1 Division of Neurovirology, Beth Israel Deaconess Medical Centre, Harvard Medical School, Boston, MA, USA.
Brain. 2013 Nov;136(Pt 11):3441-50. doi: 10.1093/brain/awt268. Epub 2013 Oct 1.
We sought to characterize perfusion patterns of progressive multifocal leukoencephalopathy lesions by arterial spin labelling perfusion magnetic resonance imaging and to analyse their association with immune reconstitution inflammatory syndrome, and survival. A total of 22 patients with progressive multifocal leukoencephalopathy underwent a clinical evaluation and magnetic resonance imaging of the brain within 190 days of symptom onset. The presence of immune reconstitution inflammatory syndrome was determined based on clinical and laboratory criteria. Perfusion within progressive multifocal leukoencephalopathy lesions was determined by arterial spin labelling magnetic resonance imaging. We observed intense hyperperfusion within and at the edge of progressive multifocal leukoencephalopathy lesions in a subset of subjects. This hyperperfusion was quantified by measuring the fraction of lesion volume showing perfusion in excess of twice normal appearing grey matter. Hyperperfused lesion fraction was significantly greater in progressive multifocal leukoencephalopathy progressors than in survivors (12.8% versus 3.4% P = 0.02) corresponding to a relative risk of progression for individuals with a hyperperfused lesion fraction ≥ 4.0% of 9.1 (95% confidence interval of 1.4-59.5). The presence of hyperperfusion was inversely related to the occurrence of immune reconstitution inflammatory syndrome at the time of scan (P = 0.03). Indeed, within 3 months after symptom onset, hyperperfusion had a positive predictive value of 88% for absence of immune reconstitution inflammatory syndrome. Arterial spin labelling magnetic resonance imaging recognized regions of elevated perfusion within lesions of progressive multifocal leukoencephalopathy. These regions might represent virologically active areas operating in the absence of an effective adaptive immune response and correspond with a worse prognosis.
我们旨在通过动脉自旋标记灌注磁共振成像来描述进行性多灶性白质脑病病变的灌注模式，并分析其与免疫重建炎症综合征和生存的关系。共有 22 名进行性多灶性白质脑病患者在症状出现后 190 天内接受了临床评估和脑部磁共振成像检查。根据临床和实验室标准确定免疫重建炎症综合征的存在。通过动脉自旋标记磁共振成像确定进行性多灶性白质脑病病变内的灌注。我们观察到在一部分患者中，进行性多灶性白质脑病病变内和边缘存在强烈的高灌注。通过测量显示灌注超过正常外观灰质两倍的病变体积分数来量化这种高灌注。进行性多灶性白质脑病进展者的高灌注病变比例明显高于幸存者（12.8%比 3.4%，P=0.02），这对应于高灌注病变比例≥4.0%的个体进展的相对风险为 9.1（95%置信区间为 1.4-59.5）。高灌注的存在与扫描时免疫重建炎症综合征的发生呈负相关（P=0.03）。事实上，在症状出现后 3 个月内，高灌注对无免疫重建炎症综合征的阳性预测值为 88%。动脉自旋标记磁共振成像识别出进行性多灶性白质脑病病变内灌注升高的区域。这些区域可能代表在缺乏有效适应性免疫反应的情况下具有病毒活性的区域，与预后较差相对应。
