儿童血液病并发后部可逆性脑病综合征临床分析

Cerebral posterior reversible encephalopathy syndrome in 9 children with hematological diseases

目的探讨儿童血液病治疗期间并发后部可逆性脑病综合征（PRES）的临床及影像学特点．以提高对该病的认识。方法收集南方医科大学珠江医院儿科血液组自2012年1月至2016年12月收治的并发PRES的9例血液病患儿资料。对该病的诱发因素、临床表现、头颅MRJ特点及预后情况等进行回顾性分析。结果（1）诱发因素分析：急性淋巴细胞白血病诱导缓解化疗阶段发生PRES比例高（6／9）。（2）临床表现：患儿均为急性发病，主要表现为癫痫发作（8／9）和高血压（7／9），可伴有头痛、呕吐、视觉障碍、意识障碍、精神差等症状。（3）头颅MRI特点：病灶主要位于顶枕叶，呈片状长T1、长T2信号，双侧不完全对称，FLAIR像呈高信号，亦可累及其他部位。（4）预后：7例患儿恢复良好，1例随访2年后仍有癫痫发作，1例遗留精神反应迟滞。结论甲氨蝶呤及环孢素A等药物是儿童血液病治疗期间并发PRES的重要诱因；癫痫发作和高血压是主要的临床表现；MRI检查是确诊该病的重要手段；PRES并非完全可逆。

Objective To investigate the precipitating factors, clinical manifestations, magnetic resonance imaging （MRI） features and prognoses of children with posterior reversible encephalopathy syndrome （PRES） during the treatment of hematological diseases, and to improve the understanding of the diseases. Methods A total of 9 children with PRES, admitted to our hospital from January 2012 to December 2016, were chosen. The clinical data, including precipitating factors, clinical manifestations, MRI features and prognoses, were retrospectively analyzed. Results （1） Precipitating factors： 6 patients with acute lymphoblastic leukemia occurred PRES during remission induction therapy （6/9, 66.7%） and 3 occurred PRES during oral cyclosporine A after hematopoietic stem cell transplantation or autoimmune diseases （3/9, 33.3%）. （2） Clinical manifestations： all of them were acute onset, and the main symptoms were seizures （8/9, 88.9%） and hypertension （7/9, 77.8%）; some patients suffered from headache, vomiting, visual disturbances, disturbance of consciousness and poor mental symptoms. （3） Features of head MR imaging： the lesions were mainly located in the parietal-occipital lobe, showing patchy long T1 and long T2 signals, and bilateral imperfect symmetry; FLAIR imaging showed high signal distinctly, and other parts of brain could also been involved in. （4） Prognoses： 7 children （77.8%） recovered well, one （11.1%） left frequent seizures during 2 years of follow up, one （11.1%） left mental retardation. Conclusion Methotrexate, cyclosporine A and other agents are important incentives in children with PRES during the treatment of hematological diseases; seizures and hypertension are the main clinical manifestations; MR imaging is important in diagnosing the disease; and PRES is not completely reversible.