Rasmussen's encephalitis - Early characteristics allow diagnosis
Rasmussen's encephalitis - Early characteristics allow diagnosis
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DOI：
10.1212/wnl.60.3.422
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发表时间：
2003-02-11
期刊：
影响因子：
9.9
通讯作者：
Fusco, L
中科院分区：
文献类型：
--
作者：
Granata, T;Gobbi, G;Fusco, L
目的：确定拉斯穆森脑炎（RE）的早期表现，可以提示早期和合理安全的诊断，允许在早期阶段进行药物或手术治疗，因为它们可能更有效地减缓疾病。研究方法：作者研究了12例临床和神经病理诊断为RE的患者，从疾病开始，评估临床病史，影像学和EEG，并关注早期特征。还考虑在11例患者中进行抗GluR3抗体测定。结果如下：在首次出现症状后4个月，所有病例均出现1）以运动成分为主的难治性局灶性癫痫发作，2）运动表现对侧EEG上的缓慢局灶性活动，3）神经影像学上的局灶性对侧白色高信号伴岛叶皮质萎缩。不太恒定或较晚的结果是癫痫部分连续，寡克隆带，血清抗GluR3抗体。结论：部分性癫痫发作与局灶性EEG和神经影像学改变的相关性允许在首次症状后4至6个月初步诊断RE。
Objective: To identify early manifestations of Rasmussen encephalitis (RE) that can prompt early and reasonably secure diagnosis, allowing medical or surgical therapies at an early stage when they may be more effective in slowing the disease. Methods: The authors studied 12 patients with clinical and neuropathologic diagnosis of RE, followed from disease onset, assessing clinical history, imaging, and EEG and focusing on early characteristics. Anti-GluR3 antibody assays were also considered in 11 patients. Results: By 4 months from first symptoms, all cases had 1) refractory focal seizures with a predominant motor component, 2) slow focal activity on EEG contralateral to the motor manifestations, and 3) focal contralateral white matter hyperintensity with insular cortical atrophy on neuroimaging. Less constant or later findings were epilepsia partialis continua, oligoclonal bands, and serum anti-GluR3 antibodies. Conclusions: The association of partial seizures with focal EEG and neuroimaging changes allows a tentative diagnosis of RE 4 to 6 months after first symptoms.