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Question Date: 17/07/2023

Question: A 77-year-old man without significant medical history presents for evaluation of falls and dysarthria. He started developing balance problems 1 to 2 years ago, which have been worsening significantly. He has fallen a number of times over the past year. His primary care physician evaluated him with a head CT that did not demonstrate any significant abnormalities. Over the past 3 months, he has developed slurring of his speech as well as difficulty swallowing. On examination, he demonstrates slowing of vertical saccades, decreased blink rate, square-wave jerks, Myerson sign, bilateral symmetric bradykinesia, increased neck tone, normal tone in the extremities, and slow gait with positive pull test<br><br>What is the most likely finding expected on MRI in this patient?

Options:

• Extensive subcortical white matter disease
• Iron deposition in the putamen
• Knifelike frontal lobe atrophy
• Midbrain atrophy

Correct Answer: Midbrain atrophy

Explaination: The most likely diagnosis in this patient is progressive supranuclear palsy. Supporting features include early falls, axial greater than appendicular rigidity, slowing of vertical saccades with square-wave jerks, and frontal release signs. Progressive supranuclear palsy is associated with midbrain atrophy on MRI, creating the classic hummingbird sign. Extensive subcortical white matter disease is typical of vascular dementia and vascular parkinsonism. Iron deposition in the putamen is seen in multiple system atrophy. Knifelike frontal lobe atrophy may be seen in frontotemporal dementias.

Reference: Williams DR, Litvan I. Parkinsonian syndromes. Continuum (Minneap Minn). 2013 Oct;19(5 Movement Disorders):1189-212. doi: 10.1212/01.CON.0000436152.24038.e0. PMID: 24092286; PMCID: PMC4234134