Lindgren Erik, Shu Liqi, Simaan Naaem, Krzywicka Katarzyna, de Winter Maria A, Sánchez van Kammen Mayte, Molad Jeremy, Klein Piers, Hallevi Hen, Barnea Rani, Heldner Mirjam R, Hiltunen Sini, de Sousa Diana Aguiar, Ferro José M, Arauz Antonio, Putaala Jukka, Arnold Marcel, Nguyen Thanh N, Stretz Christoph, Tatlisumak Turgut, Jood Katarina, Yaghi Shadi, Leker Ronen R, Coutinho Jonathan M, Mansour Maryam, Canhão Patrícia, Ekizoglu Esme, Rodrigues Miguel, Silva Elisa M, Garcia-Esperon Carlos, Arnao Valentina, Aladin Shorooq, Mendel Rom, Aridon Paolo, Sezgin Mine, Alasheev Andrey, Smolkin Andrey, Guisado-Alonso Daniel, Yesilot Nilufer, Barboza Miguel A, Ghiasian Masoud, Silvis Suzanne M, Fang Ton, Siegler James E, Wu Teddy, Wilson Duncan, Asad Syed Daniyal, Al Kasab Sami, Almallouhi Eyad, Frontera Jennifer, Rothstein Aaron, Bakradze Ekaterina, Omran Setareh Salehi, Henninger Nils, Kuohn Lindsey, Zubair Adeel, Sharma Richa, Kerrigan Deborah, Aziz Yasmin, Mistry Eva, Zuurbier Susanna M
Department of Clinical Neuroscience, Institute of Neuroscience and Physiology, Sahlgrenska Academy at University of Gothenburg and Department of Neurology, Sahlgrenska University Hospital, Gothenburg, Sweden.
Department of Neurology, Brown University, Providence, Rhode Island.
JAMA Neurol. 2024 Dec 1;81(12):1274-1283. doi: 10.1001/jamaneurol.2024.3481.
One of 10 patients develop epilepsy in the late phase after cerebral venous thrombosis (CVT) diagnosis but predicting the individual risk is difficult.
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.
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.
Time to a first late seizure, defined as occurring more than 7 days after diagnosis of CVT.
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.
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.
10名脑静脉血栓形成（CVT）患者中有1人在诊断后的晚期发生癫痫，但预测个体风险具有挑战性。
开发并外部验证一种预后评分，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纳入了1994年至2022年登记的回顾性和前瞻性患者。为了开发DIAS3评分，使用了国际CVT联盟（n = 1128）的数据，这是一个大型的以医院为基础的国际多中心CVT队列。为了进行验证，使用了2个独立多中心队列的数据，即ACTION-CVT（n = 543）和以色列CVT研究（n = 556）。在2937名符合条件、连续入组的经影像学证实为CVT的成年患者中，排除了710名在CVT之前有癫痫病史、随访少于8天以及晚期癫痫发作状态缺失的患者。
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个易于获得的临床变量组成：减压性颅骨切除术、就诊时的脑出血、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以及就诊时的硬膜下血肿。
首次晚期癫痫发作的时间，定义为在CVT诊断后7天以上发生。
在推导队列中的1128名患者（中位年龄41岁[四分位间距，30 - 53岁]；805名女性[71%]）中，128名（11%）在中位随访12个月（四分位间距，3 - 26个月）期间发生了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%。内部和外部验证显示，推导队列（1年和3年：C统计量，0.74；95%置信区间，0.70 - 0.79）以及2个独立验证队列（ACTION-CVT，1年：C统计量，0.76；95%置信区间，0.67 - 0.84；3年：C统计量，0.77；95%置信区间，0.66 - 0.84；以及以色列CVT研究，1年：C统计量，0.80；95%置信区间，0.75 - 0.86）具有充分的区分度。校准图表明预测风险与观察风险之间具有充分的一致性。
DIAS3评分（可在网上免费获取）是一种简单工具，可帮助预测个体患者CVT后癫痫的风险。该模型可改善个性化医疗的机会，并可能有助于在抗癫痫药物治疗、患者咨询以及促进CVT癫痫发生机制研究方面的决策制定。
