with prominent basal ganglia, diencephalic and brainstem involvement. Option A describes corticobasal degeneration, another four-repeat tauopathy distinguished by astrocytic plaques, ballooned neurons and predominantly asymmetric cortical disease. Option B describes multiple system atrophy. Option D describes Alzheimer disease, in which tau forms paired helical filaments alongside amyloid-beta plaques. Option E describes Pick disease, a three-repeat tauopathy associated with circumscribed frontotemporal atrophy.

Reference: Mahale R et al. Progressive supranuclear palsy: Neuropathology, clinical presentation, diagnostic challenges, management, and emerging therapies. 2024. https://pubmed.ncbi.nlm.nih.gov/38908985/