Diffusion MRI abnormalities after prolonged febrile seizures with encephalopathy
Diffusion MRI abnormalities after prolonged febrile seizures with encephalopathy
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DOI：
10.1212/01.wnl.0000210487.36667.a5
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发表时间：
2006-05-09
期刊：
影响因子：
9.9
通讯作者：
Koyasu, Y
中科院分区：
文献类型：
--
作者：
Takanashi, J;Oba, H;Koyasu, Y
背景：以长时间癫痫发作为首发症状的脑病患者在弥散加权磁共振成像(DWI)上常可见皮质下白质异常。目的：确定这些患者是否有其他共同特征。方法：对以长时间惊厥为先兆，经MRI发现皮质下白质异常的脑病患者进行回顾性分析。回顾了他们的临床、实验室和放射学数据。结果：确诊患者17例，年龄10个月至4岁。所有患者的首发症状都是长时间的热性发作(12例患者超过1小时)。16名患者在首次发作后4至6天出现继发性癫痫发作，最常见的是复杂的部分癫痫发作。结果从几乎正常到严重的精神发育迟缓。在发病后2天内进行核磁共振检查，未发现异常。17例患者在3~9天的DWI上均可见皮质下白质病变。T2加权像显示13例患者皮质下U纤维呈线状高信号。病变部位以额叶或额顶为主，少有乳突周围区。弥散异常在第9~25天消失，2周后出现脑萎缩。3例仅有额叶病变的患者有相对较好的临床结果。结论：虽然病理生理机制尚不清楚，但这些患者似乎有一种独特的脑病综合征。MRI对本病的诊断有一定帮助。
Background: Patients with encephalopathy heralded by a prolonged seizure as the initial symptom often have abnormal subcortical white matter on diffusion-weighted MRI (DWI). Objective: To determine if these patients share other common features. Methods: Patients with encephalopathy heralded by a prolonged seizure and followed by the identification of abnormal subcortical white matter on MRI were collected retrospectively. Their clinical, laboratory, and radiologic data were reviewed. Results: Seventeen patients were identified, ages 10 months to 4 years. All had a prolonged febrile seizure (longer than 1 hour in 12 patients) as their initial symptom. Subsequent seizures, most often in clusters of complex partial seizures, were seen 4 to 6 days after the initial seizure in 16 patients. Outcome ranged from almost normal to severe mental retardation. MRI performed within 2 days of presentation showed no abnormality. Subcortical white matter lesions were observed on DWI between 3 and 9 days in all 17 patients. T2-weighted images showed linear high intensity of subcortical U fibers in 13 patients. The lesions were predominantly frontal or frontoparietal in location with sparing of the perirolandic region. The diffusion abnormality disappeared between days 9 and 25, and cerebral atrophy was detected later than 2 weeks. Three patients having only frontal lesions had relatively good clinical outcome. Conclusions: Although the pathophysiologic mechanism remains unknown, these patients seem to have a distinctive encephalopathy syndrome. MRI is helpful in establishing the diagnosis of this encephalopathy.