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Fulminant encephalopathy in a child with hyperferritinemic sepsis: a case report.
BMC Neurology ( IF 2.2 ) Pub Date : 2020-03-02 , DOI: 10.1186/s12883-020-01661-z
Lilin Huang 1 , Shumei Peng 1 , Ronghan Li 1 , Danyu Xie 1 , Dongping Huang 1
Affiliation  

BACKGROUND Sepsis-associated encephalopathy (SAE) is epidemic in intensive care units and recognized as a fatal complication of sepsis. SAE is characterized by diffuse brain dysfunction and the correct diagnosis of SAE requires ruling out direct central nervous system (CNS) infection or other types of encephalopathy, such as hepatic encephalopathy, pulmonary encephalopathy, and other encephalopathy. CASE PRESENTATION We reported a rare case of a 5-year-old girl who presented with abdominal pain, vomiting, recurrent seizures, and coma. Brain magnetic resonance imaging (MRI) showed diffuse white matter abnormalities in the brain on day 1. Cerebrospinal fluid (CSF) tests revealed that protein levels and glucose levels were normal without pleocytosis. CSF PCRs for pathogens were all negative. The electroencephalography examination demonstrated diffuse, generalized and slow background activity. The patient showed the symptom of hyperferritinemic sepsis with multiple organ dysfunction syndrome (MODS). SAE was also diagnosed by ruling out other encephalitis or encephalopathy. The patient made marked improvements of clinical symptoms and the lesions on brain imaging disappeared completely within two months after appropriate treatment including antibiotic treatments, methylprednisolone, intravenous immunoglobulin, membrane-based therapeutic plasma exchange (TPE), and continuous renal replacement therapy (CRRT). CONCLUSIONS SAE can be a fatal complication of sepsis which asks for immediate diagnosis and treatment. Few reports have focus on MRI imaging findings on the early onset of hyperferritinemic sepsis with MODS since these children were too ill to undergo an MRI scan. However, SAE might appear before other systemic features of sepsis are obvious, and MRI could show abnormal lesion in the brain during the early course. Therefore, MRI should be performed early to diagnose this fatal complication which would play important roles in improving the clinical outcomes by early initiation with appropriate treatments.

中文翻译:

高铁蛋白败血症患儿的暴发性脑病:一例报告。

背景技术脓毒症相关性脑病(SAE)在重症监护病房中流行,并且被认为是脓毒症的致命并发症。SAE以弥漫性脑功能障碍为特征,正确诊断SAE需要排除直接中枢神经系统(CNS)感染或其他类型的脑病,例如肝性脑病,肺性脑病和其他脑病。病例介绍我们报告了一个罕见的5岁女孩的案例,该女孩表现出腹痛,呕吐,反复发作和昏迷。第1天的脑磁共振成像(MRI)显示了大脑中弥漫性的白质异常。脑脊液(CSF)测试显示,蛋白水平和葡萄糖水平正常,无卵黄细胞增多症。病原体的CSF PCR均为阴性。脑电图检查显示背景活动弥漫,泛滥和缓慢。该患者表现出高铁蛋白败血症伴多器官功能障碍综合征(MODS)的症状。通过排除其他脑炎或脑病也可以诊断出SAE。该患者的临床症状明显改善,经过适当的治疗,包括抗生素治疗,甲基强的松龙,静脉内免疫球蛋白,基于膜的血浆置换治疗(TPE)和连续性肾脏替代治疗(CRRT),在两个月内脑成像的病变完全消失了。结论SAE可能是败血症的致命并发症,需要立即诊断和治疗。很少有报道关注MODS高铁蛋白血症败血症的早期发作的MRI影像学发现,因为这些孩子病得太重,无法进行MRI扫描。但是,SAE可能在败血症的其他全身特征明显之前就出现了,MRI在早期病程中可能显示出大脑中的异常病变。因此,应该尽早进行MRI诊断这种致命的并发症,这将在通过适当的治疗及早开始改善临床结果方面发挥重要作用。
更新日期:2020-03-03
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