Boston University School of Medicine, Suite 7B, 7th Floor, 725 Albany St, Boston, MA 02118, USA.
Austin Epilepsy Care Center, Suite 203, 2200 Park Bend Drive Building 2, Austin, TX 78758, USA.
Epilepsy Behav. 2020 Sep;110:107146. doi: 10.1016/j.yebeh.2020.107146. Epub 2020 Jun 18.
Lennox-Gastaut syndrome (LGS) is a severe developmental epileptic encephalopathy diagnosed in childhood that persists through adolescence and into adulthood. While the characteristics of LGS in pediatric patients are well defined, including "drop attacks", interictal slow spike and wave electroencephalogram (EEG) activity, and intellectual disability, these features can evolve over time, and different EEG activities may be present in adult patients with LGS. This may result in missed diagnoses in these patients and subsequent challenges for the adequate treatment of their seizures. Based on discussions held during the LGS Transition of Care advisory board meeting and thereafter, we developed proposed diagnostic and treatment algorithms for LGS in adult patients. We highlight readily available assessments to facilitate diagnosis of LGS, based on past medical history and physical examination. The LGS diagnostic algorithm recommends that clinicians consider the occurrence of wider seizure types and abnormal EEG activities to be potentially indicative of LGS. Seizure types may include atypical absence seizures, myoclonic seizures, focal seizures, and tonic-clonic seizures, and EEG may demonstrate background slowing, focal or multifocal epileptiform discharges, and diffuse fast rhythms during sleep, among other activities. Extended EEG during sleep and video-EEG should be used in equivocal cases. Treatment of LGS in adult patients should incorporate both antiseizure drug (ASD) therapy and nonpharmacologic approaches. Frequent reassessment of patients is considered a central aspect. ASDs were categorized based on order of preference for use in the treatment of LGS; Tier 1 comprises recommended first-line ASDs, and includes valproate, clobazam, lamotrigine, rufinamide, topiramate, and cannabidiol. Other treatment options include diet, neurostimulation, and surgical approaches. Developments with the potential to improve diagnosis in the future include genetic screening, while novel ASDs and advances in neurostimulation techniques may provide valuable treatment options. These algorithms should be frequently revisited to incorporate improved techniques and therapies.
Lennox-Gastaut 综合征(LGS)是一种在儿童期诊断的严重发育性癫痫性脑病,持续到青少年期和成年期。虽然儿科患者的 LGS 特征定义明确,包括“跌倒发作”、间发性棘慢复合波脑电图(EEG)活动和智力障碍,但这些特征会随时间演变,并且不同的 EEG 活动可能存在于成年 LGS 患者中。这可能导致这些患者的漏诊,并随后对其癫痫发作的充分治疗带来挑战。基于在 Lennox-Gastaut 综合征过渡护理顾问委员会会议期间和之后进行的讨论,我们为成年患者制定了 LGS 的诊断和治疗算法建议。我们根据既往病史和体格检查,强调了便于 LGS 诊断的现成评估方法。LGS 诊断算法建议临床医生考虑更广泛的癫痫发作类型和异常 EEG 活动可能提示 LGS。癫痫发作类型可能包括非典型失神发作、肌阵挛发作、局灶性发作和强直-阵挛发作,而 EEG 可能显示背景减慢、局灶或多灶性癫痫样放电以及睡眠时弥漫性快节律等活动。在可疑病例中应使用睡眠中延长 EEG 和视频 EEG。成年患者的 LGS 治疗应结合抗癫痫药物(ASD)治疗和非药物方法。频繁重新评估患者被认为是一个核心方面。ASD 根据在 LGS 治疗中的使用优先级进行分类;第 1 层包括推荐的一线 ASD,包括丙戊酸、氯巴占、拉莫三嗪、鲁非酰胺、托吡酯和大麻二酚。其他治疗选择包括饮食、神经刺激和手术方法。未来有潜力改善诊断的发展包括基因筛查,而新型 ASD 和神经刺激技术的进步可能提供有价值的治疗选择。这些算法应经常重新审查,以纳入改进的技术和疗法。