Abstract
Background: Studies exploring the predictive performance of major risk factors associated with future stroke events are insufficient, and a useful tool to predict individual risk is not available. Therefore, personalized advice for preventing future stroke in patients with moyamoya disease (MMD) cannot provide evidence-based recommendations. The aim of this study was to develop a novel nomogram with reliable validity to predict the individual risk of future stroke for adult MMD patients.Methods: This study included 450 patients from seven medical centers between January 2013 and December 2018. Follow-ups were performed via clinical visits and/or telephone interviews from initial discharge to December 2019. The cohort was randomly assigned to a training set (2/3, n = 300) for nomogram development and a test set (1/3, n = 150) for external validation. The Kaplan-Meier analyses and receiver operating characteristic (ROC) curves were applied to assess the clinical benefits of this nomogram.Results: Diabetes mellitus, a family history of MMD, a past history of stroke or transient ischemic attack, clinical manifestation, and treatment were identified as major risk factors via the least absolute shrinkage and selection operator (LASSO) method. A nomogram including these predictors was established via a multivariate Cox regression model, which displayed excellent discrimination [Harrell's concordance index (C-index), 0.85; 95% confidence interval (CI): 0.75–0.96] and calibration. In the external validation, the nomogram was found to have good discrimination (C-index, 0.81; 95% CI: 0.68–0.94) and calibration. In the subgroup analysis, this predictive nomogram also showed great performance in both ischemic-type (C-index, 0.90; 95% CI: 0.77–1.00) and hemorrhagic-type MMD (C-index, 0.72; 95% CI: 0.61–0.83). Furthermore, the nomogram was shown to have potential in clinical practice through Kaplan-Meier analyses and ROC curves.Conclusions: We developed a novel nomogram incorporating several clinical characteristics with relatively good accuracy, which may have considerable potential for evaluating individual future stroke risk and providing useful management recommendations for adult patients with MMD in clinical practice.
Highlights
Moyamoya disease (MMD) is a rare chronic cerebrovascular disease characterized by progressive stenosis and/or occlusion of the distal internal carotid artery (ICA) and its major branches with the development of abnormal collateral circulation at the base of the brain [1]
The inclusion criteria were as follows: [1] a diagnosis of moyamoya disease (MMD) based on the 2012 Tokyo criteria [6] [definitive MMD was defined as the bilateral stenosis and/or occlusion of the terminal portion of ICAs and/or the proximal portion of the anterior cerebral artery (ACA) and/or the middle cerebral artery (MCA) with the abnormal vascular network via cerebral digital subtraction angiography (DSA) and/or magnetic resonance angiography (MRA); probable MMD was defined as the unilateral involvement with the abnormal vascular network near the lesion via DSA while lacking predisposing factors for steno-occlusive changes]; [2] age 18 years or older; [3] no history of prior use of antiplatelet agents; [4] no history of prior neurosurgery; and [5] complete data for all predictors of interest
We found that a family history of MMD, a past history of stroke or transient ischemic attack (TIA) and treatment choice were remarkably different between both groups in the training and test sets, suggesting that these clinical features could be the potential risk factors for MMD with future strokes
Summary
Moyamoya disease (MMD) is a rare chronic cerebrovascular disease characterized by progressive stenosis and/or occlusion of the distal internal carotid artery (ICA) and its major branches with the development of abnormal collateral circulation at the base of the brain [1]. MMD is an important cause of stroke and is associated with a relatively high recurrence risk of cerebrovascular events and a poor prognosis [5, 6]. Several independent risk factors, including an initial symptom of intraventricular hemorrhage, smoking, Asian ethnicity, a history of transient ischemic attack (TIA), and a reduced hemodynamic reserve, have been reported to significantly contribute to recurrent stroke in MMD [7,8,9]. A single risk factor might be insufficient to predict the individual probability of cerebrovascular events in the future. Studies exploring the predictive performance of major risk factors associated with future stroke events are insufficient, and a useful tool to predict individual risk is not available. The aim of this study was to develop a novel nomogram with reliable validity to predict the individual risk of future stroke for adult MMD patients
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