Stokes A C, Hernández-Cossio O, Hernández-Fustes O J, Munhoz R P, Hernández-Fustes O J, Francisco A N
Servicio de Neurología, Hospital Universitário Cajuru, Pontifícia Universidad Católica Del Paraná, Curitiba, Brasil.
Rev Neurol. 2000;30(1):41-4.
The Sturge-Weber syndrome is characterized by facial cutaneous angioma associated with leptomeningeal and cerebral angioma, typically ipsilateral to the facial lesion, which is accompanied by convulsions, mental retardation, contralateral hemiparesia, hemiatrophy, homonymous hemianopsia and glaucoma. Most of the patients with radiographic evidence of intracranial angioma develop convulsive crises, but only half have severe mental retardation. The image of calcification on cranial tomography often leads to confusion in diagnosis, especially with neurocysticercosis, particularly in places where this is endemic and the patients present with minimal skin lesions or these are at atypical sites.
We present the case of a 13 year-old boy hospitalized with status epilepticus who, since the age of 1 year and 3 months, had had convulsive seizures which were of generalized tonic-clonic type and partially complex with secondary generalization, treated with carbamazepine at a dose of 400 mg per day. Neurocysticercosis was diagnosed on a tomogram showing calcification of the left parieto-occipital gyrus. Following physical examination and complementary tests the diagnosis of Sturge-Weber syndrome was made.
We emphasize the importance of the diagnosis of Sturge-Weber syndrome, its clinical picture and treatment.
斯特奇-韦伯综合征的特征是面部皮肤血管瘤伴软脑膜和脑血管瘤，通常与面部病变同侧，伴有惊厥、智力迟钝、对侧偏瘫、半侧萎缩、同向性偏盲和青光眼。大多数有颅内血管瘤影像学证据的患者会发生惊厥性危机，但只有一半有严重智力迟钝。头颅断层扫描上的钙化影像常导致诊断混淆，尤其是与神经囊尾蚴病混淆，特别是在该病流行地区，且患者皮肤病变轻微或位于非典型部位时。
我们报告一例13岁男孩因癫痫持续状态住院，自1岁3个月起就有惊厥发作，为全身性强直阵挛型，部分为复杂性发作继发全身性发作，每日服用400毫克卡马西平治疗。头颅断层扫描显示左顶枕叶钙化，诊断为神经囊尾蚴病。经过体格检查和补充检查后，确诊为斯特奇-韦伯综合征。
我们强调斯特奇-韦伯综合征诊断、临床表现及治疗的重要性。
