Objective: Characterize the syndrome resembling progressive supranuclear palsy (PSP) following aortic surgery.

Background: Mokri, et al. reported a PSP-like syndrome seemingly triggered by aortic surgery (2004).   Limited reports have surfaced in the interim.

Method: We searched the Mayo Clinic electronic medical record for patients evaluated for neurologic symptoms after cardiac-aortic surgery and identified 25 patients with this syndrome.   From this cohort, we describe the clinical features, neuroimaging and outcomes.

Results: Surgeries performed at multiple medical centers across the country were done with thoracic aortic bypass and deep hypothermia.  Surgery included aortic aneurysm, aortic valve repair, and/or aortic dissection repair, and was uncomplicated in 60% of cases without specific complications in the remaining patients. A typical triad was documented:  supranuclear gaze palsy (SNGP; 100%), gait imbalance (80%), and dysarthria (96%). Part or all of the triad presented prior to hospital discharge and stabilized over the course of days to weeks. A second phase of symptom worsening as well as onset of new symptoms developed up to a year later; this decline continued for up to several years before stabilization. Delayed epileptic seizures occurred in 32% of patients.  Brain MRI revealed only non-specific (age-related) findings.  FDG-PET in one patient revealed hypometabolism in the cerebellum, caudate nucleus and parieto-occipital regions; AV-1451 tau-PET did not show uptake in PSP regions.

Conclusion: This syndrome following adult thoracic aortic bypass surgery with deep hypothermia remains unexplained. It follows a biphasic course and is characterized by the triad of vertical supranuclear gaze palsy, unsteady gait, and a predominantly ataxic dysarthria.

References: Mokri B, Ahlskog JE, Fulgham JR, Matsumoto JY. Syndrome resembling PSP after surgical repair of ascending aorta dissection or aneurysm. Neurology. 2004:971-974.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/psp-like-syndrome-after-aortic-in-adults-mokri-syndrome/