Characteristics of clinical presentation and neuropathology in idiopathic hypereosinophilic syndrome encephalopathy / 中华神经科杂志

ABSTRACT

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.