Popkirov Stoyan, Schlegel Uwe, Skodda Sabine
Department of Neurology, University Hospital Knappschaftskrankenhaus Bochum, Ruhr-University Bochum, Germany.
Department of Neurology, University Hospital Knappschaftskrankenhaus Bochum, Ruhr-University Bochum, Germany.
Med Hypotheses. 2016 Apr;89:21-3. doi: 10.1016/j.mehy.2016.02.001. Epub 2016 Feb 6.
Postoperative encephalopathy with choreoathetosis ("postpump chorea") is a rare complication of open-heart surgery and, in particular, the employment of a cardiopulmonary bypass pump. It almost exclusively occurs in young children. While risk factors and the underlying histopathology have been identified, the pathogenesis of postpump chorea, crucially, remains largely unknown. Transient cerebral hypoperfusion associated with cardiopulmonary bypass is considered a likely candidate mechanism, but the evidence is insufficient and inconclusive. It is hypothesized in this article, that postpump chorea may be caused by mechanical trauma to red blood cells and resulting acanthocytosis. These dysfunctional erythrocytes could then lead to damage to the globus pallidus and disease development akin to that presumed in neuroacanthocytosis. In patients with neuroacanthocytosis an association between acanthocytosis and basal ganglia pathology has been suggested. To test the mechanism hypothesized here, the effects of cardiopulmonary bypass on erythrocyte morphology and function could be systematically tested in children undergoing cardiac surgery. Ideally, the extent of erythrocyte damage could be correlated with the risk of developing postpump chorea. Finally, if the proposed hypothesis is supported by empirical findings, efforts to reduce blood cell damage during extracorporeal circulation in children might prevent this devastating complication.
术后伴有舞蹈手足徐动症的脑病（“泵后舞蹈症”）是心脏直视手术尤其是使用体外循环泵后的一种罕见并发症。它几乎只发生在幼儿身上。虽然已经确定了风险因素和潜在的组织病理学，但至关重要的是，泵后舞蹈症的发病机制在很大程度上仍然未知。与体外循环相关的短暂性脑灌注不足被认为是一种可能的机制，但证据不足且尚无定论。本文提出假说，泵后舞蹈症可能是由红细胞的机械性损伤及由此导致的棘红细胞增多症引起的。这些功能失调的红细胞随后可能导致苍白球损伤，并引发类似于神经棘红细胞增多症中推测的疾病发展过程。在神经棘红细胞增多症患者中，已经有人提出棘红细胞增多症与基底神经节病变之间存在关联。为了验证此处提出的机制，可以对接受心脏手术的儿童进行系统测试，以观察体外循环对红细胞形态和功能产生的影响。理想情况下，红细胞损伤的程度可以与发生泵后舞蹈症的风险相关联。最后，如果所提出的假说得到实证研究结果的支持，那么在儿童体外循环期间减少血细胞损伤的努力可能会预防这种毁灭性的并发症。
