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Progressive supranuclear palsy — SCE Neurology MCQ

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EasyMovement DisordersProgressive supranuclear palsySCE Neurology

A 27-year-old man presented with early backward falls, axial rigidity and difficulty looking down. On examination, there was vertical supranuclear gaze palsy and frontal dysexecutive change. Initial investigations showed: MRI showed midbrain atrophy with relative pontine preservation. What is the most likely underlying mechanism?

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Correct answer: E — four-repeat tau neurodegeneration

four-repeat tau neurodegeneration is the best answer because the vignette describes Progressive supranuclear palsy: early falls plus vertical gaze palsy suggest PSP. Post-infectious immune polyradiculoneuropathy is plausible in a neighbouring presentation, but the chronology, examination or investigation pattern does not match the key discriminator here. Raised intracranial pressure from impaired CSF dynamics and Small-vessel lipohyalinosis are less appropriate because they would require different localisation, timing or test findings; Prion protein misfolding would fit a different syndrome. Clinical pearl: Parkinson's disease usually has later falls and better levodopa response.

Reference: NICE NG71; Movement Disorder Society consensus criteria