Taddeucci Grazia, Bonuccelli Alice, Polacco Paola
Department of Pediatrics, Section of Pediatric Neurology, University of Studies of Pisa, Via Fermi 9, 56126 Pisa, Italy.
Pediatr Neurol. 2005 Feb;32(2):131-3. doi: 10.1016/j.pediatrneurol.2004.08.003.
The Sturge-Weber syndrome was recently subdivided into type I (facial and leptomeningeal angioma, possible glaucoma), type II (facial angioma, without evident endocranial involvement), and type III (exclusive leptomeningeal angioma). Thus far in the literature only 24 cases of Sturge-Weber syndrome type III have been reported. This study presents a case of a 2-year 9-month-old child with normal psychomotor development and skin free (no angiomas), who presented repeated episodes of severe headache, vertiginous symptoms, vomiting, and drowsiness, separated by complete recovery. The cranial computed tomography and magnetic resonance imaging with gadolinium revealed left occipital leptomeningeal angiomatosis with calcifications, suggesting a diagnosis of Sturge-Weber syndrome type III. Considering the normal psychomotor development, the improved electroencephalographic reports between the episodes, and the absence of hypoperfusion areas on single-photon emission computed tomography at 30 months of follow-up, the symptomatology appears an expression of migraine-like symptoms resulting from vasomotor disturbances within and around the angioma, more than an expression of partial seizures arising through an epileptic focus in the ischemic region around the vascular malformation.
斯特奇-韦伯综合征最近被细分为I型(面部和软脑膜血管瘤,可能伴有青光眼)、II型(面部血管瘤,无明显颅内受累)和III型(单纯软脑膜血管瘤)。迄今为止,文献中仅报道了24例III型斯特奇-韦伯综合征。本研究报告了一例2岁9个月大的儿童,其精神运动发育正常且皮肤无异常(无血管瘤),但反复出现严重头痛、眩晕症状、呕吐和嗜睡,发作间期完全恢复。头颅计算机断层扫描和钆增强磁共振成像显示左侧枕叶软脑膜血管瘤病伴钙化,提示诊断为III型斯特奇-韦伯综合征。考虑到其精神运动发育正常、发作间期脑电图报告有所改善以及随访30个月时单光子发射计算机断层扫描未发现灌注不足区域,这些症状似乎是由血管瘤内及周围血管运动障碍引起的偏头痛样症状的表现,而非由血管畸形周围缺血区域的癫痫病灶引发的部分性癫痫发作的表现。