本文選題：可逆性后部腦白質病綜合征 + 兒童��； 參考：《中國實用兒科雜志》2017年07期

【摘要】：目的探討兒童可逆性后部腦白質病綜合征(RPLS)臨床及影像學特點,提高兒科臨床醫(yī)師對RPLS的認識。方法回顧性分析深圳市兒童醫(yī)院腎臟免疫科2009—2015年10例RPLS患兒臨床表現、影像學資料、治療及預后。結果 10例均急性起病,其中繼發(fā)于過敏性紫癜4例、腎病綜合征3例、系統(tǒng)性紅斑狼瘡2例、IgA腎病1例。10例均應用免疫抑制劑。10例RPLS均有癲癇發(fā)作,8例合并高血壓,4例出現頭暈頭痛,3例出現視覺異常,3例出現意識障礙,2例出現惡心、嘔吐。10例患兒均完善頭顱磁共振成像(MRI)檢查,影像學表現主要為大腦后部為主的長T1、長T2信號,大部分呈對稱性。10例RPLS經控制驚厥、降顱壓、積極控制原發(fā)疾病等治療后,臨床癥狀及影像學表現均短期內好轉。結論 RPLS主要臨床表現包括癲癇發(fā)作、頭痛、視覺障礙、意識障礙。頭顱MRI是診斷RPLS的重要輔助檢查。早期診斷及積極治療RPLS患兒,大部分預后良好。
[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.
【作者單位】： 深圳市兒童醫(yī)院腎臟免疫科;
【基金】：深圳市科技創(chuàng)新委員會項目(201404163000461) 深圳市衛(wèi)生和計劃生育委員會科研項目(201606036)
【分類號】：R742

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