From the Departments of Neurosurgery (O.A., A.C., B.M.), Neuro-Oncology (C.H., A.A., K.H.-X.), Hematology (S.C.), Epileptology (S.D.), Neuropathology (K.M.), Neuroradiology (D.L., L.N.), Nuclear Medicine (A.K., L.R.), and Biological Hematology (M.L.G.-T.), the Paris Brain Institute (ICM) (O.A., A.A., B.M.), LIB, INSERM U1146 (A.K., L.R.), INSERM U1127 (B.M.), CNRS UMR 7225 (B.M.), and GRC 23, Brain Machine Interface (A.C), La Pitié-Salpêtrière Hospital, Sorbonne University, Paris, France.
Neurology. 2024 Sep 10;103(5):e209748. doi: 10.1212/WNL.0000000000209748. Epub 2024 Aug 8.
Epilepsy is a common comorbidity of brain tumors; however, little is known about the prevalence, onset time, semiology, and risk factors of seizures in primary CNS lymphoma (PCNSL). Our objectives were to determine the prevalence of epilepsy in PCNSL, to identify factors associated with epilepsy, and to investigate the prognostic significance of seizures in PCNSL.
We performed an observational, retrospective single-center study at a tertiary neuro-oncology center (2011-2023) including immunocompetent patients with PCNSL and no history of seizures. We collected clinical, imaging, and treatment data; seizure status over the course of PCNSL; and oncological and seizure outcome. The primary outcome was to determine the prevalence of epilepsy. Furthermore, we aimed to identify clinical, radiologic, and treatment-related factors associated with epilepsy. Univariate analyses were conducted using the χ test for categorical variables and unpaired test for continuous variables. Predictors identified in the unadjusted analysis were included in backward stepwise logistic regression models.
We included 330 patients, 157 (47.6%) were male, median age at diagnosis was 68 years, and the median Karnofsky Performance Status score was 60. Eighty-three (25.2%) patients had at least 1 seizure from initial diagnosis to the last follow-up, 40 (12.1%) as the onset symptom, 16 (4.8%) during first line of treatment, 27 (8.2%) at tumor progression and 6 (1.8%) while in remission. Focal aware seizures were the most frequent seizure type, occurring in 43 (51.8%) patients. Seizure freedom under antiseizure medication was observed in 97.6% patients. Cortical contact (odds ratio [OR] 8.6, 95% CI 4.2-15.5, < 0.001) and a higher proliferation index (OR 5.7, 95% CI 1.3-26.2, = 0.02) were identified as independent risk factors of epilepsy. Patients with PCNSL and epilepsy had a significantly shorter progression-free survival (median progression-free survival 9.6 vs 14.1 months, adjusted hazard ratio 1.4, 95% CI 1.0-1.9, = 0.03), but not a significantly shorter overall survival (17 vs 44.1 months, log-rank test, = 0.09).
Epilepsy affects a quarter of patients with PCNSL, with half experiencing it at the time of initial presentation and potentially serving as a marker of disease progression. Further research is necessary to assess the broader applicability of these findings because they are subject to the constraints of a retrospective design and tertiary center setting.
癫痫是脑肿瘤的常见合并症;然而,对于原发性中枢神经系统淋巴瘤(PCNSL)中癫痫的患病率、发病时间、症状学和危险因素知之甚少。我们的目标是确定 PCNSL 中癫痫的患病率,确定与癫痫相关的因素,并研究癫痫发作对 PCNSL 的预后意义。
我们在一家三级神经肿瘤中心(2011-2023 年)进行了一项观察性、回顾性单中心研究,纳入了免疫功能正常的 PCNSL 患者且无癫痫发作史。我们收集了临床、影像学和治疗数据;PCNSL 病程中的癫痫发作状态;以及肿瘤学和癫痫发作结果。主要结局是确定癫痫的患病率。此外,我们旨在确定与癫痫相关的临床、影像学和治疗相关因素。使用 χ 检验对分类变量和未配对 t 检验对连续变量进行单变量分析。未调整分析中确定的预测因素被纳入向后逐步逻辑回归模型。
我们纳入了 330 例患者,其中 157 例(47.6%)为男性,诊断时的中位年龄为 68 岁,中位卡氏功能状态评分(Karnofsky Performance Status score)为 60。83 例(25.2%)患者从初始诊断到最后一次随访至少有 1 次癫痫发作,40 例(12.1%)为首发症状,16 例(4.8%)在一线治疗期间,27 例(8.2%)在肿瘤进展时,6 例(1.8%)在缓解期。局灶性意识障碍性癫痫发作是最常见的癫痫发作类型,发生在 43 例(51.8%)患者中。抗癫痫药物治疗后癫痫发作无发作率为 97.6%。皮质接触(比值比[OR] 8.6,95%置信区间[CI] 4.2-15.5,<0.001)和较高的增殖指数(OR 5.7,95% CI 1.3-26.2,=0.02)被确定为癫痫的独立危险因素。患有 PCNSL 和癫痫的患者无进展生存期显著缩短(中位无进展生存期 9.6 与 14.1 个月,调整后的危险比 1.4,95% CI 1.0-1.9,=0.03),但总生存期无显著缩短(17 与 44.1 个月,对数秩检验,=0.09)。
癫痫影响四分之一的 PCNSL 患者,其中一半在初次就诊时就出现癫痫发作,可能是疾病进展的标志物。需要进一步研究来评估这些发现的更广泛适用性,因为它们受到回顾性设计和三级中心设置的限制。