Department of Neurosurgery, Charitè-Universitätsmedizin Berlin, Berlin, Germany.
Klinik für Neurochirurgie mit Arbeitsbereich Pädiatrische Neurochirurgie, Charité - Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Germany.
Acta Neurochir (Wien). 2019 Feb;161(2):371-378. doi: 10.1007/s00701-019-03811-w. Epub 2019 Jan 24.
Moyamoya disease (MMD) is a cerebrovascular disorder characterized by fragile vascular system. Previous studies suggested that the blood-brain barrier (BBB) destabilizing cytokine angiopoietin-2 plays a critical role in increasing vascular plasticity and endothelial disintegration in MMD. The aim of this study was to assess cerebrovascular integrity in vivo in patients affected by MMD.
We retrospectively analyzed 11 patients that underwent bypass for MMD (MMD group), 11 patients that underwent bypass for atherosclerotic cerebrovascular disease (ACVD-control group I), and 5 patients that underwent clipping for unruptured aneurysms (non-ischemic-control group II). Sodium fluorescein (NaFL) extravasation was evaluated during videoangiography when checking for bypass patency. A grading system (0, +, ++, +++) was used to define the extent of extravasation. Frequency and intensity of leakage was compared among different groups.
NaFL extravasation appeared in 10/11 (91%) patients with MMD and in 8/11 (73%) patients with ACVD during bypass procedures. Extravasation was observed in none of the patients undergoing clipping for unruptured aneurysms. Although both chronic ischemic patient groups showed a comparably high incidence of NaFL extravasation, the MMD group was characterized by a much greater intensity of NaFL extravasation (grade +++ in 82%) than the ACVD group (grade +++ in 27%, p < 0.05).
We demonstrate blood-brain barrier impairment in MMD patients for the first time in vivo. This may be due to mechanisms intrinsic to the unique pathology of MMD, probably explaining the higher association with hemorrhage and post-operative hyperperfusion.
烟雾病（MMD）是一种以脆弱的血管系统为特征的脑血管疾病。先前的研究表明，破坏血脑屏障（BBB）的促血管生成素-2 等细胞因子在增加 MMD 患者的血管可塑性和内皮细胞解体方面起着关键作用。本研究旨在评估患有 MMD 的患者的脑血管完整性。
我们回顾性分析了 11 例行 MMD 旁路手术的患者（MMD 组）、11 例行动脉粥样硬化性脑血管病旁路手术的患者（ACVD-对照组 I）和 5 例行未破裂动脉瘤夹闭的患者（非缺血性对照组 II）。当检查旁路通畅性时，通过视频血管造影评估荧光素钠（NaFL）渗漏。使用分级系统（0、+、++、+++）定义渗漏程度。比较不同组之间渗漏的频率和强度。
NaFL 渗漏出现在 11 例 MMD 患者中的 10 例（91%）和 11 例 ACVD 患者中的 8 例（73%）。未破裂动脉瘤夹闭的患者均未观察到 NaFL 渗漏。尽管两组慢性缺血患者的 NaFL 渗漏发生率都很高，但 MMD 组的 NaFL 渗漏强度明显大于 ACVD 组（82%为+++级，27%为+++级，p<0.05）。
我们首次在体内证明了 MMD 患者的血脑屏障受损。这可能是由于 MMD 特有的病理机制所致，这可能解释了与出血和术后过度灌注的更高相关性。
