Department of Pediatrics, Chonbuk National University Medical School, Jeonju, Korea.
Pediatr Neurol. 2010 Mar;42(3):172-6. doi: 10.1016/j.pediatrneurol.2009.10.011.
The aim of this study was to investigate the correlation between magnetic resonance imaging (MRI) and histopathologic findings in Rasmussen syndrome. Serial MRIs were obtained for five patients who had histologically proven Rasmussen syndrome. The histopathologic grades of the lesions were subdivided into phases: active 1-3, resolving 1-3, and chronic inflammatory. The images were then correlated with histopathologic findings. Neuropathologic findings in the central areas on MRI demonstrated only the chronic and resolving grades, but active inflammatory abnormalities were present not only at the margins of the lesions, but also in areas of subtle signal abnormality on MRI. Atrophic areas on MRI exhibited all grades of histopathologic abnormalities, but chronic and resolving grades were predominant. Seizure duration of less than 6 months was associated with very active grades, duration of 1-2 years with variable grades, and duration greater than 6 years with chronic and resolving grades only. The MRI images correlated highly with histopathologic analysis. These findings suggest that the lesions initially arise from one site in the brain, and so support the centrifugal spreading theory of this disease. Findings also suggest that the margin rather than the center of the MRI abnormality may be the most ideal site for biopsy.
本研究旨在探讨磁共振成像(MRI)与拉森综合征的组织病理学发现之间的相关性。对 5 名经组织学证实患有拉森综合征的患者进行了连续 MRI 检查。将病变的组织病理学分级分为活动期 1-3 级、缓解期 1-3 级和慢性炎症期。然后将图像与组织病理学发现相关联。MRI 上中央区域的神经病理学发现仅显示慢性和缓解期,但不仅在病变边缘,而且在 MRI 上的轻微信号异常区域也存在活动期炎症异常。MRI 上的萎缩区域表现出所有组织病理学异常的分级,但慢性和缓解期更为常见。癫痫持续时间小于 6 个月与非常活跃的分级相关,持续时间为 1-2 年与可变分级相关,持续时间大于 6 年仅与慢性和缓解期相关。MRI 图像与组织病理学分析高度相关。这些发现表明病变最初起源于大脑的一个部位,因此支持该疾病的离心扩散理论。研究结果还表明,MRI 异常的边缘而不是中心可能是活检的最佳部位。