Romano Sonia, Avola Giulia, Angeli Marco Cesare, Brazzale Francesca, Giacopazzi Elena, Castellini Paola, Genovese Antonio
Department of Medicine and Surgery, University of Parma, Parma, Italy.
Stroke Care, Neurology Operating Unit, Emergency-Urgency Department, University-Hospital of Parma, Parma, Italy.
Pulse (Basel). 2024 Aug 20;12(1):106-112. doi: 10.1159/000539379. eCollection 2024 Jan-Dec.
Hypereosinophilic syndrome is characterized by a peripheral blood eosinophil count >1.5 × 10/μL on two different examinations within a month of each other and/or a 20% or higher percentage of eosinophils in a bone marrow section, associated with organ damage. Rarely, neurological manifestations may occur, even in the early stages. We report a case of idiopathic hypereosinophilic syndrome with Loeffler endocarditis presenting with multiple bilateral strokes and encephalopathy as the first clinical manifestations.
Hypereosinophilia and echocardiographic findings suggested a Loeffler's endocarditis. Blood hyperviscosity and small vessels inflammation induced by the hypereosinophilia itself, the embolization of intracardiac thrombus, along with the impaired clearance of microthrombi in the watershed areas, are the main mechanisms involved in the pathophysiology of stroke in the hypereosinophilic syndrome. Additionally, encephalopathy could be considered as a consequence of multiple cerebral infarcts and neurotoxicity induced by hypereosinophilia since our patient's confusion and aggressive behavior gradually remitted after steroid therapy was started.
To the best of our knowledge, our case report is a rare instance highlighting neurological involvement as the earliest manifestation of hypereosinophilia. We aimed to elucidate the central nervous system involvement in this intriguing disorder, with the goal of encouraging clinicians to consider hypereosinophilic syndrome in the diagnostic assessment of rare stroke etiologies.
嗜酸性粒细胞增多综合征的特征是在彼此相隔一个月的两次不同检查中,外周血嗜酸性粒细胞计数>1.5×10⁹/μL,和/或骨髓切片中嗜酸性粒细胞百分比达到20%或更高,并伴有器官损害。即使在早期阶段,也很少会出现神经系统表现。我们报告一例特发性嗜酸性粒细胞增多综合征合并吕弗勒心内膜炎,以多发性双侧中风和脑病作为首发临床表现。
嗜酸性粒细胞增多和超声心动图检查结果提示吕弗勒心内膜炎。嗜酸性粒细胞增多本身引起的血液高黏滞度和小血管炎症、心内血栓的栓塞,以及分水岭区微血栓清除障碍,是嗜酸性粒细胞增多综合征中中风病理生理学的主要机制。此外,脑病可被认为是多发性脑梗死和嗜酸性粒细胞增多引起的神经毒性的结果,因为在开始使用类固醇治疗后,我们患者的意识模糊和攻击性行为逐渐缓解。
据我们所知,我们的病例报告是一个罕见的例子,突出了神经系统受累作为嗜酸性粒细胞增多的最早表现。我们旨在阐明这种有趣疾病中中枢神经系统的受累情况,目的是鼓励临床医生在罕见中风病因的诊断评估中考虑嗜酸性粒细胞增多综合征。