Adamsbaum C, Pinton F, Rolland Y, Chiron C, Dulac O, Kalifa G
Service de Radiologie, Hôpital St. Vincent de Paul, F-75674 Paris Cedex 14, France.
Pediatr Radiol. 1996 Nov;26(11):759-62. doi: 10.1007/BF01396195.
The prognosis of Sturge-Weber syndrome (SWS) is partly related to early occurrence of seizures but the diagnosis of this phakomatosis may be difficult during the 1st year of life. We have performed a retrospective study of seven patients with confirmed SWS (age 7 days to 3 months). None of the patients was asymptomatic at the time of the study. They all underwent MRI (T1 and T2 sequences) and single photon emission computed tomography (SPECT) at the same time. Regional cerebral blood flow was measured using xenon-133. In all cases, myelination appeared to be accelerated in the areas underlying the leptomeningeal angioma on both MRI sequences. In five cases, SPECT showed hyperperfusion in the damaged hemisphere. In one case, the SPECT was symmetrical and in another it showed hypoperfusion in the damaged hemisphere which was already atrophied. These data suggest that the accelerated myelination is not related to ischemia but to transient hyperperfusion. This MRI pattern can be helpful for the early diagnosis of SWS, which is of utmost importance for preventive antiepileptic treatment.
斯特奇-韦伯综合征（SWS）的预后部分与癫痫发作的早期发生有关，但这种错构瘤病在生命的第一年可能难以诊断。我们对7例确诊为SWS的患者（年龄7天至3个月）进行了回顾性研究。在研究时，没有患者无症状。他们都同时接受了MRI（T1和T2序列）和单光子发射计算机断层扫描（SPECT）。使用氙-133测量局部脑血流量。在所有病例中，在两个MRI序列上，软脑膜血管瘤下方区域的髓鞘形成似乎都加速了。在5例中，SPECT显示受损半球血流灌注增加。在1例中，SPECT是对称的，在另一例中，它显示受损半球（已萎缩）血流灌注减少。这些数据表明，髓鞘形成加速与缺血无关，而是与短暂的血流灌注增加有关。这种MRI模式有助于SWS的早期诊断，这对预防性抗癫痫治疗至关重要。
