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脑皮层发育畸形:新的手术进展。

Malformations of cortical development: New surgical advances.

机构信息

Department of Neurosurgery, Hospices Civils de Lyon, Neurology & Neurosurgery Hospital Pierre Wertheimer, 59, boulevard Pinel, 69003 Lyon, France; Faculty of medicine Claude Bernard, University of Lyon, 69003 Lyon, France; Sorbonne university, 75005 Paris, France; Inserm U1127, CNRS, UMR7225, Brain and Spine Institute, 75013 Paris, France.

Faculty of medicine Claude Bernard, University of Lyon, 69003 Lyon, France; Department of Functional Neurology and Epileptology, Hospices Civils de Lyon, Neurology & Neurosurgery Hospital Pierre Wertheimer, 69003 Lyon, France; TIGER, Inserm U1028, CNRS 5292, Neuroscience research center of Lyon, 69003 Lyon, France.

出版信息

Rev Neurol (Paris). 2019 Mar;175(3):183-188. doi: 10.1016/j.neurol.2019.01.392. Epub 2019 Feb 26.

DOI:10.1016/j.neurol.2019.01.392
PMID:30819503
Abstract

Epilepsy related to malformations of cortical development is frequently drug resistant or requires heavy medication, therefore surgery is key in their management. The role of stereotactic surgery has recently changed the diagnosis and treatment of focal cortical dysplasias (FCD), hypothalamic hamartomas (HH) and periventricular nodular heterotopias (PNH). In HH, radiosurgery using Gammaknife leads to 60 % of seizure control and is associated with excellent neuropsychological results without significant endocrine function impairment. The seizure control rate is even higher (more than 80 %) with monopolar multiple stereotactic thermocoagulations and Laser interstitial Thermal Therapy (LiTT). While the first technique is associated with a 2 % complications rate (but with excellent neuropsychological outcomes), the latest has up to 22 % side effects in some series. All three of these techniques have encouraging results, but controlled studies are still lacking to provide evidence-based new therapeutic algorithms. With regard to the PNH, surgical management has long been limited by the depth of the lesions and their close anatomical relations with the functional brain connectome. Stereotactic approaches required to perform a SEEG, to locate the part of the PNH responsible for the seizure onset, are later followed by a stereotactic lesioning procedure, therefore doubling the bleeding risk. That is why SEEG-guided radiofrequency-thermocoagulation (SEEG guided-RF-TC), which makes it possible to perform these two steps in a single procedure, was considered as a promising option. A recent meta-analysis confirmed this intuition and reported 38 % of seizure-free patients and 81 % of responders with only 0.3 % of complications, making this approach the first treatment line, followed by LiTT. Among the multiple advances in the FCD identification by non-invasive investigations, a new modality of per-operative diagnostic procedure, the three-dimensional electrocorticography may lead to simplify the preoperative investigation and enhance the accuracy of FCD delineation. Evidence is nevertheless still insufficient to validate this promising concept. Conventional surgical resection has also been concerned by significant conceptual advances during the past few years, in particular with the development of the hodotopic approach, initially in oncologic surgery. Associated with a better understanding of neuroplasticity in epilepsy and the setting up of functional mapping during SEEG or during awake surgery, the possibility of surgical resections grew up. A short-term perspective in this field, when surgical resection remains impossible, would be to target crucial nodes of the epileptic network, distinct from the core functional connectome.

摘要

与皮质发育畸形相关的癫痫通常对药物有抵抗力或需要大量药物治疗,因此手术是其治疗的关键。立体定向手术的作用最近改变了局灶性皮质发育不良(FCD)、下丘脑错构瘤(HH)和脑室周围结节性异位(PNH)的诊断和治疗。在 HH 中,使用伽玛刀进行放射外科手术可使 60%的癫痫得到控制,并且具有出色的神经心理学结果,而没有明显的内分泌功能损害。单极多立体定向热凝固和激光间质热疗(LiTT)的癫痫控制率甚至更高(超过 80%)。虽然第一种技术的并发症发生率为 2%(但神经心理学结果出色),但在某些系列中,最新技术的副作用高达 22%。所有这三种技术都有令人鼓舞的结果,但仍缺乏对照研究来提供基于证据的新治疗方案。至于 PNH,由于病变的深度及其与功能性大脑连接组的密切解剖关系,手术治疗一直受到限制。立体定向方法需要进行 SEEG,以定位 PNH 引起癫痫发作的部分,然后进行立体定向病变切除术,从而使出血风险增加一倍。这就是为什么 SEEG 引导下射频热凝(SEEG 引导-RF-TC)被认为是一种有前途的选择,它可以在单个程序中完成这两个步骤。最近的一项荟萃分析证实了这一直觉,并报告了 38%的无癫痫发作患者和 81%的反应者,并发症仅为 0.3%,使这种方法成为一线治疗方法,其次是 LiTT。在非侵入性研究中识别 FCD 的多项进展中,一种新的术中诊断程序三维皮质电图可能会简化术前研究并提高 FCD 描绘的准确性。然而,目前还没有足够的证据来验证这一有前途的概念。近年来,传统的手术切除也受到了重大概念上的进步的关注,特别是在肿瘤外科中的同源性方法的发展。随着对癫痫神经可塑性的更好理解以及在 SEEG 或清醒手术期间进行功能映射的建立,手术切除的可能性增加了。在这个领域的短期前景是,当手术切除仍然不可能时,将针对癫痫网络的关键节点,与核心功能连接组不同。

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