MRI in Late-Onset Rasmussen Encephalitis: A Long-Term Follow-Up Study.
MRI in Late-Onset Rasmussen Encephalitis: A Long-Term Follow-Up Study.
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DOI：
10.3390/diagnostics12020502
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发表时间：
2022-02-15
期刊：
影响因子：
--
通讯作者：
Villani F
中科院分区：
文献类型：
--
作者：
Doniselli FM;Deleo F;Criscuolo S;Stabile A;Pastori C;Di Giacomo R;Didato G;Chiapparini L;Villani F
迟发性拉斯穆森脑炎（LoRE）是一种罕见的单侧半球进行性炎症性疾病，可引起神经功能缺损和癫痫。长期的放射性演变从未得到充分描述。我们回顾性分析了136项研究中13例LoRE患者的MR图像，并在灰质或白色物质中寻找体积损失或信号强度异常的局灶性区域。每例受试者接受了9项MRI研究（中位数为IQR 7-13）。额叶和颞叶是受影响最严重的区域（分别为13/13和8/13），随着时间的推移，萎缩性变化的恶化程度最大（分别为9/13和5/8）。岛叶和顶叶皮质轻度萎缩。尾状核受累7例。T2-WI和FLAIR图像上所有患者均可见灰质和白色物质的高信号，8例为一过性高信号。在后者患者中的两例中，这些短暂的改变演变为同一区域的萎缩。疾病持续时间与最后随访时灰质信号异常显著相关。LoRE MRI改变较轻，与儿童期表格中描述的放射学结果相比，其进展明显较慢。
Late-onset Rasmussen encephalitis (LoRE) is a rare unihemispheric progressive inflammatory disorder causing neurological deficits and epilepsy. The long-term radiological evolution has never been fully described. We retrospectively analyzed the MR images of 13 LoRE patients from a total of 136 studies, and searched for focal areas of volume loss or signal intensity abnormality in grey matter or white matter. Each subject had a median of nine MRI studies (IQR 7–13). Frontal and temporal lobes were the most affected regions (13/13 and 8/13, respectively) and showed the greatest worsening over time in terms of atrophic changes (9/13 and 5/8, respectively). A milder cortical atrophy was found in the insular and parietal lobes. The caudate nucleus was affected in seven patients. Hyperintensities of grey matter and white matter on T2-WI and FLAIR images were observed in all patients, and transiently in eight patients. In two cases out of the latter patients, these transient alterations evolved into atrophy of the same region. Disease duration was significantly associated with signal abnormalities in the grey matter at last follow-up. LoRE MRI alterations are milder, and their progression is markedly slower compared to radiological findings described in the childhood form.
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影响因子：
9.9
作者：
Villani, F;Spreafico, R;Avanzini, G
通讯作者：
Avanzini, G
影响因子：
9.9
作者：
RASMUSSEN, T;OLSZEWSKI, J;LLOYDSMITH, D
通讯作者：
LLOYDSMITH, D
影响因子：
14.5
作者：
Schwab, Nicholas;Bien, Christian G.;Wiendl, Heinz
通讯作者：
Wiendl, Heinz
影响因子：
9.9
作者：
Larionov, S;Urbach, H;Bien, CG
通讯作者：
Bien, CG
影响因子：
2.2
作者：
Marras, Carlo Efisio;Granata, Tiziana;Broggi, Giovanni
通讯作者：
Broggi, Giovanni