Letters

Progressive Supranuclear Palsy-Like Syndrome After Aortic Aneurysm Repair: A Case Series

Sirisha Nandipati1*, Janet C. Rucker1,2 and Steven J. Frucht1

1Department of Neurology Icahn School of Medicine at Mount Sinai, New York, New York, United States of America, 2Ophthalmology Icahn School of Medicine at Mount Sinai, New York, New York, United States of America

Abstract

The syndrome of progressive supranuclear palsy-like syndrome is a rare complication of ascending aortic aneurysm repair. We report two patients with videos and present a table of prior reported cases. To our knowledge there is no previously published video of this syndrome. The suspected mechanism is brainstem injury though neuroimaging is often negative for an associated infarct. We hope our report will increase recognition of this syndrome after aortic surgery, especially in patients with visual complaints.

Keywords: Progressive supranuclear palsy, supranuclear gaze palsy, aortic aneurysm repair

Citation: Nandipati S, Rucker JC, Frucht SJ. Progressive supranuclear palsy-like syndrome after aortic aneurysm repair: a case series. Tremor Other Hyperkinet Mov 2013; 3: http://tremorjournal.org/article/view/201

*To whom correspondence should be addressed. E-mail: Sirisha.nandipati@mssm.edu

Editor: Elan D. Louis, Columbia University, United States of America

Received: September 16, 2013 Accepted: November 11, 2013 Published: December 10, 2013

Copyright: © 2013 Nandipati et al. This is an open-access article distributed under the terms of the Creative Commons Attribution–Noncommercial–No Derivatives License, which permits the user to copy, distribute, and transmit the work provided that the original author(s) and source are credited; that no commercial use is made of the work; and that the work is not altered or transformed.

Funding: None.

Financial Disclosures: None.

Conflict of Interest: The authors report no conflict of interest.

Introduction

We present and demonstrate by video two unusual patients with a progressive supranuclear palsy-like syndrome following ascending aortic aneurysm repair. While previous patients have been reported with the disorder, to our knowledge no video has been published. The rarity of the condition and the availability of videos of both patients may be of value to readers of the journal.

Cases

A 25-year-old man with a family history of medial arterial dissections developed severe dysarthria and severe saccadic gaze palsy after a complicated repair of an ascending aortic aneurysm (Video 1). He noticed his impaired vision in the days following the repair. He was completely unable to generate saccades, and navigated by turning his head to fix a target and then tracking it. Mild parkinsonism with masked facies was also present. He also had a slight delay in reopening his eyes after forcefully closing them, suggestive of dystonia. Brain magnetic resonance imaging (MRI) revealed hyperintensities in the splenium of the corpus callosum and left frontal lobe. He ultimately succumbed 2 years later to a chronic bacterial infection of the chest cavity.

Our second patient, a 53-year-old man with hypertension and hyperlipidemia, underwent two sequential repairs of a dissecting ascending aortic aneurysm. After his first repair, he had right occipital infarct seen on MRI and several transient ischemic attack-like episodes. During the following 6 weeks he developed prominent dysarthria, dysphagia, and gait imbalance with a left homonymous hemianopia. After his second repair for progressive dilatation of the proximal descending thoracic aorta, he had progression of his neurologic symptoms, becoming nearly anarthric and also requiring a walker to walk. He had no response to Sinemet 25/100 mg three times daily. A horizontal and vertical saccadic gaze palsy was present; however, there was some preservation of ability to generate rightward voluntary eye movements (Video 2). He had bilateral dysmetria on the finger-to-nose test. No rest tremor or bradykinesia was found, though he had mildly spastic tone. Brain MRI was significant for a right occipital infarct only.

The syndrome of saccadic gaze palsy with parkinsonism is a rare and devastating complication of ascending aortic aneurysm repair.1, 2 Although some patients have been reported with infarcts in the pons, substantia nigra, centrum semiovale, frontal subcortex, striatum, corona radiata, internal capsule, and basal ganglia,3 the saccadic gaze palsy is due to brainstem injury.4 Saccadic gaze palsy is characterized by slow, hypometric saccades and absent quick phases of optokinetic nystagmus, with intact vestibular ocular reflexes. Vertical saccades may be affected in isolation, though both vertical and horizontal saccades are typically impaired. As in most cases (summarized in Table 1), our patients’ MRIs did not reveal a brainstem injury, likely because of insufficient imaging resolution.5 Importantly, one of our cases did have an occipital infarction as evidence of posterior circulation ischemia. The mechanism of injury in this syndrome remains unclear, although a perioperative ischemic stroke from embolism, hypothermia protocol, hypotension, hyperviscosity, or cardiopulmonary bypass is possible.6 A possible location of embolism may be in the posterior thalamo-subthalamic paramedian artery, a branch of the proximal posterior cerebral artery, which supplies an area of the rostral midbrain that is crucial to generation of vertical saccades.4 Because it is difficult to unify the constellation of supranuclear gaze palsy with dysarthria, dysphagia, and gait imbalance into a single infarct, multiple embolic infarcts are likely. Many cases involved surgery of the ascending aorta, suggesting multiple micro-emboli to the posterior circulation that may have been too small to generate symptoms in the anterior circulation. This syndrome is usually permanent, and symptomatic treatment is rarely successful, although treatment with levodopa, dopamine agonists, and anticholinergic agents has been attempted.

Discussion

Whether or not this devastating syndrome can be prevented is still uncertain, as is the possibility that more limited forms of the syndrome may be more common, perhaps overlooked in the immediate postoperative state. To improve accurate diagnosis, dynamic eye movements such as saccades should be assessed in any patient with visual complaints after aortic artery surgery.

References

1. Mokri B, Ahlskog E, Fulgham J, Matsumoto J. Syndrome resembling PSP after surgical repair of ascending aorta dissection or aneurysm. Neurology 2004;62:971–973, doi: http://dx.doi.org/10.1212/01.WNL.0000115170.40838.9B.

2. Kim HT, Shields S, Bhatia K, Quinn N. Progressive supranuclear palsy-like phenotype associated with bilateral hypoxic-ischemic striatopallidal lesions. Move Disord 2005;20:755–757, doi: http://dx.doi.org/10.1002/mds.20407.

3. Josephs KA, Ishizawa T. A clinicopathological study of vascular progressive supranuclear palsy. Arch Neurol 2002;99:1597–1601, doi: http://dx.doi.org/10.1001/archneur.59.10.1597.

4. Solomon D, Ramat S, Tomsak R, et al. Saccadic palsy after cardiac surgery: characteristics and pathogenesis. Ann Neurol 2008;63:355–365, doi: http://dx.doi.org/10.1002/ana.21201.

5. Antonio-Santos A, Eggenberger E. Asaccadia and ataxia after repair of ascending aortic aneurysm. Semin Ophthalm 2007;22:33–34, doi: http://dx.doi.org/10.1080/08820530701193275.

6. Yee RD, Purvin V. Acquired ocular motor apraxia after aortic surgery. Trans Am Ophthalmol Soc 2007;105:152–158.

Table 1. Cases of Progressive Supranuclear Palsy-like Syndrome After Aortic Surgery

Case Age/Gender Procedure Complications Initial Signs and Symptoms Later symptoms Time Course MRI findings

Case number with superscript = reference number (see list of references).

MRI = Magnetic Resonance Imaging, AAA = ascending aortic aneurysm, AA = aortic aneurysm, AVR = aortic valve replacement, SNGP = supranuclear gaze palsy, WNL = within normal limits, MRA = Magnetic Resonance Angiogram, CT = Computer Tomography.

1 25/M AVR and resection of infected graft Graft infection Vision difficulty Dysarthria, dysphagia, unsteady gait 2 months Small T2/FLAIR hyperintensity in splenium of corpus collosum and another in frontal lobe
2 53/M AAA repair and repair dissecting descending aortic aneurysm Descending aortic aneurysm dissection following initial repair Dysarthria, dysphagia, gait Imbalance Anarthria, further gait imbalance 6 weeks Right occipital infarct
31 56/M Resection of AA and AVR SNGP, mild gait unstability, dysarthria Marked unsteady gait, dysarthria, SNGP After 3-4 months WNL
41 45/F Resection of acute aortic dissection SNGP, transient memory deficits Marked unsteady gait, dysarthria, SNGP After 2 months WNL
51 52/M Resection of acute aortic dissection SNGP, unsteady gait Marked unsteady gait, SNGP, dysarthria, dysphagia, several partial seizures 3-4 months Subtle T2 signal abnormality mesial temporal lobes
61 44/M Resection of AA, AVR SNGP Unsteady gait, dysarthria, dysphagia, SNGP, dystonic pharyngeal movements Several weeks MRI WNL, MRA with mild anomalous irregularities of MCA
71 57/M Repair AV and ascending aorta SNGP Unsteady gait, SNGP, dysarthria 5 months Tiny lacunar infarct caudate head
81 50/M AAA repair and AVR SNGP, unsteady gait SNGP, unsteady gait, dysarthria 2 months Head CT old R cerebral infarct
91 45/F Resection of AAA, AVR Mild dysarthria and dysphagia, probable SNGP Dysarthria, dysphagia, drooling, gait unst, SNGP 2 months WNL
102 65/M AA repair Hypotension Dysarthria, dysphagia Reduced vertical gaze and gait instability 6 months Hypoxic-ischemic bilateral striopallidal lesions
114 64/M AAA repair and AVR SNGP, balance difficulty, dysarthria Not provided 2 years None performed, CT WNL
124 41/F Repair of patent ductus arteriosis SNGP, dysarthria, gait difficulty Progessive gait difficulty 5 years MRI WNL, MRA narrow P1 segment of L PCA
134 44/F Several repairs of aortic dissection and AVR SNGP, dysphagia Not provided 10 years Periventricular small vessel changes, MRA narrow P1 segment of PCA
144 46/M Resection of malignancy from right atrium 3 minutes circulatory arrest SNGP, dysphagia and drooling Not provided 4 months WNL
154 45/F AVR SNGP, emotional lability Not provided 10 months None performed, CT WNL
164 40/M Aortic dissection repair SNGP Not provided 10 months Increased signal L posterior thalamus and L medial temporal lobe
174 52/M Repair thoracoabdominal aneurysm Post-operative hypotension followed by hypertension SNGP Not provided 6 months Diffuse signal changes, no evidence of infarction
184 59/M Aortic dissection repair Difficulty weaning from cardiopulmonary bypass SNGP, transient diplopia, R lower facial weakness Not provided 2 months Nondiagnostic, diffusion negative
194 70/M Aortic aneurysm repair and AVR Post-operative septic shock SNGP, gait difficulty, dysarthria Not provided 18 months Mild diffuse atrophy
204 56/M Aortic aneurysm repair and AVR SNGP, dysarthria Not provided 4 months Mild periventricular white matter lesions
215 54/M AAA repair Hypoxia Absent volitional saccades Dysphagia, bradykinesia, and wide-based gait 12 months Chronic microvascular disease L parietal lobe
226 52/M AA resection and aortic valve repair Slurred speech, unsteady gait Unsteady gait, absence of saccades 3 months Small acute infarcts R cerebellar hemisphere and both sensory motor cortices
236 37/M Aortic root repair and AVR Blurred vision, dysphagia, imbalance Slow small amplitude saccades 2 months WNL
246 70/F AVR, aorta resection, aortic arch replacement Blurred vision, trouble tracking objects Small slow horizontal volitional saccades 8 months WNL

Video 1. The Patient at Initial Visit.

The patient is unable to initiate horizontal or vertical saccades. However, tracking his cellphone with auditory cues enabled the patient to look in all directions. Mild facial masking and dystonia are also present.

Video 2. The Patient at Initial Visit.

The patient demonstrates facial masking and a mild quizzical stare. He demonstrates a saccadic gaze palsy, with inability to look left or vertically on command. Rightward eye movements are possible, but attempts at saccades demonstrate severe slowing.