Kwon S U, Kim J C, Kim J S
Department of Neurology, Asan Medical Centre, University of Ulsan, 388-1 Pungnap-dong, Songpa-gu, Seoul.
J Neurol. 2001 Apr;248(4):279-84. doi: 10.1007/s004150170201.
Hypereosiophilia-induced encephalopathy (HE) is a rare but well-described clinical syndrome. However, serial magnetic resonance imaging (MRI) findings of HE have rarely been reported. We describe serial MRI findings of three patients with HE. The patients presented with acute confusion, focal neurological deficits and/or seizures. Eosinophils in repeated blood tests were more than 3000/mm3 in all the patients. Echocardiography in two patients showed findings consistent with eosinophilic endomyocardial fibrosis or global hypokinesia. The initial MRI revealed multiple high-signal lesions on T2-weighted images with gadolinium-DTPA enhancement on T1-weighted images, which were predominantly distributed in the border zone of the middle-anterior cerebral arteries and the middle-posterior cerebral arteries. The second MRIs taken prior to the initiation of steroid therapy showed that the lesions increased in size and number in the same area. The third MRIs performed long after the therapy showed that the lesions were shrunken. A brain biopsy specimen in one patient showed reactive gliosis following infarction with abundant intravascular eosinophils. The MRI-identified lesions in the patients with HE thus develop mainly in the border zone. The lesions occasionally increase in size and number and shrink if the eosinophilia is adequately treated. Although the nature of the MRI-identified lesions remains unclear, their pathogenesis may be related to multiple embolisms associated with concomitant cardiac abnormality and hypercoagulable state.
嗜酸性粒细胞增多性脑病(HE)是一种罕见但已被充分描述的临床综合征。然而,HE的系列磁共振成像(MRI)表现鲜有报道。我们描述了3例HE患者的系列MRI表现。这些患者表现为急性意识模糊、局灶性神经功能缺损和/或癫痫发作。所有患者多次血液检查中的嗜酸性粒细胞均超过3000/mm³。2例患者的超声心动图显示与嗜酸性粒细胞性心内膜纤维化或整体运动减弱一致的表现。初始MRI在T2加权图像上显示多个高信号病变,在T1加权图像上有钆-二乙三胺五乙酸(Gd-DTPA)强化,主要分布在大脑中动脉与大脑前动脉以及大脑中动脉与大脑后动脉的边界区。在开始使用类固醇治疗前进行的第二次MRI显示,同一区域的病变在大小和数量上均增加。治疗很久后进行的第三次MRI显示病变缩小。1例患者的脑活检标本显示梗死灶后反应性胶质增生,血管内有大量嗜酸性粒细胞。因此,HE患者MRI识别出的病变主要发生在边界区。如果嗜酸性粒细胞增多症得到充分治疗,病变偶尔会在大小和数量上增加,然后缩小。虽然MRI识别出的病变性质仍不清楚,但其发病机制可能与伴有心脏异常和高凝状态的多发性栓塞有关。