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目的探讨环孢霉素A致儿童可逆性后部白质脑病综合征(RPLS)的病因、发病机制、MRI影像学表现及治疗。方法回顾性分析环孢霉素A致儿童RPLS 1例的临床资料并复习文献。结果儿童RPLS临床少见,系有明显神经影像学特征,伴随头痛、抽搐、视力障碍及精神行为异常等临床表现的一种主要为大脑后部白质病变相关的综合征。MRI表现以顶枕叶皮层下白质为主的片状对称性长T1长T2信号、T2FLAIR高信号灶,有效治疗后临床症状及脑内病灶可消失。儿童RPLS通常发生在各种原因引起的肾功能不全、自身免疫性疾病或血液系统肿瘤等,并接受糖皮质激素、免疫抑制剂或细胞毒药物等治疗的患儿。药物诱发RPLS机制仍不十分清楚。结论大多数药物诱发的儿童RPLS,经过停用可疑药物、控制高血压和抽搐等治疗,预后较好。早期诊断和治疗是避免不可逆神经损害的关键。
Objective To investigate the etiology, pathogenesis, MRI imaging and treatment of cyclophosphamide-induced reversible posterior leukoencephalopathy syndrome (RPLS) in children. Methods The clinical data of 1 case of cyclosporin A-induced RPLS were retrospectively reviewed and reviewed. Results RPLS was a rare clinical syndrome in children. It had obvious neuroimaging features and associated with clinical manifestations such as headache, convulsion, visual impairment and mental and behavioral abnormalities. MRI manifestations of the top of the occipital white matter-based sheet symmetry long T1 long T2 signal, T2FLAIR high signal stove, the effective treatment of clinical symptoms and brain lesions can disappear. Children RPLS usually occurs in a variety of causes of renal insufficiency, autoimmune diseases or hematological malignancies, and received glucocorticoids, immunosuppressive agents or cytotoxic drugs in children. Drug-induced RPLS mechanism is still not very clear. Conclusion Most children with RPLS have good prognosis after discontinuing suspicious drugs, controlling hypertension and twitching. Early diagnosis and treatment is the key to avoid irreversible nerve damage.