Neuronal Networks Group, Neuropharmacology and Neurobiology, School of Clinical and Experimental Medicine, University of Birmingham, Birmingham, United Kingdom.
Epileptic Disord. 2011 Mar;13(1):76-81. doi: 10.1684/epd.2011.0407.
Sturge-Weber syndrome is a neurocutaneous disorder classically characterized by the presence of facial port-wine stain and ipsilateral leptomeningeal angiomatosis. It is often associated with refractory epilepsy which requires surgical treatment. We present a case of a patient who initially presented with partial seizures of temporo-occipital origin, ipsilateral to the pial angiomatosis. During the course of the disease, the patient developed medically refractory epilepsy with partial seizures originating predominantly from the contralateral temporo-occipital area as well as myoclonic and myoclonic-astatic seizures. Resection of the occipital and temporal lobe affected by the pial angioma resulted in favourable outcome. Bilateral dysfunction observed in Sturge-Weber syndrome may result in an increased capability of focal discharges to generate synchronous epileptiform activity leading to an increased incidence of generalised seizures, most probably via a mechanism of secondary bilateral synchrony. [Published with video sequences].
斯特奇-韦伯综合征是一种神经皮肤疾病，其特征为存在面部葡萄酒色斑和同侧软脑膜血管畸形。它常伴有难治性癫痫，需要手术治疗。我们报告了一例患者，其最初表现为起源于软脑膜血管畸形同侧的颞枕部部分性发作。在疾病过程中，患者出现药物难治性癫痫，部分性发作主要起源于对侧颞枕区，以及肌阵挛和肌阵挛-强直发作。切除受软脑膜血管瘤影响的枕叶和颞叶导致了良好的结果。斯特奇-韦伯综合征双侧功能障碍可能导致局灶性放电产生同步癫痫样活动的能力增加，从而导致全身性发作的发生率增加，这很可能是通过继发性双侧同步的机制。[附有视频序列]。
