Background

Autopsy studies in Rasmussen encephalitis reveal areas of sparing within the affected hemisphere. Clinical progression and inflammation are milder with an older onset. We sought to demonstrate radiological corroboration for these patterns.

Methods

In our retrospective study, 38 cases were dichotomized into severe pan-hemispheric (all lobes involved) and sub-hemispheric groups (others) to identify age demographics and other severity predictors. The extent and patterns of radiological pathology in the cortex and subcortical structures were assessed by structured visual grading. Relevant clinical data were also reported.

Results

Children with pan-hemispheric involvement were younger at onset (P < 0.001) and were more likely to present with status epilepticus (odds ratio 8.5, 95% confidence interval 1.5 to 50.0, P = 0.022). A history of perinatal asphyxia/hospitalization (P < 0.001) and delayed milestones (P = 0.013) were encountered exclusively in this group, and progression to a low-amplitude record background on electroencephalography, suggesting that cortical damage was identified frequently (P = 0.038, odds ratio = 5.7, 95% confidence interval 1.3 to 25.0). Visual grading revealed significant differences among both cortical (P < 0.001) and subcortical (P < 0.001) regions. On multivariate analysis, the odds for pan-hemispheric disease decreased per year of age at onset (P = 0.022, odds ratio 0.51, 95% confidence interval 0.085 to 0.725). Epilepsy surgery (n = 14) was associated with Engel Class 1 seizure control (P < 0.001). Immunosuppressive therapy (n = 20) did not demonstrate a significant seizure remission (P = 0.157, odds ratio 0.39, 95% confidence interval 0.10 to 1.55).

Conclusions

Our case series confirms the presence of specific topographical patterns of macroscopic radiological pathology over the affected hemisphere with a marked age-associated reduction in the odds for severe pan-hemispheric disease.

中文翻译：

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拉斯穆森脑炎严重程度和离散地形模式的年龄依赖性降低：与皮质成熟的联系？

背景

Rasmussen 脑炎的尸检研究揭示了受影响半球内的保留区域。临床进展和炎症较轻，发病年龄较大。我们试图证明这些模式的放射学证实。

方法

在我们的回顾性研究中，38 例病例被分为严重的泛半球（所有肺叶）和亚半球组（其他），以确定年龄人口统计学和其他严重程度的预测因素。通过结构化视觉分级评估皮层和皮层下结构中放射病理学的范围和模式。还报告了相关的临床数据。

结果

泛半球受累的儿童发病年龄更小（P  < 0.001），更可能出现癫痫持续状态（比值比 8.5，95% 置信区间 1.5 至 50.0，P  = 0.022）。围产期窒息/住院史 ( P  < 0.001) 和里程碑延迟 ( P  = 0.013) 仅在该组中出现，并且进展为脑电图低振幅记录背景，表明皮层损伤经常被发现 ( P  = 0.038 ，优势比 = 5.7，95% 置信区间 1.3 到 25.0）。视觉分级显示皮质（P  < 0.001）和皮质下（P < 0.001) 区域。在多变量分析中，泛半球疾病的几率在发病时每岁下降（P  = 0.022，优势比 0.51，95% 置信区间 0.085 至 0.725）。癫痫手术 (n = 14) 与 Engel 1 级癫痫控制相关 ( P  < 0.001)。免疫抑制治疗（n = 20）未表现出显着的癫痫缓解（P  = 0.157，优势比 0.39，95% 置信区间 0.10 至 1.55）。

结论

我们的病例系列证实了受累半球存在宏观放射病理学的特定地形模式，严重泛半球疾病的几率显着降低，与年龄相关。