Objective: We report three patients who developed movement disorders after major cardiac surgery and suggest a common underlying mechanism.

Background: Choreiform movement disorder occurs in a number of children undergoing cardiopulmonary surgery utilizing cardiopulmonary bypass (CPB) and deep hypothermia circulatory arrest (DHCA), namely the post-pump chorea. However, several adult cases were also reported. In addition to this peculiar condition, there have been reports of a distinct condition mimicking features of progressive supranuclear palsy (PSP) following complex aortic surgeries.

Method: Case series.

Results: Patient 1, a 25-year-old male, presented with acute onset of generalized choreoathetosis that developed 7 days after bilateral pulmonary endarterectomy (PEA) for acute pulmonary embolism. Magnetic resonance imaging (MRI) revealed bilateral T2 high signal intensities of the globus pallidus with diffusion restriction, which normalized after 2 weeks. Symptoms peaked at 2 postoperative weeks then slowly lessened. Patient 2 was a 38-year-old male who presented with generalized choreoathetosis that developed 10 days after bilateral PEA for chronic thromboembolic pulmonary hypertension. The chorea worsened for the following 2 weeks and then stabilized. MRI taken 3 weeks after the onset showed no demonstrable abnormalities.Patient 3, a 48-year-old male, presented with gait disturbance and an inability to look downward that developed 1 month after aortic replacement surgery for acute thoracic aortic dissection. Absent vertical saccades, dysarthria, axial rigidity and gait freezing were observed. MRI taken at 4 months and at 48 months postoperatively revealed interval midbrain atrophy.

Conclusion: We highlight that the post-pump chorea and PSP-like syndrome after major cardiac surgery, commonly manifest with a brief latent period before the onset. Therefore, we assume that specific brain regions are vulnerable to the hypoxic consequences of cardiopulmonary manipulation procedures. In recent autopsy report on post-pump chorea showed profound neuronal loss in the globus pallidus, and another autopsy report of PSP-like syndrome demonstrated fragmentation and severe loss of perineuronal nets surrounding the saccadic burst neurons. Whether our assumption of specific vulnerability could bridge the gap between the apoptosis model and perineural net model requires further investigations.

References: 1. Przekop A, McClure C, Ashwal S. Postoperative encephalopathy with choreoathetosis. Handbook of clinical neurology 2011;100:295-305. 2. Mokri B, Ahlskog JE, Fulgham JR, Matsumoto JY. Syndrome resembling PSP after surgical repair of ascending aorta dissection or aneurysm. Neurology 2004;62:971-973. 3. Ditsworth D, Priestley MA, Loepke AW, et al. Apoptotic neuronal death following deep hypothermic circulatory arrest in piglets. Anesthesiology 2003;98:1119-1127. 4. Eggers SD, Horn AK, Roeber S, et al. Saccadic Palsy following Cardiac Surgery: Possible Role of Perineuronal Nets. PLoS One 2015;10:e0132075.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/post-pump-chorea-and-progressive-supranuclear-palsy-like-syndrome-following-major-cardiac-surgery-a-case-series/