van Niftrik Christiaan Hendrik Bas, Sebök Martina, Nicholson Patrick, Olijnyk Leonardo, Thurner Patrick, Venkatraghavan Lashmi, Schaafsma Joanna, Radovanovic Ivan, Fisher Joseph A, Krings Timo, Kulcsár Zsolt, Tymianski Michael, Regli Luca, Mikulis David J, Fierstra Jorn
Department of Neurosurgery, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
Clinical Neuroscience Center, University Hospital Zurich, Zurich, Switzerland.
Quant Imaging Med Surg. 2023 Jul 1;13(7):4618-4632. doi: 10.21037/qims-22-1062. Epub 2023 Jun 15.
Prior Infarcts, Reactivity, and Angiography in Moyamoya Disease (PIRAMD) is a recently proposed imaging-based scoring system that incorporates the severity of disease and its impact on parenchymal hemodynamics in order to better support clinical management and evaluate response to intervention. In particular, PIRAMD may have merit in identifying symptomatic patients that may benefit most from revascularization. Our aim was to validate the PIRAMD scoring system.
Patients with ischemic Moyamoya disease, who underwent catheter angiographic [modified Suzuki Score (mSS) and collateralization status], morphological MRI and a parenchymal hemodynamic evaluation with blood oxygenation-level dependent cerebrovascular reactivity (BOLD-CVR) at two transatlantic centers, were retrospectively included. The primary outcome was the presence of neurological symptoms. The diagnostic capacity of each PIRAMD feature alone was evaluated, as well as combined and the inter-institutional differences of each parameter were evaluated.
Seventy-two hemispheres of 38 patients were considered for analysis, of which 39 (54%) were classified as symptomatic. The presence of a prior infarct had the highest odds ratio [odds ratio (OR) =24; 95% CI: 6.7-87.2] for having neurological symptoms, followed by impaired CVR (OR =17; 95% CI: 5-62). No inter-institutional differences in the odds ratios or area under the curve (AUC) were found for any study parameter. The PIRAMD score had an AUC of 0.88 (95% CI: 0.80-0.96) with a similar AUC for the PIRAMD grading score.
Our multicentric validation of the recently published PIRAMD scoring system was highly effective in rating the severity of ischemic Moyamoya disease with excellent inter-institutional agreement. Future studies should investigate the prognostic value of this novel imaging-based score in symptomatic patients with Moyamoya disease.
烟雾病的既往梗死、反应性和血管造影（PIRAMD）是最近提出的一种基于影像的评分系统，该系统纳入了疾病的严重程度及其对实质血流动力学的影响，以便更好地支持临床管理并评估干预反应。特别是，PIRAMD在识别可能从血运重建中获益最大的有症状患者方面可能具有价值。我们的目的是验证PIRAMD评分系统。
回顾性纳入在两个跨大西洋中心接受导管血管造影[改良铃木评分（mSS）和侧支循环状态]、形态学MRI以及采用血氧水平依赖脑血管反应性（BOLD-CVR）进行实质血流动力学评估的缺血性烟雾病患者。主要结局是神经症状的存在。评估了每个PIRAMD特征单独的诊断能力，以及组合后的诊断能力，并评估了每个参数的机构间差异。
对38例患者的72个半球进行了分析，其中39个（54%）被归类为有症状。既往梗死的存在对于出现神经症状的比值比最高[比值比（OR）=24；95%置信区间：6.7-87.2]，其次是CVR受损（OR =17；9%置信区间：5-62）。对于任何研究参数，未发现机构间在比值比或曲线下面积（AUC）方面存在差异。PIRAMD评分的AUC为0.88（95%置信区间：0.80-0.96），PIRAMD分级评分的AUC与之相似。
我们对最近发表的PIRAMD评分系统进行的多中心验证在评估缺血性烟雾病的严重程度方面非常有效，机构间一致性良好。未来的研究应调查这种基于影像的新评分在烟雾病有症状患者中的预后价值。
