Tisel Sarah M, Ahlskog J Eric, Duffy Joseph R, Matsumoto Joseph Y, Josephs Keith A
Department of Neurology (SMT, JEA, JRD, KAJ), Mayo Clinic, Rochester, MN; and Department of Neurology (JYM), University of Minnesota, Minneapolis, MN.
Neurol Clin Pract. 2020 Jun;10(3):245-254. doi: 10.1212/CPJ.0000000000000708.
A rare progressive supranuclear palsy-like syndrome seemingly triggered by aortic surgery was first described in 2004. This largest case series to date describes the features of this syndrome.
We searched the Mayo Clinic electronic medical records using the advanced cohort explorer search engine for patients evaluated for neurologic symptoms after cardiac-aortic surgery in the past 30 years. Data were extracted to Microsoft Excel from the identified patients and included clinical and neuroimaging features and outcomes.
Twenty-five patients met the inclusion criteria. All surgeries were performed under thoracic aortic bypass and deep hypothermia. Surgery included aortic aneurysm, aortic valve repair, and/or aortic dissection repair. Surgical records were unavailable, although surgery was documented in the Mayo record as uncomplicated in 60% of cases. In the remaining cases, no particular intraoperative or postoperative complications were documented at a high frequency. A typical triad was documented: supranuclear gaze palsy (SNGP; 100%), gait imbalance (80%), and dysarthria (96%). Part or all of the triad was observed before hospital discharge and stabilized over the course of days-weeks. A second phase of symptom worsening plus new symptoms developed up to a year later; this decline continued for up to several years before stabilization. Delayed epileptic seizures occurred in 32% of patients. Brain MRI revealed only nonspecific findings.
This syndrome following adult thoracic aortic bypass surgery with deep hypothermia remains unexplained. It follows a biphasic course and is characterized by the triad of SNGP, unsteady gait, and a predominantly ataxic dysarthria.
2004年首次描述了一种看似由主动脉手术引发的罕见的进行性核上性麻痹样综合征。本迄今为止最大的病例系列描述了该综合征的特征。
我们使用高级队列浏览器搜索引擎在梅奥诊所电子病历中搜索过去30年接受心脏主动脉手术后出现神经症状评估的患者。从确定的患者中提取数据至Microsoft Excel，包括临床和神经影像学特征及结果。
25例患者符合纳入标准。所有手术均在胸主动脉旁路和深度低温下进行。手术包括主动脉瘤、主动脉瓣修复和/或主动脉夹层修复。虽然梅奥记录中60%的病例记录手术无并发症，但手术记录不可用。在其余病例中，未高频记录到特定的术中或术后并发症。记录到一个典型的三联征：核上性凝视麻痹（SNGP；100%）、步态失衡（80%）和构音障碍（96%）。三联征的部分或全部在出院前出现，并在数天至数周内稳定。症状加重及新症状的第二阶段在长达一年后出现；这种衰退持续长达数年才稳定。32%的患者发生迟发性癫痫发作。脑部MRI仅显示非特异性表现。
这种在成人胸主动脉旁路手术伴深度低温后出现的综合征仍无法解释。它呈双相病程，其特征为SNGP、步态不稳和以共济失调为主的构音障碍三联征。
