Douglass Laurie M, Salpekar Jay
Boston Medical Center, Boston University School of Medicine, Boston, Massachusetts, U.S.A.
Epilepsia. 2014 Sep;55 Suppl 4:21-8. doi: 10.1111/epi.12742.
Despite ongoing investigation into pharmacologic treatments for Lennox-Gastaut syndrome (LGS), outcomes for chronic administration of medications remain disappointing. In many instances LGS is treatment refractory, resulting in poor prognoses that include intellectual disability, persisting seizures, and psychiatric conditions. For patients with treatment resistance to other modalities for LGS, a further option is surgical intervention. Evaluation for surgery should involve interictal electroencephalogram (EEG), magnetic resonance imaging (MRI) analysis, and age-appropriate neuropsychological/developmental assessment. Resective surgery, where seizure foci are removed, successfully controls seizures in many cases, particularly where lesionectomy or lobar resections are appropriate. Recent studies of resective surgery on individuals with LGS show promising results, with a high percentage of patients having improved seizure control. Corpus callosotomy is a palliative surgical approach that aims at controlling potentially injurious seizures, for example, atonic or drop seizures, by preventing the bilateral spread of epileptic activity. Once associated with a high risk for morbidity and mortality, microsurgical techniques and surgery limited to the anterior region of the callosum have greatly diminished complications of corpus callosotomy surgery. Vagus nerve stimulation, another palliative procedure, offers rates of seizure improvement similar to those of corpus callosotomy, with the exception of atonic seizure for which corpus callosotomy may lead to a greater reduction. Recent advances in surgical techniques offer encouraging options for treatment of LGS.
尽管对伦诺克斯 - 加斯东综合征(LGS)的药物治疗仍在进行研究,但长期用药的效果仍然令人失望。在许多情况下,LGS对治疗具有难治性,导致预后不良,包括智力残疾、持续性癫痫发作和精神疾病。对于对LGS其他治疗方式耐药的患者,另一种选择是手术干预。手术评估应包括发作间期脑电图(EEG)、磁共振成像(MRI)分析以及适合年龄的神经心理学/发育评估。切除性手术,即切除癫痫病灶,在许多情况下能成功控制癫痫发作,特别是在适合进行病灶切除术或脑叶切除术的情况下。最近对LGS患者进行切除性手术的研究显示出有希望的结果,有很高比例的患者癫痫控制情况得到改善。胼胝体切开术是一种姑息性手术方法,旨在通过防止癫痫活动的双侧传播来控制潜在的有害癫痫发作,例如失张力性或跌倒发作。胼胝体切开术曾经与高发病率和死亡率相关,而显微手术技术以及仅限于胼胝体前部区域的手术已大大减少了胼胝体切开术手术的并发症。迷走神经刺激术是另一种姑息性手术,其癫痫发作改善率与胼胝体切开术相似,但失张力性发作除外,胼胝体切开术可能对其有更大程度的减少作用。手术技术的最新进展为LGS的治疗提供了令人鼓舞的选择。