Department of Clinical Neurosciences, CHUV, University Hospital of Lausanne and University of Lausanne, Lausanne, Switzerland.
Division of Neurology, Centre Hospitalier Université de Montréal, Montreal, Québec, Canada.
Curr Opin Neurol. 2021 Apr 1;34(2):153-165. doi: 10.1097/WCO.0000000000000907.
The increased identification of seizures with insular ictal onset, promoted by the international development of stereo-electroencephalography (SEEG), has led to the recent description of larger cohorts of patients with insular or insulo-opercular epilepsies than those previously available. These new series have consolidated and extended our knowledge of the rich ictal semiology and diverse anatomo-clinical correlations that characterized insular seizures. In parallel, some experiences have been gained in the surgical treatment of insular epilepsies using minimal invasive procedures.
The large majority of patients present with auras (mostly somatosensory and laryngeal) and motor signs (predominantly elementary and orofacial), an underlying focal cortical dysplasia, and an excellent postoperative seizure outcome. Many other subjective and objective ictal signs, known to occur in other forms of epilepsies, are also observed and clustered in five patterns, reflecting the functional anatomy of the insula and its overlying opercula, as well as preferential propagation pathways to frontal or temporal brain regions. A nocturnal predominance of seizure is frequently reported, whereas secondary generalization is infrequent. Some rare ictal signs are highly suggestive of an insular origin, including somatic pain, reflex seizures, choking spells, and vomiting. Minimal invasive surgical techniques have been applied to the treatment of insular epilepsies, including Magnetic Resonance Imaging-guided laser ablation (laser interstitial thermal therapy (LITT)), radiofrequency thermocoagulation (RFTC), gamma knife radiosurgery, and responsive neurostimulation. Rates of seizure freedom (about 50%) appear lower than that reported with open-surgery (about 80%) with yet a significant proportion of transient neurological deficit for LITT and RFTC.
Significant progress has been made in the identification and surgical treatment of insular and insulo-opercular epilepsies, including more precise anatomo-clinical correlations to optimally plan SEEG investigations, and experience in using minimal invasive surgery to reduce peri-operative morbidity.
随着国际立体脑电图(SEEG)技术的发展,越来越多的局灶性癫痫发作起源于岛叶,近期描述了比以往更多的岛叶或岛盖部癫痫患者的大型队列。这些新系列巩固和扩展了我们对丰富的癫痫发作症状学和多样化的解剖-临床相关性的认识,这些特征是岛叶癫痫发作的特点。与此同时,使用微创程序对岛叶癫痫进行手术治疗也积累了一些经验。
绝大多数患者表现为先兆(主要是躯体感觉和喉部)和运动症状(主要是基本的和口腔面部的)、潜在的局灶性皮质发育不良和极佳的术后癫痫发作结果。许多其他已知发生在其他形式癫痫中的主观和客观的癫痫发作症状也被观察到,并聚类为五种模式,反映了岛叶及其覆盖的脑盖的功能解剖结构,以及向额叶或颞叶脑区的优先传播途径。夜间发作常常占优势,而继发性全面性发作则很少见。一些罕见的癫痫发作症状高度提示起源于岛叶,包括躯体疼痛、反射性癫痫发作、窒息发作和呕吐。微创技术已应用于岛叶癫痫的治疗,包括磁共振成像引导下激光消融(激光间质热疗(LITT))、射频热凝(RFTC)、伽玛刀放射外科和反应性神经刺激。无癫痫发作的比例(约 50%)似乎低于开放性手术(约 80%),但 LITT 和 RFTC 的暂时性神经功能缺损比例仍然较高。
在岛叶和岛盖部癫痫的识别和手术治疗方面取得了重大进展,包括更精确的解剖-临床相关性,以优化 SEEG 研究计划,以及使用微创手术降低围手术期发病率的经验。