原发性嗜酸性粒细胞增多综合征脑病一例临床及病理特征

Characteristics of clinical presentation and neuropathology in idiopathic hypereosinophilic syndrome encephalopathy

目的 探讨原发性嗜酸性粒细胞增多综合征（IHES）脑病的临床和病理特征以及相关治疗方式.方法 总结1例通过临床表现、实验室检查、影像学检查、骨髓和脑活体组织检查明确诊断的IHES脑病患者临床和病理特点,给予皮质类固醇等治疗并长期随访观察预后.结果 本例IHES脑病患者以进行性肢体无力和智能减退发病,血常规检查显示嗜酸性粒细胞持续增高,各种实验室检查排除继发性嗜酸性粒细胞增多,骨髓活体组织检查排除克隆性嗜酸性粒细胞增多如嗜酸性粒细胞白血病等,头颅MRI检查示右侧额叶、双侧顶叶和枕叶多发片状长T1、长T2及FLAIR高信号影,病变部位脑活体组织检查显示小血管内膜增厚、管腔狭窄,神经元坏死或呈缺血样改变,给予长期泼尼松治疗后病情稳定.结论 嗜酸性粒细胞增多造成的脑血管病变是IHES脑病的重要病理生理基础,治疗中须使用皮质类固醇或羟基脲.

Objective To discuss the mechanism and characteristics of clinic presentation and neuropathology in idiopathic hypereosinophilic syndrome （IHES） encephalopathy. Methods IHES encephalopathy was diagnosed by clinical presentations,lab examinations,neurologic images,marrow and brain biopsy,then treated with corticosteroids and continuous follow-up. Results The IHES patient presented progressive limbs weakness and cognitive deficit with elevated eosinophil count. Results of lab examinations and bone marrow biopsy ruled out secondary eosinophilia and clonal eosinophilia such as eosinophilic leukemia.Brain magnetic resonance imaging （MRI） study showed multiple lesions in right frontal lobe, bilateral parietal and occipital lobe, presenting hypointensity in T1 weighted images,hyperintensity in T2 and fluid-attenuated inversion recovery weighed images. Brain biopsy showed proliferation of vascular membrane,small vessels stenosis,ischemia-induced morphological change and necrosis of neurons in the lesions.The patient was continuously treated with corticosteroids,and the situation was stabilized with follow-up.Conclusions IHES encephalopathy should be paid more attention in clinical practice of neurologists for its rarity. Brain vascular damages caused by elevated eosinophil may be an important pathophysiological mechanism of IHES encephalopathy,and corticosteroids or hydroxycarbamide should be used for the treatment.