Lee Dokyung, Ahn Tae-Beom
Department of Neurology, College of Medicine, Kyung Hee University, Seoul, Republic of Korea.
Department of Neurology, College of Medicine, Kyung Hee University, Seoul, Republic of Korea.
J Neurol Sci. 2014 Dec 15;347(1-2):281-7. doi: 10.1016/j.jns.2014.10.023. Epub 2014 Oct 29.
The central nervous system (CNS) may be affected in those cases of peripheral eosinophila without a secondary cause (hypereosinophilic syndrome, HES). The pathomechanism of CNS involvement in HES remained uncertain.
We included those with CNS symptoms and peripheral eosinophilia (>1500/μl) at the time of presentation. Those with identifiable causes of eosinophilia were excluded. In addition to data analysis of our cases, we reviewed the literature using the Medline database.
Ten patients were recruited. Half of them presented with altered mental status. None of the patients had significant risk factors such as endomyocardial disease (EMD). Their brain magnetic resonance imaging (MRI) showed multiple ischemic lesions in the border zone (BZ) and other cortical areas. In addition to our 10 cases we included 67 cases from a literature review. The total 77 cases were systemically analyzed. We found that the most common type of CNS involvement was cerebrovascular disease (CVD, 63.6%), multifocal lesions in BZ were the most common feature on MRI (45.5%), many patients presented with altered mental status (40.3%), and direct eosinophil infiltration into brain parenchyma was rare in pathologic studies and not found in the patients with CVD. Furthermore, although EMD was one of the major cardiac abnormalities (26.0%), cardiac workup more frequently showed non-EMD or normal findings. Cardiac abnormalities, altered mental status, and higher eosinophil counts were more common in those with poor outcomes, whereas an isolated CNS manifestation was more frequent in those with favorable outcomes. Finally, the presence of altered mental status was the most significant predictor of poor clinical outcomes, whereas isolated CNS involvement and corticosteroid treatment were significant predictors for favorable outcomes.
Multiple lesions on MRI were suggestive of an embolic mechanism, but diagnostic workup failed to find significant risk factors such as EMD in the most cases. Impaired anticoagulation by eosinophilic cationic proteins could be an alternative mechanism. Direct invasion of eosinophils into the CNS was rare and those cases with isolated CNS manifestations showed a better prognosis. The preferential indirect involvement of CNS by eosinophils ("CNS-tropism") may underlie the neurological manifestations of HES. Early administration of corticosteroid after ruling out secondary causes can be beneficial in HES with CNS manifestation.
在那些无继发原因的外周嗜酸性粒细胞增多症（高嗜酸性粒细胞综合征，HES）病例中，中枢神经系统（CNS）可能会受到影响。HES中CNS受累的发病机制仍不确定。
我们纳入了就诊时出现CNS症状且外周嗜酸性粒细胞增多（>1500/μl）的患者。排除有可识别的嗜酸性粒细胞增多原因的患者。除了对我们的病例进行数据分析外，我们还使用Medline数据库回顾了文献。
招募了10名患者。其中一半出现精神状态改变。没有患者有诸如心内膜疾病（EMD）等显著危险因素。他们的脑磁共振成像（MRI）显示在边界区（BZ）和其他皮质区域有多个缺血性病变。除了我们的10例病例外，我们还从文献回顾中纳入了67例病例。对总共77例病例进行了系统分析。我们发现，CNS受累最常见的类型是脑血管疾病（CVD，63.6%），BZ的多灶性病变是MRI上最常见的特征（45.5%），许多患者出现精神状态改变（40.3%），在病理研究中嗜酸性粒细胞直接浸润脑实质很少见，在CVD患者中未发现。此外，虽然EMD是主要的心脏异常之一（26.0%），但心脏检查更常显示非EMD或正常结果。心脏异常、精神状态改变和较高的嗜酸性粒细胞计数在预后不良的患者中更常见，而孤立的CNS表现在预后良好的患者中更常见。最后，精神状态改变的存在是临床预后不良的最显著预测因素，而孤立的CNS受累和皮质类固醇治疗是预后良好的显著预测因素。
MRI上的多个病变提示栓塞机制，但在大多数情况下诊断检查未能发现诸如EMD等显著危险因素。嗜酸性阳离子蛋白对抗凝的损害可能是一种替代机制。嗜酸性粒细胞直接侵入CNS很少见，那些有孤立CNS表现的病例预后较好。嗜酸性粒细胞对CNS的优先间接累及（“CNS嗜性”）可能是HES神经表现的基础。在排除继发原因后早期给予皮质类固醇对有CNS表现的HES可能有益。
