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直接切除是控制下丘脑错构瘤患者癫痫发作的一种安全有效的策略。

Direct resection is a safe and effective strategy to control seizures in patients with hypothalamic hamartoma.

机构信息

Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.

The Institute of Artificial Intelligence, Hefei Comprehensive National Science Center, Hefei, Anhui, China.

出版信息

Sci Rep. 2024 Jun 6;14(1):13001. doi: 10.1038/s41598-024-63480-3.

Abstract

Achieving favorable seizure outcomes is challenging in patients with seizures resulting from hypothalamic hamartoma. Although minimally invasive and non-invasive surgical procedures are used to treat this population, these procedures have limitations. Therefore, we analyzed the outcomes of patients with hypothalamic hamartoma following direct resection. We included 159 patients with hypothalamic hamartoma who underwent direct resection using the transcallosal interforniceal approach between 2011 and 2018. The relationships between clinical parameters and seizure outcomes were analyzed. In total, 55.3% achieved gross total resection and 25.2% underwent near-total resection. Of all patients, 79.2% were overall seizure-free at one year, but this number dropped to 77.0% at more than five years. Moreover, 88.4% (129/146) reached gelastic seizure (GS)-free status at one year and this number increased to 89.0% (97/109) at more than five years. Seventy-one patients took antiseizure medication (ASM) long-term, 68 took it for one year, and 11 took it for one-half year. The duration of ASM consumption (p < 0.001) and extent of hypothalamic hamartoma resection (p = 0.016) were significant independent predictors of long-term overall seizure-free survival, while the duration of ASM consumption (p = 0.011) and extent of hypothalamic hamartoma resection (p = 0.026) were significant independent predictors of long-term GS-free survival. Most patients' behavior, school performance, and intelligence were not affected after surgery. Direct resection is effective and safe strategy for patients with hypothalamic hamartomas. Hypothalamic hamartomas should be removed as completely as possible, and patients should take ASM long-term following surgery to reach long-term overall seizure-free or GS-free status.

摘要

实现下丘脑错构瘤致痫患者的良好癫痫结局具有挑战性。虽然采用微创和非侵入性手术治疗该人群,但这些方法存在局限性。因此,我们分析了直接切除下丘脑错构瘤患者的结局。我们纳入了 2011 年至 2018 年间采用经胼胝体-中间帆池入路行直接切除术的 159 例下丘脑错构瘤患者。分析了临床参数与癫痫发作结局的关系。共有 55.3%患者达到大体全切除,25.2%患者行近全切除。所有患者中,1 年时总体无癫痫发作率为 79.2%,但 5 年以上时降至 77.0%。此外,1 年时 88.4%(129/146)患者达到痴笑发作(GS)无发作状态,5 年以上时增至 89.0%(97/109)。71 例患者长期服用抗癫痫药物(ASM),68 例患者服用 1 年,11 例患者服用半年。ASM 服用时间(p<0.001)和下丘脑错构瘤切除范围(p=0.016)是长期总体无癫痫发作生存的显著独立预测因素,而 ASM 服用时间(p=0.011)和下丘脑错构瘤切除范围(p=0.026)是长期 GS 无发作生存的显著独立预测因素。大多数患者术后行为、学业成绩和智力不受影响。直接切除术是治疗下丘脑错构瘤患者的有效且安全的策略。下丘脑错构瘤应尽可能完全切除,术后患者应长期服用 ASM,以达到长期总体无癫痫发作或 GS 无发作状态。

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本文引用的文献

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Advances in epileptic network findings of hypothalamic hamartomas.下丘脑错构瘤癫痫网络研究进展。
J Cent Nerv Syst Dis. 2024 Mar 5;16:11795735241237627. doi: 10.1177/11795735241237627. eCollection 2024.
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Management of Epilepsy Due to Hypothalamic Hamartomas.下丘脑错构瘤相关癫痫的治疗。
Pediatr Neurol. 2017 Oct;75:29-42. doi: 10.1016/j.pediatrneurol.2017.07.001. Epub 2017 Jul 5.

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