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Neurology:局灶性皮质发育不良IIB的黑线征:7T MRI和电临床病理研究

2022-06-30 Naomi MedSci原创

局灶性皮质发育不良(FCD)是内科难治性局灶性癫痫患者手术切除后最常见的结构损害之一。研究表明,在接受手术的顽固性局灶性癫痫患者中,足够图像质量的7T磁共振成像中,黑线征或成为FCD ⅡB生物标志物。

局灶性皮质发育不良(FCD)是内科难治性局灶性癫痫患者手术切除后最常见的结构损害之一。近年来,随着术前评估的长足进步,FCD患者的癫痫发作结果有了显著的改善,尤其是FCD IIB,术后2-4个月达到了80%的癫痫无发作。完全切除FCD病变是有利癫痫发作结果的主要决定因素,这突显了优化病变识别和描绘的重要性,特别是使用非侵入性MRI技术。1.5T/3TMRI在癫痫成像方案和后处理方法方面的进展已显著提高了对FCD6-12的识别;然而,区分亚型和揭示病灶内细微细节的能力仍然不佳。

近日,一项发表在Neurology上的研究试图提供有关黑线征的详细的成像-电-临床-病理特征,这是一种显示局灶性皮质发育不良(FCD)IIB的新MRI标志物。

回顾一组经病理证实的顽固性癫痫患者的7TT2*加权梯度回波(T2*w-GRE)图像,观察黑线征的出现情况。检查了黑线区域和由颅内脑电(ICEEG)定义的癫痫起始区(SOZ)之间的重叠,并另外评估了手术切除中完全包括黑线区域是否与术后癫痫缓解有关。组织病理标本与MRI对准,观察黑线征的病理基础。在黑线区域、整个病变灰质(GM)以及对侧/同侧正常灰质和白质(WM)进行基于感兴趣区的7TT1图的qMRI分析。

  • 研究共纳入20例FCD II患者(14例IIB和6例IIA)。
  • 在7TT2*w-GRE上,FCD IIB组12/14(85.7%)、FCD IIA组0/6可见黑线征。黑线区域与ICEEG定义的SOZ高度一致(5/7完全重叠,2/7部分重叠)。
  • 8例手术切除完全包括黑线区的患者中有8例发作缓解,2例切除不完全包括黑线区的患者均有反复发作。
  • 手术切除的黑线区域与无癫痫发作显著相关(p=0.02)。
  • QMRI分析显示黑线区域与WM有显著差异(p<0.001),但与GM相似。
  • 1例匹配良好的组织病理切片显示黑线区域聚集的畸形神经元和气球细胞。

黑线征可作为FCD IIB的无创生物标志物。MRI-病理学和qMRI分析都表明黑线区域是FCD内的异常GM成分。黑线征与ICEEG定义的SOZ高度一致,并与切除时的癫痫发作自由显著相关,黑线征可能有助于FCD IIB难治性癫痫患者的ICEEG/手术计划。

这项研究提供了II类证据,表明在接受手术的顽固性局灶性癫痫患者中,如果有足够图像质量的7T磁共振成像,黑线征的出现可能提示FCD IIB,与ICEEG的SOZ一致,如果完全包括在切除中,则与更多的癫痫发作自由相关。 

文献来源:Tang Y, Blumcke I, Su TY, et al. Black Line Sign in Focal Cortical Dysplasia IIB: A 7T MRI and Electro-clinico-pathologic Study [published online ahead of print, 2022 May 16]. Neurology. 2022;10.1212/WNL.0000000000200702. doi:10.1212/WNL.0000000000200702

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    2022-12-25 yinhl1978
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    2022-06-30 neurowu
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