Service de Neurologie, CH Jacques Lacarin, France.
Cephalalgia. 2014 Jan;34(1):73-7. doi: 10.1177/0333102413505237. Epub 2013 Sep 17.
Sturge-Weber syndrome (SWS) is an uncommon etiology of hemiplegic migraine-like (HM-like) attacks, associated with epilepsy and mental retardation.
We report the case of a 40-year-old woman with SWS who has been suffering from HM-like episodes since she was 24, with no history of seizure or mental retardation. Susceptibility weighted imaging (SWI)-MRI and CT scans have shown bilateral calcifications of the choroidal plexuses, a developmental venous anomaly with dilated transmedullary veins and a left parieto-occipital leptomeningeal angioma. (18)F-Fluorodeoxyglucose (FDG)-PET/CT revealed a diffuse left-hemisphere hypometabolism. The comparison between the MRI performed at the age of 24 and the one performed at the age of 40 highlighted a progressive unilateral fronto-temporo-parietal atrophy. Surprisingly, even now, cognitive functions of this patient are relatively preserved. Lamotrigine permitted an improvement of HM-like attacks.
Explanations for this minimally symptomatic form of SWS may be the absence of seizure, the importance of her deep venous drainage, the absence of cortical calcification and white matter impairment in the affected hemisphere, and, paradoxically, the severely asymmetric cortical metabolism. Furthermore, this case reinforces the hypothesis that alteration of cerebral hemodynamics could precipitate the cortical spreading depression giving rise to migraine with aura.
We propose to consider SWS as a cause of apparently isolated hemiplegic migraine and lamotrigine as a preventive medication in HM-like attacks.
Sturge-Weber 综合征（SWS）是一种罕见的偏头痛样偏瘫（HM-like）发作的病因，与癫痫和智力迟钝有关。
我们报告了一例 40 岁女性 SWS 患者，自 24 岁以来一直患有 HM-like 发作，无癫痫或智力迟钝病史。磁敏感加权成像（SWI）-MRI 和 CT 扫描显示双侧脉络丛钙化、发育性静脉异常伴扩张的贯穿髓质静脉和左顶枕部软脑膜血管畸形。（18）F-氟脱氧葡萄糖（FDG）-PET/CT 显示左侧半球弥漫性代谢低下。与 24 岁时进行的 MRI 相比，40 岁时进行的 MRI 显示出进行性单侧额颞顶枕叶萎缩。令人惊讶的是，即使到现在，该患者的认知功能仍相对保留。拉莫三嗪改善了 HM-like 发作。
这种症状较轻的 SWS 可能的解释是无癫痫、深部静脉引流的重要性、受累半球无皮质钙化和白质损伤，以及矛盾的是严重的皮质代谢不对称。此外，该病例进一步支持了脑血流动力学改变可能引发皮质扩散性抑制导致偏头痛伴先兆的假说。
我们建议将 SWS 视为偏头痛样偏瘫的一个病因，并将拉莫三嗪作为 HM-like 发作的预防性药物。
