Importance One of 10 patients develop epilepsy in the late phase after cerebral venous thrombosis (CVT) diagnosis but predicting the individual risk is difficult. Objective To develop and externally validate a prognostic score to estimate the individual risk of post-CVT epilepsy. Design, Setting, and Participants This observational cohort study included both retrospective and prospective patients enrolled from 1994 through 2022. For development of the DIAS3 score, data from the International CVT Consortium (n = 1128), a large international hospital-based multicenter CVT cohort, were used. For validation, data from 2 independent multicenter cohorts, the ACTION-CVT (n = 543) and the Israel CVT study (n = 556), were used. Of 2937 eligible, consecutively enrolled adult patients with radiologically verified CVT, 710 patients with a history of epilepsy prior to CVT, follow-up less than 8 days, and missing late seizure status were excluded. Exposure The prediction score (DIAS3) was developed based on available literature and clinical plausibility and consisted of 6 readily available clinical variables collected during the acute phase: decompressive hemicraniectomy, intracerebral hemorrhage at presentation, age, seizure(s) in the acute phase (excluding status epilepticus), status epilepticus in the acute phase, and subdural hematoma at presentation. Main Outcome and Measure Time to a first late seizure, defined as occurring more than 7 days after diagnosis of CVT. Results Of 1128 patients included in the derivation cohort (median age, 41 [IQR, 30-53] years; 805 women [71%]), 128 (11%) developed post-CVT epilepsy during a median follow-up of 12 (IQR, 3-26) months. According to the DIAS3 score, the predicted 1-year and 3-year risk of epilepsy in individual patients ranged from 7% to 68% and 10% to 83%, respectively. Internal and external validation showed adequate discrimination in the derivation cohort (1 year and 3 years: C statistic, 0.74; 95% CI, 0.70-0.79) and the 2 independent validation cohorts, (ACTION-CVT) 1 year: C statistic, 0.76; 95% CI, 0.67-0.84; 3 years: C statistic, 0.77; 95% CI, 0.66-0.84; and Israel CVT study 1 year: C statistic, 0.80; 95% CI, 0.75-0.86. Calibration plots indicated adequate agreement between predicted and observed risks. Conclusions and Relevance The DIAS3 score (freely available online) is a simple tool that can help predict the risk of post-CVT epilepsy in individual patients. The model can improve opportunities for personalized medicine and may aid in decision-making regarding antiseizure medication, patient counseling, and facilitation of research on epileptogenesis in CVT.

中文翻译：

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开发和验证预测脑静脉血栓形成后癫痫的临床评分。


重要性 10 名患者中有 1 名在脑静脉血栓形成 （CVT） 诊断后的晚期发展为癫痫，但很难预测个体风险。目的 开发并外部验证预后评分，以估计 CVT 后癫痫的个体风险。设计、设置和参与者 这项观察性队列研究包括 1994 年至 2022 年入组的回顾性和前瞻性患者。为了开发 DIAS3 评分，使用了来自国际 CVT 联盟 （n = 1128） 的数据，这是一个基于大型国际医院的多中心 CVT 队列。为了进行验证，使用了来自 2 个独立的多中心队列的数据，即 ACTION-CVT （n = 543） 和以色列 CVT 研究 （n = 556）。在 2937 名符合条件的连续入组经放射学验证的 CVT 成年患者中，排除了 710 名在 CVT 之前有癫痫病史、随访少于 8 天和漏诊晚期癫痫发作状态的患者。暴露 预测评分 （DIAS3） 是根据现有文献和临床合理性开发的，由急性期收集的 6 个现成的临床变量组成：减压性偏侧颅骨切除术、就诊时脑出血、年龄、急性期癫痫发作（不包括癫痫持续状态）、急性期癫痫持续状态和就诊时硬膜下血肿。主要结果和测量 首次晚期癫痫发作的时间，定义为在诊断为 CVT 后 7 天以上发生。结果 在纳入衍生队列的 1128 名患者 （中位年龄，41 [IQR，30-53] 岁;805 名女性 [71%]）中，128 名 （11%） 在中位随访 12 （IQR，3-26） 个月期间发生 CVT 后癫痫。 根据 DIAS3 评分，个体患者预测的 1 年和 3 年癫痫风险分别为 7% 至 68% 和 10% 至 83%。内部和外部验证在派生队列 （1 年和 3 年： C 统计量，0.74;95% CI，0.70-0.79） 和 2 个独立验证队列 （ACTION-CVT） 1 年中显示出足够的区分度： C 统计量，0.76;95% CI，0.67-0.84;3 年：C 统计量，0.77;95% CI，0.66-0.84;和以色列 CVT 研究 1 年：C 统计量，0.80;95% CI，0.75-0.86。校准图表明预测风险和观察到的风险之间具有足够的一致性。结论和相关性 DIAS3 评分（在线免费提供）是一种简单的工具，可以帮助预测个体患者发生 CVT 后癫痫的风险。该模型可以增加个性化医疗的机会，并可能有助于有关抗癫痫药物的决策、患者咨询和促进 CVT 癫痫发生研究。