Kokoszka Malgosia A, McGoldrick Patricia E, La Vega-Talbott Maite, Raynes Hillary, Palmese Christina A, Wolf Steven M, Harden Cynthia L, Ghatan Saadi
Departments of 1 Neurosurgery and.
Neurology, Mount Sinai Health System, New York, New York.
J Neurosurg Pediatr. 2017 Feb;19(2):196-207. doi: 10.3171/2016.7.PEDS1651. Epub 2016 Nov 25.
OBJECTIVE The purpose of this study was to report outcomes of epilepsy surgery in 56 consecutive patients with autism spectrum disorder. METHODS Medical records of 56 consecutive patients with autism who underwent epilepsy surgery were reviewed with regard to clinical characteristics, surgical management, postoperative seizure control, and behavioral changes. RESULTS Of the 56 patients with autism, 39 were male, 45 were severely autistic, 27 had a history of clinically significant levels of aggression and other disruptive behaviors, and 30 were considered nonverbal at baseline. Etiology of the epilepsy was known in 32 cases, and included structural lesions, medical history, and developmental and genetic factors. Twenty-nine patients underwent resective treatments (in 8 cases combined with palliative procedures), 24 patients had only palliative treatments, and 3 patients had only subdural electroencephalography. Eighteen of the 56 patients had more than one operation. The mean age at surgery was 11 ± 6.5 years (range 1.5-35 years). At a mean follow-up of 47 ± 30 months (range 2-117 months), seizure outcomes included 20 Engel Class I, 12 Engel Class II, 18 Engel Class III, and 3 Engel Class IV cases. The age and follow-up times are stated as the mean ± SD. Three patients were able to discontinue all antiepileptic drugs (AEDs). Aggression and other aberrant behaviors observed in the clinical setting improved in 24 patients. According to caregivers, most patients also experienced some degree of improvement in daily social and cognitive function. Three patients had no functional or behavioral changes associated with seizure reduction, and 2 patients experienced worsening of seizures and behavioral symptoms. CONCLUSIONS Epilepsy surgery in patients with autism is feasible, with no indication that the comorbidity of autism should preclude a good outcome. Resective and palliative treatments brought seizure freedom or seizure reduction to the majority of patients, although one-third of the patients in this study required more than one procedure to achieve worthwhile improvement in the long term, and few patients were able to discontinue all AEDs. The number of palliative procedures performed, the need for multiple interventions, and continued use of AEDs highlight the complex etiology of epilepsy in patients with autism spectrum disorder. These considerations underscore the need for continued analysis, review, and reporting of surgical outcomes in patients with autism, which may aid in better identification and management of surgical candidates. The reduction in aberrant behaviors observed in this series suggests that some behaviors previously attributed to autism may be associated with intractable epilepsy, and further highlights the need for systematic evaluation of the relationship between the symptoms of autism and refractory seizures.
目的 本研究旨在报告56例连续性自闭症谱系障碍患者接受癫痫手术的结果。方法 回顾了56例连续性接受癫痫手术的自闭症患者的病历,内容包括临床特征、手术治疗、术后癫痫控制及行为变化。结果 56例自闭症患者中,男性39例,重度自闭症45例,有临床显著攻击性行为及其他破坏性行为史者27例,基线时30例被认为无语言能力。32例患者癫痫病因明确,包括结构性病变、病史、发育及遗传因素。29例患者接受了切除性治疗(8例联合姑息性手术),24例患者仅接受姑息性治疗,3例患者仅接受硬膜下脑电图检查。56例患者中有18例接受了不止一次手术。手术平均年龄为11±6.5岁(范围1.5 - 35岁)。平均随访47±30个月(范围2 - 117个月),癫痫发作结果包括20例Engel I级、12例Engel II级、18例Engel III级和3例Engel IV级。年龄和随访时间以均值±标准差表示。3例患者能够停用所有抗癫痫药物(AEDs)。24例患者临床观察到的攻击性行为及其他异常行为有所改善。据照料者称,大多数患者在日常社交和认知功能方面也有一定程度的改善。3例患者未出现与癫痫发作减少相关的功能或行为变化,2例患者癫痫发作及行为症状恶化。结论 自闭症患者的癫痫手术是可行的,没有迹象表明自闭症合并症会妨碍良好的手术结果。切除性和姑息性治疗使大多数患者实现了癫痫发作自由或发作减少,尽管本研究中有三分之一的患者需要不止一次手术才能在长期内取得有价值的改善,且很少有患者能够停用所有AEDs。进行的姑息性手术数量、多次干预的必要性以及AEDs的持续使用突出了自闭症谱系障碍患者癫痫病因的复杂性。这些考虑强调了对自闭症患者手术结果进行持续分析、审查和报告的必要性,这可能有助于更好地识别和管理手术候选者。本系列中观察到的异常行为减少表明,一些先前归因于自闭症的行为可能与难治性癫痫有关,并进一步强调了系统评估自闭症症状与难治性癫痫之间关系的必要性。