DeMarco P, Lorenzin G
Centro Angeli Custodi, Istituti Ospedalieri, Trento, Italy.
Brain Dev. 1990;12(3):342-4. doi: 10.1016/s0387-7604(12)80318-4.
Recently presented data have allowed us to detect an increasing number of cases which present bilateral occipital calcifications and epilepsy or migraine. They have been indicated for the most part to have atypical forms of Sturge-Weber disease without facial nevus flammeus. Two pediatric patients are dealt with here, who, while presenting some differences from the electroclinical point of view, are characterized by typical cortico-subcortical bilateral occipital growing calcifications. Generally, other authors consider the first phase of this syndrome to comprise benign development; only in a second phase does worsening of the fits follow, as well as a bad prognosis. On the contrary, in our case up to now, the patients have been well; the seizures are under control with AEDs and EEG has not worsened, in spite of growing occipital calcifications.
最近公布的数据使我们能够检测到越来越多出现双侧枕叶钙化以及癫痫或偏头痛的病例。其中大部分病例被认为是无面部葡萄酒色斑的非典型斯特奇-韦伯综合征。本文讨论了两名儿科患者，他们虽然在电临床方面存在一些差异，但均以典型的双侧枕叶皮质-皮质下钙化生长为特征。一般来说，其他作者认为该综合征的第一阶段包括良性发展；只有在第二阶段，癫痫发作才会恶化，预后也不佳。相反，到目前为止，在我们的病例中，患者情况良好；尽管枕叶钙化在不断发展，但癫痫发作通过抗癫痫药物得到了控制，脑电图也没有恶化。
