儿童急性淋巴细胞白血病治疗致可逆性后部白质脑病综合征二例

目的 探讨儿童急性淋巴细胞白血病化疗后合并可逆性后部白质脑病综合征的病因、临床表现、影像学特征及治疗.方法 分析2例可逆性后部白质脑病综合征患儿的临床资料并复习文献.结果 2例患儿主要表现均有嗜睡、头痛、视物不清、伴有癫癎发作,影像学以可逆性白质异常病变为特征.多位于半球后部白质.经正确治疗后,患儿的临床表现消失,影像学恢复正常.结论 可逆性后部白质脑病综合征的临床表现无特异性,经恰当治疗均可恢复正常.     

儿童急性肾小球肾炎合并可逆性后部白质脑病综合征1例

患儿男,12岁4个月,因水肿、头昏伴视物模糊6d入院。患儿6d前无明显诱因出现颜面、腹部及双下肢水肿,头昏、视物模糊并呕吐,在当地医院采用头孢类抗生素治疗4 d,无好转,后转至我院。起病以来,患儿精神差,食欲差,睡眠尚可,小便色黄、量正常,大便正常。既往无高血压及其他特殊病史。家族史无特殊。     

儿童可逆性后部脑病综合征研究进展

可逆性后部脑病综合征是一种临床神经影像学综合征。临床特点包括头痛、意识障碍、癫癎发作及视觉障碍。影像学特征为大脑后部白质或 （和） 灰质病变。该病发生机制尚未完全明确,内皮损伤机制可能是关键因素。基础疾病是发病的重要因素,也是临床诊断的关键线索。大多数病例预后良好,临床和影像学异常可恢复。但严重病例如合并急性脑出血或大面积后颅窝水肿,导致梗阻性脑积水或脑干受压者可出现严重神经系统损害,常遗留后遗症,甚至导致死亡。早期识别,积极、恰当的治疗具有十分重要的意义。     

过敏性紫癜并可逆性后部白质脑病综合征1例

过敏性紫癜是儿童时期最常见的血管炎之一,以非血小板减少性紫癜、关节炎或关节痛、腹痛、胃肠道出血及肾炎为主要临床表现,可逆性后部白质脑病综合征(RPLES)是由多种病因引起的以神经系统受损为主要表现的临床综合征,现将本院儿科收治的1例过敏性紫癜并RPLES患儿临床特征报告如下.     

儿童急性淋巴细胞白血病并发可逆性后部白质脑病综合征1例

<正>患儿,女,8岁,因间断发热20余d,咳嗽2 d入院。入院时查体:T41℃,P125次/min,R30次/min,BP 105/75 mmHg。神志清楚,贫血貌,颈部可触及蚕豆大小淋巴结数枚,质韧无触痛,周身无皮疹。双肺呼吸音粗,无罗音。心音有力,节律规整,可闻及     

合并可逆性后部白质脑病综合征的横纹肌溶解症一例

患儿,男,13岁,因“双下肢疼痛10 d、呕吐6 d、尿少及头痛2 d”入我院PICU。10 d前患儿做100次深蹲运动后双下肢肌肉剧烈疼痛,无发热、关节肿痛、皮疹、水肿、尿色异常等,未告知家属,症状逐渐加重至不能行走;6d前出现纳差、呕吐,无腹泻,当地查腹部彩超未见明显异常;2d前出现尿少、头痛、精神差,无意识障碍、抽搐等,服用头孢类抗生素效差,症状逐渐加重,遂急诊至我院。平素体健,既往史、个人史及家族史无特殊。     

儿童可逆性后部脑病综合征2例报告并文献复习

目的提高对儿童可逆性后部脑病综合征(RPES)的认识。方法回顾性分析首都儿科研究所收治的2例可逆性后部脑病综合征患儿的临床资料,并结合文献进行分析。结果2例患儿原发病分别为系统性红斑狼疮和肾病综合征,均在病程中突然出现头痛、视觉异常、意识障碍、高血压和抽搐等症状。头颅MRI显示双侧大脑顶、颞、枕叶皮层或皮层下片状长T1/T2信号。经过及时降血压和对症治疗后,短期内症状很快缓解,2～3周内影像学异常完全消失。结论RPES的发病机制是多方面的,急剧增高的血压可能是发生RPES的主要原因之一,及时有效的降压治疗可使病情在短期内逆转。     

儿童肾脏疾病合并可逆性后部脑病综合征6 例临床分析

目的探讨儿童肾脏疾病合并可逆性后部脑病综合征(PRES)的临床特点。方法回顾分析2015-2018年确诊的6例肾脏疾病合并PRES患儿的临床资料,并复习相关文献。结果 6例患儿中男4例、女2例,年龄3～14岁。原发病分别为肾病综合征2例、紫癜性肾炎2例、过敏性紫癜1例、狼疮性肾炎1例,均有激素及免疫抑制剂应用史。临床表现均为急性起病,抽搐、意识丧失、血压升高,还有精神行为异常、呕吐、头痛、视物障碍表现。影像学表现均以可逆性白质异常病变为特征,多位于半球后部白质。5例患儿恢复至发病前状态,1例死亡。结论肾脏疾病应用激素及免疫抑制剂的患儿易合并PRES,一般预后良好,但并非完全可逆。     

Hypertension induced reversible posterior leukoencephalopathy syndrome: a report of two cases

Reversible posterior leukoencephalopathy syndrome (RPLS) is a recently described disorder with typical radiological findings of bilateral grey and white matter abnormalities in the posterior regions of the cerebral hemispheres. The majority of patients with RPLS are adults and it is rare in children. In this report, two patients with RPLS are presented. In the first patient the primary diagnosis was acute post-streptococcal glomerulonephritis, a known cause of RPLS both in adults and in children. The second patient had Henoch Schönlein purpura. Conclusion:These patients are presented to highlight the importance of reversible posterior leukoencephalopathy syndrome. As the spectrum of associated diseases is diverse, paediatricians must be aware of this syndrome in order to initiate appropriate management.     

Simultaneous occurrence of posterior reversible leukoencephalopathy syndrome in two cases of childhood acute lymphoblastic leukemia induction chemotherapy

We describe two cases Posterior Reversible Encephalopathy Syndrome (PRES) occurring in childhood B-precursor acute lymphoblastic leukemia within a week of each other during induction chemotherapy in our ward. We review the literature related to the possible etiology of PRES in these cases.     

儿童紫癜性肾炎相关的可逆性后部脑白质综合征 2 例报告

目的探讨儿童紫癜性肾炎(HSPN)合并可逆性后部脑白质综合征(PRLS)的发病机制、诊断、治疗和预后。方法回顾分析2例HSPN合并PRLS患儿的临床资料,并复习相关文献。结果 2例患儿均为男性,分别为8岁、14岁。因水肿、蛋白尿明显,给予激素、免疫抑制剂、丙种球蛋白、血浆、白蛋白等治疗。病程中出现抽搐发作、意识丧失,头颅MRI显示额叶、顶叶、枕叶等部位多发异常信号,经止惊、降颅压等治疗,抽搐停止、意识恢复,MRI异常信号较前明显改善。结论在HSPN治疗过程中需警惕PRLS。     

Reversible posterior leukoencephalopathy syndrome in children with cancers

We report three cases of reversible posterior leukoencephalopathy syndrome (RPLS) in children with cancers. All patients presented with sudden confusional state, visual disturbance, and transient mild hypertension under the treatment of childhood cancers. Magnetic resonance imaging of the brain demonstrated cortical and subcortical lesions predominantly in the occipital region, which showed high intensity signal on fluid-attenuated inversion recovery images. All patients completely recovered from their neuropsychologic deficits only with antihypertensive therapy or discontinuation of the possible offending drugs. Early recognition of RPLS as a complication during cancer therapy in childhood may facilitate precise diagnosis and appropriate treatment.     

The role of tumor lysis in reversible posterior leukoencephalopathy syndrome

We report on a child with B-cell lymphoma who developed hypertension and reversible posterior leukoencephalopathy syndrome (RPLS) after chemotherapy conducted during recovery from tumor lysis syndrome. After recovery from RPLS, the patient received further combination chemotherapy without recurrence of the neurological signs or symptoms suggestive of RPLS. Many etiological factors have been reported in the development of RPLS; however, little attention has been paid to tumor lysis syndrome as a contributory factor for RPLS. Tumor lysis syndrome can precipitate the development of RPLS in patients with hematological malignancies who are undergoing chemotherapy. Knowledge and awareness would help facilitate immediate management such as normalization of blood pressure and temporary cessation of chemotherapy, helping to avoid irreversible brain damage.     

儿童系统性红斑狼疮合并脑后部可逆性脑病综合征4例并文献复习

目的 分析儿童系统性红斑狼疮（SLE）合并脑后部可逆性脑病综合征（PRES）的临床特征,提高对本病的认识。方法 报告北京协和医院诊断的4例儿童SLE合并PRES的临床资料,在PubMed数据库检索相关病例行文献复习,分析儿童SLE合并PRES的临床表现、影像学检查、治疗及预后情况。结果 ①4例SLE患儿均伴有狼疮性肾炎,从确诊SLE至出现PRES的时间1~63个月,出现PRES时1例正在接受甲泼尼龙及环磷酰胺(CTX)冲击治疗。PRES均以惊厥、头痛起病,并伴血压升高。②PubMed数据库检索到11例SLE合并PRES的儿童病例,结合本文报道的4例,15例进入分析。女性14例。年龄最小8岁。从确诊SLE至出现PRES的间隔中位时间为6个月（1个月至8年）。15例出现PRES时均有惊厥发作,10例伴头痛,7例呕吐,9例意识丧失,7例视力障碍。15例均有血压升高。12例有狼疮性肾炎。治疗SLE予甲泼尼龙或CTX冲击治疗分别为4和3例,予羟氯喹2例,予环孢素和利妥昔单抗各1例,PRES分别发生在免疫抑制剂治疗后的2 d至4年。15例行头颅MRI检查示大脑后循环皮质下白质受累为主。12例予降血压治疗,10例予抗惊厥药物短期治疗。9例SLE处于活动期,继予糖皮质激素和免疫抑制剂治疗;6例非活动期的SLE患儿减停糖皮质激素及免疫抑制剂。15例神经系统症状均恢复,随访均未遗留神经系统后遗症。9例复查头颅MRI示颅内病变完全或基本消失。结论 儿童SLE合并PRES主要表现为惊厥、头痛、意识障碍和视觉障碍。对于有狼疮性肾炎的SLE患儿血压升高时,尤其同时予大剂量糖皮质激素或CTX等免疫抑制剂治疗时,应警惕PRES的发生。早期诊断和治疗PRES预后较好。     

L-asparaginase-induced reversible posterior leukoencephalopathy syndrome in a child with acute lymphoblastic leukemia

Reversible posterior leukoencephalopathy syndrome (RPLS) is being increasingly described with various etiologies even in the absence of hypertension. We present an 11-year-old patient with acute lymphoblastic leukemia who presented with seizures while on treatment with L-asparaginase. MRI showed bilaterally symmetrical nonenhancing occipital lesions characteristic of RPLS. L-Asparaginase-induced RPLS is a rare cause of neurological symptoms in patients on induction chemotherapy.     

Reversible posterior leukoencephalopathy syndrome: report of 2 simultaneous cases in children

Reversible posterior leukoencephalopathy syndrome (RPLS) is a rare complication of cancer chemotherapy. We have recently observed two cases occurred simultaneously in children receiving different chemotherapy regimens, for hepatoblastoma and acute lymphoblastic leukaemia, respectively. Both children presented with altered mental status, severe visual disturbances, headache, seizures, backpain and hypertension. Magnetic resonance imaging showed cortical and subcortical lesions especially in the occipital and parietal regions, strongly consistent with RPLS. Both patients completely recovered from their neuropsychologic deficits in about ten days only with anticonvulsant and antihypertensive therapy, and chemotherapy regimen was promptly restarted according to the planned protocol, without any neuropsychological sequela. A mild left midriasis was the only neurologic defect that persisted in the patient with acute lymphoblastic leukemia.