Accelerated myelination in early Sturge-Weber syndrome: MRI-SPECT correlations
Accelerated myelination in early Sturge-Weber syndrome: MRI-SPECT correlations
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DOI：
10.1007/bf01396195
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发表时间：
1996-11-01
影响因子：
2.3
通讯作者：
Kalifa, G
中科院分区：
文献类型：
--
作者：
Adamsbaum, C;Pinton, E;Kalifa, G
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，但在生命的第一年诊断这种肉瘤病可能很困难。我们对7例确诊SWS患者（年龄7天至3个月）进行了回顾性研究。在研究时，没有患者是无症状的。同时行MRI （T1和T2序列）和单光子发射计算机断层扫描（SPECT）。用氙-133测定局部脑血流量。在所有病例中，在两个MRI序列上，髓鞘形成似乎在脑膜轻脑膜血管瘤下的区域加速。在5例中，SPECT显示受损半球过度灌注。在一个病例中，SPECT是对称的，而在另一个病例中，它显示受损半球已经萎缩的灌注不足。这些数据表明加速髓鞘形成与缺血无关，而与短暂性高灌注有关。该MRI模式有助于SWS的早期诊断，对预防性抗癫痫治疗具有重要意义。
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 Ist 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.