Post‐Pump Chorea and Progressive Supranuclear Palsy‐Like Syndrome Following Major Cardiac Surgery

Abstract

Background Background: Post-pump chorea and progressive-supranuclear palsy (PSP)-like syndrome after aortic surgery are 2 distinct movement disorders following major cardiac surgeries. Cases Cases: We herein report 3 patients with movement disorders that developed after major cardiac surgeries. Two patients developed post-pump chorea after pulmonary endarterectomy, and 1 further case developed PSP-like syndrome after aortic replacement surgery. The 2 conditions share several common aspects. Both are preceded by surgeries that undergo cardiopulmonary bypass and deep hypothermia circulatory arrest procedures. Most cases present with biphasic course. However, post-pump chorea occurs in all age populations after any surgeries that undergo deep hypothermia circulatory arrest, whereas PSP-like syndrome is reported exclusively in the adult population after aortic surgery. Conclusions Conclusions: Post-pump chorea and PSP-like syndrome are neurologic complications of major cardiac surgeries that should not be underrecognized. Further reports to establish their common pathogenic mechanism should be encouraged. View Supplementary VideoChoreiform movement disorder occurs in a substantial number of children undergoing major cardiac surgery utilizing cardiopulmonary bypass and deep hypothermia circulatory arrest (DHCA), namely, the post-pump chorea. 1,2 However, several cases were also reported in the adult population, suggesting that it is not necessarily a pediatric condition. 3,4 In addition to this peculiar complication of cardiac surgeries, there have been reports of a distinct movement disorder mimicking features of progressive supranuclear palsy (PSP) in adults following complex aortic surgeries. 5-7 We herein report 3 patients who developed movement disorders after major cardiac surgeries with literature reviews. Case SeriesCase 1 A 25-year-old male admitted in cardiac surgery department was consulted to neurology department for acute onset of involuntary movements. The patient had undergone bilateral pulmonary endarterectomy (PEA) for chronic thromboembolic pulmonary hypertension under 64 minutes of circulatory arrest time 7 days prior (Fig. 1A). There were no neurological symptoms or signs prior to the surgery. He did not have a family history of

Cardiac Involvement in Movement Disorders

“…Post-pump chorea has been classically recognized in 1% to 18% of children as a complication of cardiac surgery that undergoes cardiopulmonary bypass and deep hypothermia circulatory arrest, such as congenital heart defect corrective surgery, aortic surgery, or pulmonary endarterectomy, but can also occur in adults. [156][157][158][159][160] It consists of generalized chorea, choreodystonia including orolingual and facial musculatures or choreoballism that usually develops after a latent period of normal motor function of few hours or days postoperative with a peak of severity at 2 weeks and subsequent ameloriation of choreic movements that can self-limit of even persist for several months or permanently. 156,158 The exact etiology and pathophysiology of this complication is unknown, but probably centers around several factors that may lead to partial basal ganglia ischemia.…”

“…[156][157][158][159][160] It consists of generalized chorea, choreodystonia including orolingual and facial musculatures or choreoballism that usually develops after a latent period of normal motor function of few hours or days postoperative with a peak of severity at 2 weeks and subsequent ameloriation of choreic movements that can self-limit of even persist for several months or permanently. 156,158 The exact etiology and pathophysiology of this complication is unknown, but probably centers around several factors that may lead to partial basal ganglia ischemia. 161 Bilateral putaminal hypometabolism in [ 18 F] fluorodeoxyglucose-positron emission tomography and transient bilateral hyperintensities of globus pallidus on diffusion-weighted magnetic resonance imaging (MRI) were found.…”

“…161 Bilateral putaminal hypometabolism in [ 18 F] fluorodeoxyglucose-positron emission tomography and transient bilateral hyperintensities of globus pallidus on diffusion-weighted magnetic resonance imaging (MRI) were found. 158,160,162 Several factors may contribute with both the development and the severity of post-pump chorea, which include prolonged time on pump, duration of circulatory arrest time, lower temperature of deep hypothermia, quicker postsurgical rewarm, variability in blood pH and PaCO 2 resulting in fluctuations in cerebral blood flow, and age beyond early infancy. 157,159 Treatment with tetrabenazine, haloperidol, carbamazepine, pimozide, and sodium valproate were of very limited benefit in providing symptomatic relief and of no benefit in modifying evolution of the disease process or long-term outcomes.…”

“…157,159 Treatment with tetrabenazine, haloperidol, carbamazepine, pimozide, and sodium valproate were of very limited benefit in providing symptomatic relief and of no benefit in modifying evolution of the disease process or long-term outcomes. 156,158,160,162 Post-pump progressive supranuclear palsy-like syndrome has been reported less frequently than post-pump chorea and has been described exclusively in adults after aortic surgery. 158,[163][164][165][166] Symptom onset occurs usually during the first postoperative month.…”

“…156,158,160,162 Post-pump progressive supranuclear palsy-like syndrome has been reported less frequently than post-pump chorea and has been described exclusively in adults after aortic surgery. 158,[163][164][165][166] Symptom onset occurs usually during the first postoperative month. 158,163,164 Clinical manifestations usually include a predominant axial parkinsonism, vertical and/or horizontal gaze palsy, dysarthria, dysphagia, and postural instability, and in some cases seizures or blepharospasm were observed.…”