INTRODUCTION Sturge-Weber syndrome (SWS) is a neurocutaneous syndrome with typical clinical features including seizures, chronic hemiplegia, hemianopsia and intellectual impairment. Progressive clinical decline may be attributable, at least in part, to progressive venous ischemia. Transcranial Doppler (TCD) ultrasonography could be useful to monitor the degree of hemodynamic involvement and its progression. PURPOSE To determine whether there is an association between the degree of asymmetry in TCD and intensity of clinical and radiological involvement and whether there is a correlation between clinical changes and changes in serial TCD. METHODS In fourteen SWS pediatric patients and two "possible cases" (infants younger than two years old without previously known brain involvement, but with other typical signs of SWS) mean flow velocity in the middle cerebral arteries (MCA) was measured by TCD in both hemispheres. The percent difference between hemispheres (asymmetry) was calculated. Clinical and radiological severity was scored using scales. The correlation between TCD asymmetry and SWS clinical and radiological scores was analyzed at baseline, as well as the correlation between the changes in the different variables (TCD asymmetry, clinical and radiological cores) during evolution and in relation to the changes due to therapy. RESULTS The percentage of MCA velocity asymmetry was positively correlated with the clinical severity score (p = 0.04), and with seizure frequency (p = 0.014). Throughout evolution, therapeutic and clinical changes were associated with noticeable changes in transcranial doppler asymmetry in some cases. CONCLUSIONS TCD may provide a noninvasive method to assess the severity of blood flow abnormalities at baseline and a method to monitor children for progressive changes over time.

中文翻译：

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经颅多普勒在 Sturge-Weber 综合征患儿评估和随访中的应用

简介 Sturge-Weber 综合征 (SWS) 是一种神经皮肤综合征，具有典型的临床特征，包括癫痫发作、慢性偏瘫、偏盲和智力障碍。进行性临床下降可能至少部分归因于进行性静脉缺血。经颅多普勒 (TCD) 超声检查可用于监测血流动力学受累程度及其进展。目的 确定 TCD 的不对称程度与临床和放射学受累的强度之间是否存在关联，以及临床变化与系列 TCD 的变化之间是否存在相关性。方法 在 14 名 SWS 儿科患者和两个“可能的病例”（2 岁以下没有先前已知大脑受累的婴儿，但有其他典型的 SWS 迹象）大脑中动脉 (MCA) 的平均流速是通过两个半球的 TCD 测量的。计算半球之间的百分比差异（不对称）。使用量表对临床和放射学严重程度进行评分。在基线时分析了 TCD 不对称性与 SWS 临床和放射学评分之间的相关性，以及进化过程中不同变量（TCD 不对称性、临床和放射学核心）的变化与治疗引起的变化之间的相关性。结果 MCA 速度不对称的百分比与临床严重程度评分 (p = 0.04) 和癫痫发作频率 (p = 0.014) 呈正相关。在整个进化过程中，在某些情况下，治疗和临床变化与经颅多普勒不对称的显着变化有关。结论 TCD 可以提供一种无创方法来评估基线血流异常的严重程度，并提供一种方法来监测儿童随时间的渐进性变化。