Current role of surgery for tuberous sclerosis complex-associated epilepsy.

Nicola Specchio, Giusy Carfi Pavia, Luca de Palma, Alessandro De Benedictis, Chiara Pepi, Marta Conti, Carlo Efisio Marras, Federico Vigevano, Paolo Curatolo
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Abstract

Tuberous sclerosis complex (TSC) is a rare multisystem, autosomal dominant neurocutaneous syndrome in which epilepsy is the most common of several neurological and psychiatric manifestations. Around two thirds of patients develop drug-resistant epilepsy for whom surgical resection of epileptogenic foci is indicated when seizures remain inadequately controlled following trial of two antiseizure medications. The challenge with presurgical and surgical approaches with patients with TSC is overcoming the complexity from the number of tubers and the multiplex epileptogenic network forming the epileptogenic zone. Data suggest that seizure freedom is achieved by 55%-60% of patients, but predictive factors for success have remained elusive, which makes for unconfident selection of surgical candidates. This article presents three different cases as illustrations of the potential challenges faced when assessing the suitability of TSC patients for epilepsy surgery.

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手术治疗结节性硬化症复合体相关癫痫的当前作用。
结节性硬化综合征(TSC)是一种罕见的多系统常染色体显性神经皮肤综合征,在多种神经和精神表现中,癫痫是最常见的一种。约有三分之二的患者会发展为耐药性癫痫,在服用两种抗癫痫药物后仍不能有效控制癫痫发作时,就需要通过手术切除致痫灶。TSC患者的术前和手术方法面临的挑战是克服致痫区的管状体数量和多重致痫网络所带来的复杂性。有数据表明,55%-60%的患者可以摆脱癫痫发作,但成功的预测因素仍然难以捉摸,这使得手术候选者的选择缺乏信心。本文介绍了三个不同的病例,以说明在评估 TSC 患者是否适合接受癫痫手术时所面临的潜在挑战。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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