Romanelli Pantaleo, Verdecchia Magda, Rodas Raul, Seri Stefano, Curatolo Paolo
Department of Neurosurgery, Stanford University, Palo Alto, California, USA.
Pediatr Neurol. 2004 Oct;31(4):239-47. doi: 10.1016/j.pediatrneurol.2004.05.012.
Tuberous sclerosis complex is often associated with medically refractory epilepsy secondary to cortical tubers. Previous studies have identified an association between early seizure onset, greater seizure burden, and mental retardation in childhood. Early effective seizure control could therefore significantly reduce the adverse developmental effects of chronic epilepsy in tuberous sclerosis complex. In medically intractable epileptic patients, surgical treatment has been demonstrated to reduce or abolish seizures and the associated burden for the child and its environment. Epilepsy surgery has not been widely used in patients with tuberous sclerosis complex thus far, mostly because of the concern that the multifocal nature of the cortical lesions could be associated with the development of independent epileptogenic zones. Advances in electroencephalographic techniques, functional neuroimaging, and invasive cortical mapping are changing this view and allowing an increased number of tuberous sclerosis complex patients to be evaluated for resective surgery. Additional techniques emerging in the neurosurgical field may add further treatment options to the current state of the art.
结节性硬化症常与继发于皮质结节的药物难治性癫痫相关。既往研究已证实儿童期癫痫发作早、发作负担重与智力发育迟缓之间存在关联。因此,早期有效控制癫痫发作可显著减轻结节性硬化症中慢性癫痫对发育的不良影响。对于药物难治性癫痫患者,手术治疗已被证明可减少或消除癫痫发作以及对患儿及其周围环境的相关负担。迄今为止,癫痫手术在结节性硬化症患者中尚未得到广泛应用,主要是因为担心皮质病变的多灶性可能与独立致痫区的形成有关。脑电图技术、功能神经影像学和侵入性皮质图谱的进展正在改变这一观点,并使越来越多的结节性硬化症患者能够接受切除性手术评估。神经外科领域出现的其他技术可能会为当前的技术水平增加更多的治疗选择。