Liptai Zoltán, Ujhelyi Eniko, Mihály Ilona, Rudas Gábor, Barsi Péter
László Kórháza, Gyermekgyógyászati Osztály, 1097 Budapest, Gyáli út 5-7.
Ideggyogy Sz. 2009 Jul 30;62(7-8):244-54.
Acute disseminated encephalomyelitis is a rare inflammatory demyelinating disorder often preceded by infection or vaccination. The purpose of the study was the systematic analysis of clinical, radiological and microbiological profiles of children treated at Szent László Hospital, and the comparison of findings with literature data.
Demographic, infectological, clinical, radiological, laboratory and virological data of patients treated and followed-up between 1-Jan-1998 and 30-June-2008 were reviewed and analysed.
19 children met diagnostic criteria. Their mean age was 6.8 years. A prodromal illness--mostly febrile viral infection, upper respiratory infection or chickenpox--preceded neurological symptoms in 17 patients. All had polysymptomatic encephalopathy, 2 children had spinal symptoms. The cerebrospinal fluid was abnormal in all but one. A viral etiology was definite in 7 and probable in 8 cases. MRI disclosed white matter changes in 18, cortical and deep gray matter in 16, cerebellar in 6, brain stem in 14 and spinal cord changes in 2 cases. Repeat MRI performed mean 4 months later showed complete resolution in 6 and partial resolution in 11 patients. 13 patients received high-dose methylprednisolone, 2 of whom were also treated with plasma exchange and 1 with immunoglobulin. 9 children required mechanical ventilation. 2 patients died, 10 recovered without and 7 with sequelae. 2 patients developed further demyelinating events: multiple sclerosis and multiphasic disseminated encephalomyelitis, respectively.
Clinical, radiological and follow-up results were similar to those published in literature however, triggering viruses were identified in a larger proportion of cases.
急性播散性脑脊髓炎是一种罕见的炎症性脱髓鞘疾病，常继发于感染或接种疫苗之后。本研究旨在系统分析在圣拉兹洛医院接受治疗的儿童的临床、放射学和微生物学特征，并将研究结果与文献数据进行比较。
回顾并分析了1998年1月1日至2008年6月30日期间接受治疗和随访的患者的人口统计学、感染学、临床、放射学、实验室和病毒学数据。
19名儿童符合诊断标准。他们的平均年龄为6.8岁。17例患者在出现神经症状之前有前驱疾病，主要为发热性病毒感染、上呼吸道感染或水痘。所有患者均有多症状性脑病，2名儿童有脊髓症状。除1例患者外，所有患者的脑脊液均异常。7例病毒病因明确，8例可能为病毒感染。MRI显示18例有白质改变，16例有皮质和深部灰质改变，6例有小脑改变，14例有脑干改变，2例有脊髓改变。平均4个月后复查的MRI显示，6例患者完全恢复，11例部分恢复。13例患者接受了大剂量甲基强的松龙治疗，其中2例还接受了血浆置换，1例接受了免疫球蛋白治疗。9名儿童需要机械通气。2例患者死亡，10例康复无后遗症，7例有后遗症。2例患者发生了进一步的脱髓鞘事件，分别为多发性硬化症和多相性播散性脑脊髓炎。
临床、放射学和随访结果与文献报道相似，然而，在更大比例的病例中发现了引发病毒。
