儿童伴胼胝体压部可逆性病变的轻微脑炎/脑病的临床研究

目的 探讨儿童伴有胼胝体压部可逆性病变的轻微脑炎/脑病(clinically mild encepha-litis/encephalopathy with a reversible splenial lesion,MERS)的临床及影像学特征.方法 回顾性分析2016年1月至6月唐山市妇幼保健院小儿神经内科收治的8例MERS患儿,对其病因、临床表现、影像学资料、治疗及随访进行总结分析.结果 8例患儿中,男1例,女7例,年龄1.58~5岁,中位年龄1.75岁.其中7例患儿因感染起病,1例病因不明,神经系统体征轻微或无.8例患儿的头颅MRI检查均显示为胼胝体压部异常信号,T1WI等或稍低信号,T2WI高或稍高信号,DWI高信号.治疗上8例患儿均予抗感染治疗,7例加小剂量的地塞米松及甘露醇治疗,其中1例加免疫球蛋白治疗.全部患儿在发病后1周左右临床症状完全缓解,发病后8~15 d复查MRI显示病灶消失.随访1~6个月,头颅MRI均正常.结论 儿童MERS病因复杂,临床表现无特异性,诊断依赖头颅MRI,尤其是DWI序列,突出特征是胼胝体压部的高信号,预后良好.     

儿童伴胼胝体压部可逆性病变的轻微脑炎或脑病临床特征研究

目的 比较中国儿童伴胼胝体压部可逆性病变的轻微脑炎或脑病（MERS）的病因、临床特征,以及与日本、美国、比利时儿童MERS的异同点。方法　对2013年1月至2014年12月中国人民解放军总医院儿童医学中心收治的2例儿童MERS临床特征及影像学特点进行总结;同时以2000年1月至2015年2月为限,通过检索中国期刊全文数据库（CNKI）、中国生物医学文献数据库（CBM）、Pubmed及Web of science数据库,对检索到的儿童MERS临床资料进行对比分析。结果　共纳入儿童病例115例,男女比例1.1∶1,发病年龄为6个月至14岁;多发生于感染性疾病过程中,58.3%的患儿感染病原体明确,其中以流感病毒、轮状病毒感染多见,其次为细菌感染及肺炎支原体感染;神经系统症状可表现为精神行为错乱（42.6%）、抽搐发作（37.4%）、意识下降（5.2%）或意识障碍（25.2%）、构音障碍（6.9%）等;实验室检查缺乏特异性,影像学显示为孤立性胼胝体压部或累及整个胼胝体及对称性周围白质病变;经抗感染及对症支持治疗后,症状消失,随访磁共振成像（MRI）病灶可完全消失,无任何严重神经系统后遗症。结论　感染成为儿童MERS最常见的诱因,临床表现为脑炎或脑病症状,头颅MRI发现以胼胝体压部为主的可逆性病变有提示作用,无须特殊治疗,短期内可自行恢复,预后较好。     

儿童伴胼胝体压部可逆性病变的轻度脑炎/脑病的临床特点分析

目的 探讨儿童伴胼胝体压部可逆性病变的轻度脑炎/脑病(MERS)的临床特点.方法 对在我院住院的5例MERS患儿进行回顾性分析,对其病因、临床表现、头颅影像学检查、治疗及随访进行总结分析.结果 5例患儿中,发病年龄1岁6个月~6岁,平均年龄3.1岁,均有前驱感染史,3例以消化道症状起病,2例以呼吸道症状起病,5例均有不同程度的倦怠、嗜睡,4例抽搐,1例有头痛及共济失调.5例患儿血电解质、血渗透压、血糖、肝肾功能检查均正常.病原学检查轮状病毒感染3例,肺炎支原体感染2例,其中1例肺炎支原体感染合并流感病毒B感染.脑脊液检查4例正常,1例细胞数、蛋白轻度升高.脑电图检查2例呈背景弥漫性慢活动,3例正常.头颅MRI表现胼胝体压部片状T1WI稍低信号,T2WI稍高信号,DWI高信号影,其中1例同时伴有双侧半卵圆中心斑片状DWI高信号影,1~3周后复查均提示异常信号减低或消失.随访半年至2年,所有患儿均未发现神经系统后遗症.结论 儿童MERS发病多与感染有关,临床症状轻微,头颅MRI有特征性表现,多数预后良好.     

儿童肾脏疾病合并后部可逆性脑病综合征临床特点（附4例报告）

目的　提高对儿童肾脏疾病合并后部可逆性脑病综合征（PRES）的临床表现、影像学特征和治疗策略的认识。方法　回顾性分析2010年7月至2018年6月首都儿科研究所附属儿童医院肾脏内科收治的4例肾脏疾病合并PRES患儿的临床资料及影像学结果,结合文献分析其临床特点。结果　患儿原发病分别为急性链球菌感染后肾小球肾炎1例、肾病综合征2例、非典型溶血尿毒综合征1例,起病前有血压控制不良或细胞毒性药物使用史,以抽搐为首发症状,伴有头痛、头晕、视物模糊、血压急剧增高。典型头颅磁共振成像（MRI）示双侧大脑顶枕叶皮质或皮质下白质片状长T1T2 信号,对称或不对称,严重者可累及侧脑室旁、小脑、脑干。4例PRES经过及时降血压和对症治疗后,1～2 d症状缓解,2 ～3周影像学异常完全消失。结论　高血压、药物引起的血管源性脑水肿是发生儿童肾脏疾病合并PRES的主要原因,PRES的诊断依赖于头颅MRI的特征性改变,及时有效的降压和对症治疗可在短期内有效控制病情,治疗中应积极控制高血压,及时停用或减量诱发PRES的细胞毒性药物。     

儿童肾脏疾病合并后部可逆性脑病综合征临床特点（附4例报告）

目的　提高对儿童肾脏疾病合并后部可逆性脑病综合征（PRES）的临床表现、影像学特征和治疗策略的认识。方法　回顾性分析2010年7月至2018年6月首都儿科研究所附属儿童医院肾脏内科收治的4例肾脏疾病合并PRES患儿的临床资料及影像学结果，结合文献分析其临床特点。结果　患儿原发病分别为急性链球菌感染后肾小球肾炎1例、肾病综合征2例、非典型溶血尿毒综合征1例，起病前有血压控制不良或细胞毒性药物使用史，以抽搐为首发症状，伴有头痛、头晕、视物模糊、血压急剧增高。典型头颅磁共振成像（MRI）示双侧大脑顶枕叶皮质或皮质下白质片状长T1T2 信号，对称或不对称，严重者可累及侧脑室旁、小脑、脑干。4例PRES经过及时降血压和对症治疗后，1～2 d症状缓解，2 ～3周影像学异常完全消失。结论　高血压、药物引起的血管源性脑水肿是发生儿童肾脏疾病合并PRES的主要原因，PRES的诊断依赖于头颅MRI的特征性改变，及时有效的降压和对症治疗可在短期内有效控制病情，治疗中应积极控制高血压，及时停用或减量诱发PRES的细胞毒性药物。     

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。     

儿童血液肿瘤化疗期间合并可逆性后部脑病综合征5例临床分析并文献复习

目的 探讨儿童血液肿瘤化疗期间合并可逆性后部脑病综合征(posterior reversible encephalopathy syndrome, PRES)的临床特征,提高对本病的认识。方法 回顾性分析2013年11月—2020年11月在山东第一医科大学附属省立医院小儿血液肿瘤科就诊,明确诊断为儿童血液肿瘤疾病(包括白血病、淋巴瘤)且合并PRES的5例患儿的临床资料。结果 5例患儿中男性3例,女性2例,年龄2岁5个月～12岁。患儿确诊后在我院规律化疗,无原发病复发,其中4例PRES发生于本次化疗的第8～28d, 1例发生于维持治疗时,起病时5例患儿均伴有严重感染(其中1例为细菌感染,1例为真菌感染,3例为细菌及真菌合并感染),其中5例同时伴有电解质紊乱(5例低钠血症、4例低钾血症),4例伴有血压升高。临床特征主要表现为头痛、意识不清、癫痫发作(4例为全面性强直-阵挛性发作),伴呕吐、流涎、精神淡漠、尿失禁。起病后即刻行头颅MRI检查显示颞叶、顶叶、枕叶、小脑、丘脑等部位T2WI高信号,FLAIR高信号,MRI显示病灶与患儿临床特征、神经系统查体定位基本一致。经脱水降颅压、抗感染、补充...     

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。     

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??Objective To compare the etiology and clinical characteristics of clinically mild encephalitis/encephalopathy with a reversible splenial lesion of corpus callosum in Chinese children with those cases from different countries. Methods The clinical and imaging features of two children with the diagnosis of MERS were analyzed and pediatric MERS cases were screened from “China National Knowledge Infrastructure”??“China Biology Medicine”??“PubMed ” and “Web of Science” databases for analysis between January??2000 and February??2015. Results A total of 115 cases were collected and the male to female ratio was 1.1 to 1?? with onset age of 6 months to 14 years old. There was usually a certain infection before the onset. About 58.3% of infectious pathogens were detected?? and influenza virus and rotavirus infection were very common pathogens?? followed by bacterial infections and mycoplasma pneumoniae infection. The neurological symptoms manifested as mental and behavioral disorders ??42.6%???? seizures ??37.4%???? decreased consciousness ??5.2%?? or consciousness??25.2%?? and dysarthria ??6.9%??. The MR imaging ??MRI?? findings exhibited lesions in an isolated splenial or the white matter and entire corpus callosum?? and completely disappeared at follow-up MRI. With anti-infection and symptomatic treatments??the clinical symptoms of most patients were relieved without leaving any serious neurological sequelae. Conclusion Infection is a prevalent predisposing factor of MERS in children?? with nonspecific clinical characteristics. The MRI examination plays a role of implication for diagnosis. And all clinical symptoms of patients will disappear within a few days and the prognosis of patients is good.     

Kleine–Levin syndrome elicited by encephalopathy with reversible splenial lesion

Kleine–Levin syndrome is a rare sleep disorder of unknown etiology characterized by repetitive episodes of hypersomnia between asymptomatic periods. We report the case of a 13‐year‐old girl who presented with drowsiness triggered by influenza A as the first episode. Magnetic resonance imaging (MRI) on day 6 showed transient reduction of diffusion in the corpus callosum splenium. The patient was diagnosed with encephalopathy with a reversible splenial lesion. The symptoms resolved after 10 days, but additional episodes of hypersomnia lasting 5–10 days occurred 1, 5, 6, 11, 13, and 25 months after the first episode. MRI during hypersomnia indicated no lesions, and sleep duration and cognition were normal between episodes. The patient was diagnosed with Kleine–Levin syndrome. Electroencephalographic and clinical findings during the first episode were similar to those during the other episodes. Encephalopathy with a splenial lesion and Kleine–Levin syndrome may have similar pathological mechanisms causing a disturbance in consciousness.     

Transient renal dysfunction with reversible splenial lesion

We report the case of a 6‐month‐old boy with transient renal dysfunction who had an intensified signal in the splenium of the corpus callosum on magnetic resonance imaging. He presented to hospital with fever and sudden disturbance of consciousness. Cerebrospinal fluid analysis did not show pleocytosis. The mild consciousness disturbance disappeared after 30 min, but the splenial signal persisted even after 8 days. Further, renal glucosuria, increased excretion of select amino acids, and abnormal fractional excretion of electrolytes were observed, indicating renal tubular dysfunction. The abnormal urinary findings spontaneously resolved by day 9 of hospitalization. The splenial lesion took 21 days to normalize. There were no signs of neurological complications 2 months later. This case suggests the possibility of renal involvement in splenial lesions.     

Clinically mild encephalitis/encephalopathy with a reversible splenial lesion associated with febrile urinary tract infection

Common pathogens of clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) are viruses, such as influenza virus. However, bacteria are rare pathogens for MERS. We report the first patient with MERS associated with febrile urinary tract infection. A 16-year-old lupus patient was admitted to our hospital. She had fever, headache, vomiting, and right back pain. Urinary analysis showed leukocyturia, and urinary culture identified Klebsiella pneumoniae. Cerebrospinal fluid examination and brain single-photon emission computed tomography showed no abnormalities. Therefore, she was diagnosed with febrile urinary tract infection. For further examinations, 99mTc-dimercaptosuccinic acid renal scintigraphy showed right cortical defects, and a voiding cystourethrogram demonstrated right vesicoureteral reflux (grade II). Therefore, she was diagnosed with right pyelonephritis. Although treatment with antibiotics administered intravenously improved the fever, laboratory findings, and right back pain, she had prolonged headaches, nausea, and vomiting. T2-weighted, diffusion-weighted, and fluid attenuated inversion recovery images in brain magnetic resonance imaging showed high intensity lesions in the splenium of the corpus callosum, which completely disappeared 1 week later. These results were compatible with MERS. To the best of our knowledge, our patient is the first patient who showed clinical features of MERS associated with febrile urinary tract infection. Conclusion: In patients with pyelonephritis and an atypical clinical course, such as prolonged headache, nausea, vomiting, and neurological disorders, the possibility of MERS should be considered.     

Mild influenza-associated encephalopathy/encephalitis with a reversible splenial lesion in a Caucasian child with additional cerebellar features

Mild influenza-associated encephalitis/encephalopathy with a reversible splenial lesion is a rare entity recently reported almost exclusively in Asiatic individuals. Hallmarks of this clinical-radiological syndrome include severe encephalopathy at onset, prompt and complete recovery, minimal to absent pleocytosis and rapidly reversible involvement of the splenium of the corpus callosum. We report herein a young Caucasian child who in addition had cerebellar involvement and presented a transient mutism during the recovery phase.