Rosman N P, Gottlieb S M, Bernstein C A
Department of Pediatrics, New England Medical Center, Boston, MA, USA.
J Child Neurol. 1997 Oct;12(7):448-54. doi: 10.1177/088307389701200707.
A case of acute hemorrhagic leukoencephalitis (AHLE) in a 6-year-old girl is reported. The presentation was typical for acute hemorrhagic leukoencephalitis, with acute onset of a rapidly progressive neurologic disorder with asymmetric involvement of brain, with polymorphonuclear predominant peripheral leukocytosis and cerebrospinal fluid pleocytosis. Cerebrospinal fluid findings not previously reported included elevation of IgG and the presence of myelin basic protein. Additional previously unreported findings were striking abnormalities on magnetic resonance imaging (MRI) of the brain, in contrast to normal findings on computed tomography (CT). The child was treated with high dose intravenous steroids and made a full recovery, with a parallel disappearance of all of her cerebrospinal fluid abnormalities and almost all of her abnormalities on MRI. Detailed examination of cerebrospinal fluid and MRI of brain should facilitate early diagnosis in other cases of suspected acute hemorrhagic leukoencephalitis and high-dose steroid therapy may lead to improved clinical outcomes.
报告了一名6岁女孩患急性出血性白质脑炎(AHLE)的病例。其临床表现为急性出血性白质脑炎的典型症状,急性起病,迅速进展的神经系统疾病,脑受累不对称,外周血多形核白细胞为主的白细胞增多以及脑脊液细胞增多。脑脊液检查中先前未报告的发现包括IgG升高和髓鞘碱性蛋白的存在。其他先前未报告的发现是脑部磁共振成像(MRI)有明显异常,而计算机断层扫描(CT)结果正常。该患儿接受了大剂量静脉注射类固醇治疗并完全康复,其所有脑脊液异常以及MRI上几乎所有异常均同时消失。对脑脊液和脑部MRI进行详细检查应有助于疑似急性出血性白质脑炎其他病例的早期诊断,大剂量类固醇治疗可能会改善临床结局。