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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
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
BackgroundBackground: Several conditions represented mainly by movement disorders are associated with cardiac disease, which can be overlooked in clinical practice in the context of a prominent primary neurological disorder. Objectives Objectives: To review neurological conditions that combine movement disorders and primary cardiac involvement. Methods Methods: A comprehensive and structured literature search following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses criteria was conducted to identify disorders combining movement disorders and cardiac disease. ResultsResults: Some movement disorders are commonly or prominently associated with cardiac disease. Neurological and cardiac symptoms may share underlying physiopathological mechanisms in diseases, such as Friedreichʼs ataxia and Wilsonʼs disease, and in certain metabolic disorders, including Refsum disease, Gaucher disease, a congenital disorder of glycosylation, or cerebrotendinous xanthomatosis. In certain conditions, such as Sydenhamʼs chorea or dilated cardiomyopathy with ataxia syndrome (ATX-DNAJC19), heart involvement can present early in the course of disease, whereas in others such as Friedreichʼs ataxia or Refsum disease, cardiac symptoms tend to present in later stages. In another 68 acquired or inherited conditions, cardiac involvement or movement disorders are seldom reported. Conclusions Conclusions: As cardiac disease is part of the phenotypic spectrum of several movement disorders, heart involvement should be carefully investigated and increased awareness of this association encouraged as it may represent a leading cause of morbidity and mortality.Cardiac abnormalities may be a significant source of morbidity and premature mortality [1][2][3][4] and can occur in numerous neurological diseases, manifesting as cardiomyopathy, arrhythmias, conduction defects, structural malformations, coronary disease, valvulopathies, or cardiac autonomic dysfunction. In neurological conditions mainly manifested by movement disorders, cardiac disease can be a frequent and predominant clinical feature, or only be reported in isolated cases, and can be of early presentation, or occur only during later stages of disease. 5,6 In diseases such as Friedreichʼs ataxia (FRDA), where the need for heart monitoring is well established, a cross-sectional healthcare resource study revealed that recommended annual cardiac evaluations had not been conducted in up to 23% of patients with FRDA in the United States and 14% in Canada. 7 These figures may be even higher in other parts of the world.In this educational review, we describe and list movement disorders with cardiac involvement as part of the clinical picture. MethodsA comprehensive and structured search in PubMed following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines (http://www.prismastatement.org) was conducted.
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