Progressive supranuclear palsy

Case contributed by Frank Gaillard
Diagnosis almost certain

Presentation

2 year history of increasing Parkinsonism, clinically suggestive of a Parkinson's Plus syndrome.

Patient Data

Age: 70
Gender: Male

Prominent atrophy of the midbrain, and superior colliculus. The basal ganglia are normal. No changes in the putamen that are usually associated with MSA-P. No changes in the pons to suggest MSA-C. No abnormal iron accumulation noted.

Volume loss also involves the left frontal lobe and insula. Dilated lateral, 3rd and 4th ventricles with prominent bilateral Sylvian fissures. Small subependymal cyst noted along the right frontal horn. No definite transependymal edema demonstrated. The aqueduct is patent with prominent CSF flow void. Quantitative measurements of flow are within normal range (not shown). No downward bowing of the floor of the third ventricle.

No abnormal diffusion restriction.

Midbrain to pons ratio: 0.15 MRPI: 28.25

Conclusion:

Findings are most in keeping with PSP.

Case Discussion

The clinical picture, in this case, was consistent with progressive supranuclear palsy (PSP), and imaging was supportive of this diagnosis. Note, that the presence of left frontal lobe and insular atrophy also fits well as there is an overlap of PSP with non-fluent aphasia variant of frontotemporal dementia (nfvFTD) as both conditions are associated with accumulation of tau (see tauopathies). 

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