Primary angiitis of the central nervous system: serial MRI of brain and spinal cord
Primary angiitis of the central nervous system: serial MRI of brain and spinal cord
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DOI：
10.1007/s002340100561
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发表时间：
2001-08-01
期刊：
影响因子：
2.8
通讯作者：
Terreni, MR
中科院分区：
文献类型：
--
作者：
Campi, A;Benndorf, G;Terreni, MR
原发性中枢神经系统血管炎（PACNS）的MRI表现是高度可变的，从正常到弥漫性异常。我们描述了PACNS患者的脑和脊髓异常以及随时间的变化，以提供有助于鉴别诊断的标准。我们回顾了6例最终诊断为PACNS的患者，他们接受了系列对比增强的脑部和脊柱MRI。随访12 ~ 60个月。所有患者的脑部MRI均显示多个小异常，T2加权图像上显示高信号，局灶性或弥漫性，主要位于深部和皮质下白色物质; 4例患者同时存在幕上和幕下病变。在最初的MRI上，5名患者中，几乎90%的异常病灶显示出对比增强。Virchow-Robin血管周围间隙扩大，同时患者还出现脊髓异常，其中两例发生在颈段和胸段，一例仅发生在颈段。两名患者有脑活检证实的PACNS;在其余患者中，考虑到临床和MRI特征以及MRI随访的相似性，PACNS的诊断是推定的。在MRI上，类固醇和免疫抑制剂治疗后，观察到异常的数量和大小显著减少，增强和非增强以及增强的血管周围间隙。因此，在疾病开始时同时增强脑和脊髓病变以及血管周围空间，并在随访期间消退，可以提示PACNS。
MRI findings in primary angiitis of the central nervous system (PACNS) are highly variable, ranging from normal to diffusely abnormal. We describe brain and spinal cord abnormalities in patients with PACNS and changes over time, to provide criteria which could be useful for differential diagnosis. We reviewed six patients, with a final diagnosis of PACNS, who underwent serial contrast-enhanced brain and spinal MRI. Follow-up ranged from 12 to 60 months. Brain MRI showed multiple small abnormalities in all patients, giving high signal on T2-weighted images, focal or diffuse, mainly in deep and subcortical white matter; four patients had both supra- and infratentorial lesions. On the initial MRI, in five patients, almost 90 % of the abnormal foci showed contrast enhancement. Virchow-Robin perivascular spaces were enlarged and simultaneously patients also had spinal cord abnormalities, in the cervical and thoracic segments in two, and exclusively cervical segment in one. Two patients had brain biopsy-proven PACNS; in the remainder, the diagnosis of PACNS was presumptive, considering similarities in clinical and MRI features and MRI follow-up. On MRI, after steroid and immunosuppressive therapy, a significant decrease in the number and size of the abnormalities, enhancing and nonenhancing and of enhancing perivascular spaces was observed. Simultaneous enhancement of brain and spinal cord lesions and of perivascular spaces, at the onset of the disease, which resolves during follow-up, can therefore suggest PACNS.