儿童可逆性后部脑白质病综合征10例临床分析

发布时间：2018-05-12 10:53

本文选题：可逆性后部脑白质病综合征 + 儿童　；参考：《中国实用儿科杂志》2017年07期

【摘要】：目的探讨儿童可逆性后部脑白质病综合征(RPLS)临床及影像学特点,提高儿科临床医师对RPLS的认识。方法回顾性分析深圳市儿童医院肾脏免疫科2009—2015年10例RPLS患儿临床表现、影像学资料、治疗及预后。结果 10例均急性起病,其中继发于过敏性紫癜4例、肾病综合征3例、系统性红斑狼疮2例、IgA肾病1例。10例均应用免疫抑制剂。10例RPLS均有癫痫发作,8例合并高血压,4例出现头晕头痛,3例出现视觉异常,3例出现意识障碍,2例出现恶心、呕吐。10例患儿均完善头颅磁共振成像(MRI)检查,影像学表现主要为大脑后部为主的长T1、长T2信号,大部分呈对称性。10例RPLS经控制惊厥、降颅压、积极控制原发疾病等治疗后,临床症状及影像学表现均短期内好转。结论 RPLS主要临床表现包括癫痫发作、头痛、视觉障碍、意识障碍。头颅MRI是诊断RPLS的重要辅助检查。早期诊断及积极治疗RPLS患儿,大部分预后良好。
[Abstract]:Objective to investigate the clinical and imaging features of children with reversible posterior leukoencephalopathy syndrome (RPLS) and to improve the understanding of RPLS among pediatric clinicians. Methods the clinical manifestations, imaging data, treatment and prognosis of 10 patients with RPLS from 2009 to 2015 in Renal Immunology Department of Shenzhen Children's Hospital were retrospectively analyzed. Results all the 10 cases had acute onset, including 4 cases secondary to Henoch-Schonlein purpura and 3 cases of nephrotic syndrome. Two cases of systemic lupus erythematosus with IgA nephropathy were treated with immunosuppressant. 10 cases all had epileptic seizures in 8 cases with hypertension and 4 cases with dizziness, headache, 3 cases with visual abnormality, 3 cases with consciousness disorder and 2 cases with nausea. All the 10 children with vomiting were well examined with MRI. The imaging manifestations were mainly long T 1, long T 2 signal in the posterior part of the brain, and most of them were symmetrical. 10 cases of RPLS were treated with control of convulsion, reduction of intracranial pressure, active control of primary diseases, and so on. The clinical symptoms and imaging manifestations were improved in a short time. Conclusion the main clinical manifestations of RPLS include seizures, headache, visual disorders and consciousness disorders. Cranial MRI is an important auxiliary examination for the diagnosis of RPLS. Early diagnosis and active treatment of children with RPLS, most of the prognosis is good.
【作者单位】：深圳市儿童医院肾脏免疫科;
【基金】：深圳市科技创新委员会项目(201404163000461) 深圳市卫生和计划生育委员会科研项目(201606036)
【分类号】：R742

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