Giannantoni Nadia Mariagrazia, Della Marca Giacomo, Vollono Catello
Institute of Neurology, Catholic University, Rome, Italy
Institute of Neurology, Catholic University, Rome, Italy.
Clin EEG Neurosci. 2015 Oct;46(4):353-6. doi: 10.1177/1550059414538255. Epub 2014 Nov 11.
Sturge-Weber syndrome is a rare, sporadic, congenital neurocutaneous syndrome, likely due to abnormal development of the cephalic microvasculature. Symptoms and signs depend on the extent and location of the venous dysplasia. We describe a case of a 33-year-old woman presenting with drug-resistant epilepsy, chronic headache, and recurring nonepileptic seizures. Computed tomography and magnetic resonance imaging scans showed severe frontoparietal right hemisphere atrophy, prevalent right frontoparietal leptomeningeal enhancement, circumscribed angioma of the left rolandic sulcus, and prominent deep venous system. We report an apparently classical Sturge-Weber syndrome and hypothesize a shared pathophysiologic mechanism for clinical symptoms. We speculate that all the main symptoms observed in our patient could be the expression of a functional imbalance between the atrophic right hemisphere and the hyperexcitable left cortex.
斯特奇-韦伯综合征是一种罕见的、散发性的先天性神经皮肤综合征，可能是由于头部微血管发育异常所致。症状和体征取决于静脉发育异常的程度和位置。我们描述了一例33岁女性患者，她患有耐药性癫痫、慢性头痛和反复出现的非癫痫性发作。计算机断层扫描和磁共振成像扫描显示严重的右侧额顶叶萎缩、右侧额顶叶软脑膜普遍强化、左侧中央沟局限性血管瘤以及明显的深部静脉系统。我们报告了一例明显典型的斯特奇-韦伯综合征，并推测其临床症状存在共同的病理生理机制。我们推测在我们患者中观察到的所有主要症状可能是萎缩的右侧半球和兴奋性过高的左侧皮质之间功能失衡的表现。
