Cioclu Maria Cristina, Cavallieri Francesco, Napoli Manuela, Moratti Claudio, Pascarella Rosario, Valzania Franco, Zedde Marialuisa
Neurology Unit, OCB Hospital, Azienda Ospedaliera-Universitaria, 42123 Modena, Italy.
Neurology Unit, Neuromotor and Rehabilitation Department, Azienda USL-IRCCS di Reggio Emilia, 42124 Reggio Emilia, Italy.
J Clin Med. 2022 Sep 23;11(19):5595. doi: 10.3390/jcm11195595.
Hypereosinophilic syndromes (HES) are a group of relatively rare disorders in which neurological manifestations, including ischemic stroke, are common. The hypothesized pathophysiological mechanisms are hypercoagulability, cardioembolism (mainly mediated by myocardial involvement) and damage to the endothelium. A variable ischemic pattern has been described, including an association of territorial and border zone ischemic stroke.
Three patients who presented to our department with acute stroke were selected aiming to show these three different mechanisms inferred from the stroke pattern on brain Magnetic Resonance Imaging (MRI) and to simultaneously illustrate the three main causes of HES.
The first patient is a 55-year-old man with an abrupt onset of aphasia due to an acute ischemic stroke involving the left parietal lobule and the angular gyrus; recent lab test had shown hypereosinophilia. An extensive workup excluded primary and secondary causes of hypereosinophilia so a diagnosis of idiopathic hypereosinophilia was done and he was treated with high doses of steroids. The second patient had severe hypereosinophilia and developed multiple small, scattered ischemic lesions, mainly in border zone zones. The history of severe asthma and recurrent sinusitis supported the diagnosis of EGPA (Eosinophilic Granulomatosis with Polyangiitis); considering the severe clinical conditions and the presumptive role of hypereosinophilia in determining her symptoms, steroid treatment was promptly started, with good clinical response. The third patient also presented with multiple metachronous ischemic lesions, both in cortical and border zone distribution and marked eosinophilia; the diagnostic work-up found an ovarian cancer. She was treated with steroids and then underwent surgery and adjuvant chemotherapy.
HES should be considered in stroke etiological evaluation, although it is a rare disorder, and border zones pattern without large artery steno-occlusion on neuroimaging may help to raise the suspicion in the neurovascular diagnostic pathway. A thorough research of the sources of hypereosinophilia should be performed to select the appropriate therapy.
嗜酸性粒细胞增多综合征(HES)是一组相对罕见的疾病,其中包括缺血性中风在内的神经学表现较为常见。推测的病理生理机制为高凝状态、心源性栓塞(主要由心肌受累介导)和内皮损伤。已描述了多种不同的缺血模式,包括区域性和边缘带缺血性中风的关联。
选择了三名因急性中风前来我院就诊的患者,旨在展示从脑部磁共振成像(MRI)的中风模式推断出的这三种不同机制,并同时说明HES的三种主要病因。
第一名患者是一名55岁男性,因急性缺血性中风累及左顶叶小叶和角回而突然出现失语;近期实验室检查显示嗜酸性粒细胞增多。广泛的检查排除了嗜酸性粒细胞增多的原发性和继发性原因,因此诊断为特发性嗜酸性粒细胞增多症,并对其进行了高剂量类固醇治疗。第二名患者有严重的嗜酸性粒细胞增多,并出现多个小的、散在的缺血性病变,主要位于边缘带区域。严重哮喘和复发性鼻窦炎病史支持嗜酸性肉芽肿性多血管炎(EGPA)的诊断;考虑到严重的临床状况以及嗜酸性粒细胞增多在确定其症状中的推测作用,立即开始了类固醇治疗,临床反应良好。第三名患者也出现了多个不同时间的缺血性病变,分布于皮质和边缘带区域,且嗜酸性粒细胞显著增多;诊断检查发现了卵巢癌。她接受了类固醇治疗,然后接受了手术和辅助化疗。
尽管HES是一种罕见疾病,但在中风病因评估中应考虑到它,神经影像学上无大动脉狭窄闭塞的边缘带模式可能有助于在神经血管诊断过程中提高怀疑度。应彻底研究嗜酸性粒细胞增多的来源,以选择合适的治疗方法。