Session Information
Date: Tuesday, September 24, 2019
Session Title: Parkinsonisms and Parkinson-Plus
Session Time: 1:45pm-3:15pm
Location: Agora 3 West, Level 3
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.
To cite this abstract in AMA style:
S. Tisel, J. Ahlskog, J. Matsumoto, J. Duffy, K. Josephs. PSP-like syndrome after aortic in adults (Mokri syndrome) [abstract]. Mov Disord. 2019; 34 (suppl 2). https://www.mdsabstracts.org/abstract/psp-like-syndrome-after-aortic-in-adults-mokri-syndrome/. Accessed November 21, 2024.« Back to 2019 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/psp-like-syndrome-after-aortic-in-adults-mokri-syndrome/