急性淋巴细胞白血病患儿诱导治疗后发生可逆性后部脑病综合征的临床及影像学特点

Clinical and imaging characteristics of posterior reversible encephalopathy syndrome in children with acute lymphoblastic leukemia after chemotherapy induction

目的 探讨急性淋巴细胞性白血病（ALL）患儿在诱导化疗期间发生可逆性后部脑病综合征（PRES）的临床特征、MRI特点及其可能的发病机制.方法 根据中华医学会儿科学分会制定的标准进行ALL的诊断与治疗,共有11例ALL患儿在诱导化疗后出现PRES.对这11例患儿的临床表现、影像学特点、预后进行研究并随访1年.患儿均在首次影像学检查后1、3、6、12个月时随访检查.结果 临床表现主要为头痛（10/11例）、癫（痫）发作（7/11例）、高血压（4/11例）、视力障碍（6/11例）、意识障碍（5/11例）和走路不稳（2/11例）.颅脑MRI显示病灶分布于枕叶（9/11例）、顶叶（8/11例）、额叶（5/11例）、颞叶（3/11例）、侧脑室周围及半卵圆中心深部白质（2/11例）、小脑半球（1/11例）等不同部位,具有多灶性、对称性、后部分布为主的特点.PRES发生后立即停用化疗药物及对症处理,大多数病例临床症状及影像学病灶逐渐消失.随访1年,9例预后良好,无后遗症;1例遗留症状性癫（痫）（颅脑MRI示左侧颞叶病灶）,1例遗留轻度视力障碍.各病例临床症状和颅脑MRI病灶消失后继续化疗,未见再次PRES发生.结论 虽然PRES临床与影像学特征具有多样性,但当神经系统症状出现时,PRES可能是ALL化疗期间重要并发症.PRES早期诊断与治疗具有可逆性,因此应该重视PRES的发生,使ALL得到更好的治疗.

Objective To investigate the clinical features, brain imaging significance and the possible pathogenesis of posterior reversible encephalopathy syndrome （PRES） in childhood acute lymphoblastic leukemia （ALL） followed by chemotherapy induction.Methods The diagnosis and treatment of ALL were performed according to the guidelines of the Pediatric Association of Chinese Medical Association.There were 11 cases of pediatric ALL who developed PRES after chemotherapy induction.The clinical presentations, initial and follow-up radiologic features, and the neurologic outcomes of these 11 cases were investigated for one-year follow-up.All patients were reexamined 1,3,6, and 12 months after first imaging.Results Headache （10/1 1 cases）, epileptic seizure （7/11 cases）, high blood pressure （4/11 cases） ,visual impairment （6/11 cases） ,disturbance of consciousness （5/11 cases） and walking instability （2/11 cases） were the most common symptoms of these ALL patients with PRES.Magnetic resonance imaging （MRI） scanning revealed that lesions were mainly distributed in occipital lobe （9/11 cases）, parietal lobe （8/11 cases）, frontal lobe （5/11 cases） ,temporal lobe （3/11 cases）, the deep white matter of bilateral periventricular and centrum semiovale （2/11 cases） and hemisphaerium cerebelli （1/11 cases）.The radiological findings indicated that lesions had multifocal,symmetrical and posteriorly distributed characteristics in the cerebral hemispheres.After the diagnosis of PRES,patients stopped chemotherapy courses promptly and received symptomatic treatment, and then the clinical and imaging symptoms of most cases gradually disappeared.After 1-year follow-up,9 patients had good prognosis and no sequelae, 1 patient had symptomatic epilepsy （brain magnetic resonance imaging scan showed lesions in the left temporal lobe） ,and 1 patient had slight visual impairment.After the craniocerebral symptoms disappeared clinically ALL chemotherapy continued in all patients and no recurrent PRES was observed.Conclusions Although the clinical and imaging features of PRES may be diverse ,PRES should be recognized as a possible important complication of ALL when neurological symptoms appear.However, PRES is reversible when the patients are diagnosed and treated at an early stage.Thus,the occurrence of PRES should be considered and investigated to optimize the early induction schemes for ALL treatment.