National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, Shizuoka, Japan.
Epileptic Disord. 2011 Sep;13(3):229-39. doi: 10.1684/epd.2011.0464.
MRI data is essential for early diagnosis and evaluation of surgical indication in patients with Rasmussen syndrome (RS). In the present study, we examined the status and evolutionary changes in MRI lesions to identify the MRI characteristics of RS.
MRI of 15 RS patients was examined regarding frequency and distribution of atrophic lesions on T1-weighted images and high intensity lesions on FLAIR or T2-weighted images.
In 13 patients, atrophic lesions were observed predominantly in the frontal lobes with various extent of involvement. High intensity lesions were also observed in 13 patients. High intensity lesions were significantly more prevalent in the cortex of patients with later onset and were present in the insula in 37.5% of epilepsia partialis continua (EPC) type patients and in 57.1% of non-EPC type. Early MRI showed various combinations of atrophic lesions or high intensity lesions in seven of nine patients who underwent MRI examinations within one year of their first seizure. Serial MRI revealed high intensity lesions with characteristic features of regression (20.0% of patients), fluctuation (regression followed by reappearance; 33.3%) and expansion (46.7%). Appearance and reappearance of high intensity lesions in the cortex and/or subcortical white matter were associated with aggravation of seizures. Bilateral high intensity lesions were observed in three patients with unilateral epileptogenic foci, who were successfully treated by surgical intervention.
Dynamic evolutionary changes in lesions (regression, fluctuation and expansion of high intensity lesions), as observed on MRI, may be a diagnostic feature of Rasmussen syndrome.
磁共振成像(MRI)数据对于拉森综合征(RS)患者的早期诊断和手术适应证评估至关重要。本研究旨在检查 MRI 病变的状态和演变变化,以确定 RS 的 MRI 特征。
对 15 例 RS 患者的 MRI 进行检查,评估 T1 加权图像上的萎缩性病变和 FLAIR 或 T2 加权图像上的高信号病变的频率和分布。
在 13 例患者中,萎缩性病变主要位于额叶,且病变程度不一。13 例患者还存在高信号病变。发病较晚的患者皮质高信号病变更为常见,37.5%的部分性癫痫持续状态(EPC)患者和 57.1%的非-EPC 患者岛叶存在高信号病变。在首次发作后一年内接受 MRI 检查的 9 例患者中,7 例患者早期 MRI 显示出多种萎缩性病变或高信号病变的组合。连续 MRI 显示具有消退特征的高信号病变(20.0%的患者)、波动(消退后再出现;33.3%)和扩张(46.7%)。皮质和/或皮质下白质中高信号病变的出现和再现与癫痫发作加重有关。3 例单侧致痫灶患者存在双侧高信号病变,经手术干预后成功治疗。
MRI 上观察到的病变(高信号病变的消退、波动和扩张)的动态演变变化可能是拉森综合征的诊断特征。