Hahne Oscar, Rydenhag Bertil, Tranberg Anna Edelvik, Kristjánsdóttir Ragnhildur, Nilsson Daniel, Olsson Ingrid, Hallböök Tove
Department of Pediatrics, Institute of Clinical Sciences, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Gothenburg, Sweden; Member of the ERN, EpiCARE, Gothenburg, Sweden.
Member of the ERN, EpiCARE, Gothenburg, Sweden; Department of Neurosurgery, Sahlgrenska University Hospital, Gothenburg, Sweden; Institute of Neuroscience and Physiology, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.
Eur J Paediatr Neurol. 2023 Sep;46:24-29. doi: 10.1016/j.ejpn.2023.06.004. Epub 2023 Jun 17.
Hypothalamic hamartomas are benign lesions associated with drug resistant epilepsy. Surgical treatment has become an increasingly utilised approach with promising results. This study aims to evaluate seizure outcome and complications after surgery in a population-based series of patients with intractable epilepsy and hypothalamic hamartoma.
All patients with hypothalamic hamartoma treated with epilepsy surgery in Sweden since 1995 with at least two years of follow-up were included. Preoperative, two-, five- and ten-year prospective longitudinal data were collected from The Swedish National Epilepsy Surgery Register. Data included seizure types and frequency, duration of epilepsy, clinical characteristics, neurological deficits, cognitive level and complications. In a subgroup from Gothenburg, we also analysed data not included in the register such as classification of hamartomas, surgical procedures and gelastic seizures.
Eighteen patients were operated on during the period 1995-2020. The median age at epilepsy onset was 6 months and age at surgery 13 years. Four were seizure free and another four had ≥75% reduction in seizure frequency at the two-year follow-up. Two of the 13 patients with a long-term follow-up (five or ten years) were seizure-free and four had ≥75% reduction in seizure frequency. Three had an increased seizure frequency. No major complications were seen. Five had minor complications. In the Gothenburg subgroup all had open pterional disconnection or intraventricular endoscopic disconnection. Six of 12 were free from gelastic seizures at the two-year follow-up and six of eight at the long-term follow-up.
This study supports surgical treatment of hypothalamic hamartomas as a safe method with a low risk of permanent complications. The seizure reduction seems to be persistent over time.
下丘脑错构瘤是与药物难治性癫痫相关的良性病变。手术治疗已成为一种越来越常用的方法,效果良好。本研究旨在评估一系列以人群为基础的难治性癫痫合并下丘脑错构瘤患者术后的癫痫发作结局和并发症。
纳入自1995年以来在瑞典接受癫痫手术治疗且随访至少两年的所有下丘脑错构瘤患者。从瑞典国家癫痫手术登记处收集术前、术后两年、五年和十年的前瞻性纵向数据。数据包括癫痫发作类型和频率、癫痫持续时间、临床特征、神经功能缺损、认知水平和并发症。在哥德堡的一个亚组中,我们还分析了登记处未包含的数据,如错构瘤的分类、手术方式和痴笑发作。
1995年至2020年期间,18例患者接受了手术。癫痫发作的中位起始年龄为6个月,手术年龄为13岁。两年随访时,4例无癫痫发作,另外4例癫痫发作频率降低≥75%。13例长期随访(五年或十年)的患者中,2例无癫痫发作,4例癫痫发作频率降低≥75%。3例癫痫发作频率增加。未观察到重大并发症。5例有轻微并发症。在哥德堡亚组中,所有患者均接受了翼点入路开放离断术或脑室内内镜离断术。两年随访时,12例中有6例无痴笑发作,长期随访时,8例中有6例无痴笑发作。
本研究支持将下丘脑错构瘤的手术治疗作为一种安全的方法,永久性并发症风险低。癫痫发作减少似乎随时间持续存在。