摘要
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 elucidate the clinical efficacy of reversible posterior leukoencephalopathy syndrome(RPLS)in children induced by cyslosporine A,and to analyze the etiological factors,pathogenesis,MRI characteristics and treatment. Methods The clinical data of 1case with RPLS were retrospectively analyzed and related reports were reviewed. Results RPLS was a group of signs and symptoms mainly associated with posterior cerebral white matter lesions.It was an uncommon but a distinctive clinicoradiologic entity characterized by headache,seizures,visual disturbance and altered mental function.MRI showed the lesions were symmetrical and mainly involved the subcortical regions and cortical portion of parietal-occipital lobes,presenting as low signal on T1W1 and high signal on T2W1 and FLAIR sequence.Clinical conditions were improved and MRI abnormalities disappeared after effective therapy.Most of RPLS in children suffered from underlying diseases,including renal insufficiency of various aetiologies,autoimmune disorders and haematooncologic diseases,and were treated with glucocorticoid,immunosuppressant or cytotoxic drug.The mechanisms of drug-induced RPLS remain unclear.Conclusion RPLS is reversible in the majority of case with timely and appropriate management.Treatment strategies for drug-induced RPLS include the withdrawal of suspected drugs,control of blood pressure and seizures.Early diagnosis and treatment should be prompt to prevent irreversible sequels.
出处
《总装备部医学学报》
2014年第3期138-141,188,共5页
Medical Journal of General Equipment Headquarters
关键词
可逆性后部白质脑病综合征
环孢霉素A
儿童
Reversible posterior leukoencephalopathy syndrome
Cyclosporine A
Children
