Abstract：
BACKGROUND: Primary central nervous system lymphoma (PCNSL) accounts for less than 5% of primary brain tumors. Epileptic seizures are a common manifestation of brain tumors; however, literature on the prevalence, characteristics, and oncological implications of seizures in patients with PCNSL is limited, and the management of antiepileptic drugs (AEDs) is unclear. This review aimed to summarize the existing knowledge on seizures in PCNSL, their potential association with surgery, oncological treatment, survival rates, and management of AEDs.
METHODS: A systematic review was performed according to the PRISMA recommendations and included articles published between 1953 and 2023 describing seizures in patients with PCNSL.
RESULTS: The search identified 282 studies, of which 21 were included. Up to 33% of patients with PCNSL developed seizures, mostly at the initial presentation. Little information was found on changes in seizure incidence through the course of the disease, and no details were found on seizure frequency, the percentage of treatment-resistant patients, or the evolution of seizures at remission. Younger age, cortical location, and immunodeficiency have been identified as potential risk factors for seizures, but evidence is very limited. The growing use of vigorous treatments including intensive chemotherapy with autologous stem cell transplantation and immunotherapy with CAR-T cells is associated with a higher incidence of seizures. The association between seizure development and patient mortality in PCNSL remains unknown. There are no data on AED prophylaxis or the use of specific AEDs in PCNSL.
CONCLUSIONS: Further studies are needed to investigate seizures in larger cohorts of PCNSL, to clarify their prevalence, better characterize them, identify risk factors, analyze survival rates, and make recommendations on AED management. We recommend following general practice guidelines for seizures symptomatic of brain tumors and not to prescribe AED prophylaxis in PCNSL.
METHODS: A systematic review was performed according to the PRISMA recommendations and included articles published between 1953 and 2023 describing seizures in patients with PCNSL.
RESULTS: The search identified 282 studies, of which 21 were included. Up to 33% of patients with PCNSL developed seizures, mostly at the initial presentation. Little information was found on changes in seizure incidence through the course of the disease, and no details were found on seizure frequency, the percentage of treatment-resistant patients, or the evolution of seizures at remission. Younger age, cortical location, and immunodeficiency have been identified as potential risk factors for seizures, but evidence is very limited. The growing use of vigorous treatments including intensive chemotherapy with autologous stem cell transplantation and immunotherapy with CAR-T cells is associated with a higher incidence of seizures. The association between seizure development and patient mortality in PCNSL remains unknown. There are no data on AED prophylaxis or the use of specific AEDs in PCNSL.
CONCLUSIONS: Further studies are needed to investigate seizures in larger cohorts of PCNSL, to clarify their prevalence, better characterize them, identify risk factors, analyze survival rates, and make recommendations on AED management. We recommend following general practice guidelines for seizures symptomatic of brain tumors and not to prescribe AED prophylaxis in PCNSL.
摘要：
背景：原发性中枢神经系统淋巴瘤（PCNSL）占原发性脑肿瘤的不到5％。癫痫发作是脑肿瘤的常见表现；然而，关于患病率的文献，特点,PCNSL患者癫痫发作的肿瘤学意义有限，抗癫痫药物（AEDs）的管理尚不清楚。这篇综述旨在总结PCNSL癫痫发作的现有知识，他们与手术的潜在联系，肿瘤治疗,存活率,和AED的管理。
方法：根据PRISMA建议进行了系统评价，包括1953年至2023年之间发表的描述PCNSL患者癫痫发作的文章。
结果：搜索确定了282项研究，其中包括21个。高达33%的PCNSL患者出现癫痫发作,主要是在最初的演讲中。几乎没有发现关于整个疾病过程中癫痫发作发生率变化的信息，没有发现癫痫发作频率的细节，耐药患者的百分比，或缓解时癫痫发作的演变。年龄更小,皮质位置,免疫缺陷已被确定为癫痫发作的潜在危险因素，但是证据非常有限。越来越多地使用强力治疗，包括自体干细胞移植的强化化疗和CAR-T细胞的免疫疗法与癫痫发作的发生率更高相关。PCNSL中癫痫发作发展与患者死亡率之间的关系仍然未知。没有关于AED预防或在PCNSL中使用特定AED的数据。
结论：需要进一步的研究来调查更大的PCNSL队列中的癫痫发作，为了澄清它们的患病率，更好地描述它们，识别风险因素，分析生存率，并对AED管理提出建议。我们建议遵循一般实践指南，以治疗有症状的脑肿瘤癫痫发作，并且不要在PCNSL中规定AED预防。
方法：根据PRISMA建议进行了系统评价，包括1953年至2023年之间发表的描述PCNSL患者癫痫发作的文章。
结果：搜索确定了282项研究，其中包括21个。高达33%的PCNSL患者出现癫痫发作,主要是在最初的演讲中。几乎没有发现关于整个疾病过程中癫痫发作发生率变化的信息，没有发现癫痫发作频率的细节，耐药患者的百分比，或缓解时癫痫发作的演变。年龄更小,皮质位置,免疫缺陷已被确定为癫痫发作的潜在危险因素，但是证据非常有限。越来越多地使用强力治疗，包括自体干细胞移植的强化化疗和CAR-T细胞的免疫疗法与癫痫发作的发生率更高相关。PCNSL中癫痫发作发展与患者死亡率之间的关系仍然未知。没有关于AED预防或在PCNSL中使用特定AED的数据。
结论：需要进一步的研究来调查更大的PCNSL队列中的癫痫发作，为了澄清它们的患病率，更好地描述它们，识别风险因素，分析生存率，并对AED管理提出建议。我们建议遵循一般实践指南，以治疗有症状的脑肿瘤癫痫发作，并且不要在PCNSL中规定AED预防。
