Department of Neurology, Medical Center The Hague, The Hague, The Netherlands.
Epilepsia. 2013 Dec;54 Suppl 9:12-7. doi: 10.1111/epi.12437.
Epilepsy in neuroepithelial tumors is highly prevalent. Neurogliomas (dysembryoplastic neuroepitheliomas [DNETs] and gangliogliomas) have a seizure incidence of 80-100%, low-grade gliomas of 60-85%, and glioblastoma of 30-60%. With each type, the appearance of seizures is usually the presenting clinical symptom, and with neuroglial tumors often the only clinical sign. Tumor locations in the temporal and insular cortex are associated with a higher risk of developing epilepsy in both neuroglial tumors and low-grade gliomas. Focal seizures with or without alteration of consciousness and/or secondary generalization are common. Focal seizures with altered consciousness are present in 50-70% of neuroglial tumors, and secondarily generalized seizures in 70% of low-grade gliomas. Surgical treatment, particularly gross tumor resection, contributes strongly to seizure freedom, especially in neuroglial tumors. Refractory epilepsy is more common in low-grade gliomas, occurring in 30-35%. Recurrence or worsening of seizures is often associated with tumor recurrence in glioblastomas. Translational studies have revealed a strong prevalence of IDH1 enzyme mutation together with the presence of seizures and long-term survival in low-grade gliomas. Disturbances of glutamate metabolism occur both in low-grade tumors and glioblastomas, and provide insight into mutual cellular pathway abnormalities contributing to both seizure development and tumor growth. Likewise, the recent clinical observations on antitumor activity of the anticonvulsant valproic acid in glioblastoma now provide promising outlooks on single therapies that target both seizures and gliomas.
神经上皮肿瘤中癫痫非常普遍。神经胶质瘤（发育不良性神经上皮瘤[DNET]和神经节细胞瘤）的癫痫发病率为 80-100%，低级别胶质瘤为 60-85%，胶质母细胞瘤为 30-60%。对于每种类型，癫痫发作的出现通常是其首发的临床症状，也是神经胶质肿瘤的唯一临床特征。颞叶和岛叶皮质的肿瘤部位与神经胶质肿瘤和低级别胶质瘤发生癫痫的风险增加有关。常伴有或不伴有意识改变和/或继发全面性发作的局灶性发作很常见。意识改变的局灶性发作在神经胶质肿瘤中占 50-70%，继发全面性发作在低级别胶质瘤中占 70%。手术治疗，特别是大体肿瘤切除术，对癫痫发作的控制有很大帮助，尤其是在神经胶质肿瘤中。低级别胶质瘤的耐药性癫痫更为常见，发生率为 30-35%。在胶质母细胞瘤中，癫痫的复发或恶化通常与肿瘤的复发有关。转化研究揭示了 IDH1 酶突变的高发生率与低级别胶质瘤中癫痫发作和长期生存有关。谷氨酸代谢紊乱既存在于低级别肿瘤中，也存在于胶质母细胞瘤中，为癫痫发作的发展和肿瘤生长提供了共同的细胞途径异常的见解。同样，抗癫痫药丙戊酸在胶质母细胞瘤中具有抗肿瘤活性的最近临床观察，为靶向癫痫发作和胶质瘤的单一疗法提供了有希望的前景。
