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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。
Objective To investigate the clinical and imaging characteristics of children with reversible posterior leukoencephalopathy syndrome (RPLS) and to improve pediatric clinicians’ understanding of RPLS. Methods The clinical manifestations, imaging data, treatment and prognosis of 10 children with RPLS from 2009 to 2015 in Department of Nephrology, Shenzhen Children’s Hospital were retrospectively analyzed. Results 10 cases were acute onset, including secondary to allergic purpura in 4 cases, nephrotic syndrome in 3 cases, 2 cases of systemic lupus erythematosus, IgA nephropathy in 1 case. 10 cases were immunosuppressive agents. 10 cases of RPLS have seizures, 8 cases of hypertension, dizziness and headache in 4 cases, visual abnormalities in 3 cases, disturbance of consciousness in 3 cases and nausea and vomiting in 2 cases. All 10 children underwent perfect magnetic resonance imaging (MRI) examination. The imaging findings mainly consisted of long T1 and long T2 signals mainly in the posterior part of the brain, most of which were symmetrical. 10 cases of RPLS after the control of convulsions, decreased intracranial pressure, active control of primary disease and other treatment, the clinical symptoms and imaging findings are improved in the short term. Conclusion The main clinical manifestations of RPLS include seizures, headache, visual impairment and disturbance of consciousness. Head MRI is an important auxiliary examination for diagnosis of RPLS. Early diagnosis and aggressive treatment of RPLS children, most of the prognosis is good.