Ciprietti C, Russo M, Santilli M, Melchiorre S, Polito G, Thomas A, Sensi S L
Department of Neuroscience, Imaging, and Clinical Science, "G. D'Annunzio" University of Chieti-Pescara, Chieti, Italy.
Institute of Neurology, "SS Annunziata" Hospital -University of Chieti-Pescara, Chieti, Italy.
Neurol Sci. 2025 Feb;46(2):993-998. doi: 10.1007/s10072-024-07948-0. Epub 2024 Dec 21.
In children and adults with sickle-cell disease (SCD), acute ischemic stroke (AIS) associated with a vaso-occlusive crisis is a leading cause of physical and cognitive disability and death. However, neurological guidelines for acute management of AIS fail to directly address this issue. We here report a case of a man with severe cerebrovascular complications and illustrate the current evidence on the management of SCD-related AIS.
A 46-year-old man suffering from SCD and poorly controlled diabetes was admitted to the emergency room complaining of the onset, more than 10 h before, of headache, paresthesia, and right hemianopia. The brain Angio-CT study revealed bilateral occlusion of the internal carotids and the posterior cerebral arteries, with remarkable compensatory hypertrophy of other vessels. The laboratory exams confirmed severe anemia with high hemoglobin-S levels (50%) and severe dehydration. IV hydration was prompted, along with erythrocyte apheresis and antiplatelet therapy. The patient successfully underwent these treatments, and the neurological deficits significantly improved. Nonetheless, due to the time window, he could not be treated with intravenous thrombolysis (IVT).
AIS is a time-sensitive condition. In SCD, vaso-occlusive phenomena are the leading cause of AIS, but "classical" vascular risk factors can also play a role. Since current guidelines on the acute management of SCD complications are mainly focused on the hematological- rather than neurological- aspects, it is not clear whether these patients should undergo IVT before or after fluid resuscitation and erythrocyte apheresis. Furthermore, the principles of secondary prophylaxis are still controversial and require further investigation.
在患有镰状细胞病（SCD）的儿童和成人中，与血管闭塞性危机相关的急性缺血性卒中（AIS）是身体和认知残疾以及死亡的主要原因。然而，AIS急性管理的神经学指南未能直接解决这个问题。我们在此报告一例患有严重脑血管并发症的男性病例，并阐述目前关于SCD相关AIS管理的证据。
一名46岁患有SCD且糖尿病控制不佳的男性因10多个小时前开始出现头痛、感觉异常和右半侧偏盲而被送往急诊室。脑部血管CT研究显示双侧颈内动脉和大脑后动脉闭塞，其他血管有明显的代偿性肥大。实验室检查证实严重贫血，血红蛋白S水平高（50%）且严重脱水。随即进行静脉补液，同时进行红细胞单采和抗血小板治疗。患者成功接受了这些治疗，神经功能缺损明显改善。尽管如此，由于时间窗的原因，他无法接受静脉溶栓治疗（IVT）。
AIS是一种对时间敏感的疾病。在SCD中，血管闭塞现象是AIS的主要原因，但“经典”血管危险因素也可能起作用。由于目前关于SCD并发症急性管理的指南主要侧重于血液学而非神经学方面，尚不清楚这些患者应在液体复苏和红细胞单采之前还是之后接受IVT。此外，二级预防的原则仍存在争议，需要进一步研究。
