Li Sixian, Cai Xiaodong, Yao Chen, Wang Yuanqing, Xiao Xiaohua, Yang Huafeng, Yao Yi, Chen Lei
Department of Neurosurgery, Shenzhen Second People's Hospital, The First Affiliated Hospital of Shenzhen University, Shenzhen, China.
Department of Functional Neurosurgery, Xiamen Humanity Hospital, Xiamen, China.
Front Neurol. 2022 Jun 20;13:857767. doi: 10.3389/fneur.2022.857767. eCollection 2022.
The onset of Lennox-Gastaut syndrome (LGS), a severe epilepsy syndrome, is typically before 8 years of age. Late-onset LGS (with onset in adolescence and adulthood) is relatively rare clinically and has some differences from classical LGS. Herein, we describe the case of a patient with late-onset LGS and provide a literature review of such cases. The patient had focal epilepsy onset at 8 years of age. After a 9-year evolution, he suffered seizures of different types and had a diagnosis of late-onset LGS. Drug treatment was ineffective. Nothing was found on stereotactic electroencephalography (SEEG) and magnetic resonance imaging (MRI) during the course of the disease. After the second presurgical evaluation, we found a suspicious focus on high-resolution structural MRI which was verified by SEEG at last. After SEEG-guided radiofrequency thermocoagulation (RFTC), his seizures were controlled, and his cognitive function and quality of living clearly improved. However, his seizures recurred 2 years later, and he underwent left occipital resection. Thereafter, his seizures have been controlled until now. This case emphasizes the importance of high-resolution structural MRI in the treatment of LGS. Furthermore, it suggests that late-onset LGS may be caused by focal lesions and evolve from focal epilepsy. Thus, characterizing the clinical symptoms and performing individualized electroencephalographic follow-up are both very important. Additionally, the clinical outcome in this case implies the value and limitations of RFTC in patients with epilepsy and a clear focal lesion. Moreover, this case further supports differences between late-onset and classical LGS in terms of clinical manifestation, cognitive changes, prognosis, and treatment.
Lennox-Gastaut综合征（LGS）是一种严重的癫痫综合征，通常在8岁之前发病。晚发型LGS（发病于青春期和成年期）在临床上相对少见，与经典LGS存在一些差异。在此，我们描述一例晚发型LGS患者的病例，并对这类病例进行文献综述。该患者8岁时出现局灶性癫痫发作。经过9年的病情演变，他出现了不同类型的发作，并被诊断为晚发型LGS。药物治疗无效。在疾病过程中，立体定向脑电图（SEEG）和磁共振成像（MRI）均未发现异常。在第二次术前评估后，我们在高分辨率结构MRI上发现一个可疑病灶，最终经SEEG证实。经SEEG引导下射频热凝术（RFTC）治疗后，他的癫痫发作得到控制，认知功能和生活质量明显改善。然而，2年后他的癫痫发作复发，随后接受了左枕叶切除术。此后，他的癫痫发作至今得到控制。该病例强调了高分辨率结构MRI在LGS治疗中的重要性。此外，这表明晚发型LGS可能由局灶性病变引起，并从局灶性癫痫演变而来。因此，明确临床症状并进行个体化脑电图随访都非常重要。此外，该病例的临床结果暗示了RFTC在有明确局灶性病变的癫痫患者中的价值和局限性。而且，该病例进一步支持了晚发型和经典LGS在临床表现、认知变化、预后和治疗方面的差异。
