Purpose

Moyamoya syndrome (MMS) is a rare intracranial arterial vasculopathy which can occur in neurofibromatosis type 1 (NF1) disease, representing a cause of cerebrovascular reserve (CVR) impairment, possibly leading to ischemic stroke. Here, we evaluated noninvasive imaging techniques used to assess CVR in MMS patients, describing clinical and imaging findings in patients affected by MMS-NF1.

Methods

Following strict inclusion and exclusion criteria, in this retrospective observational study, we evaluated imaging data of nine consecutive MMS-NF1 patients (M/F = 5/4, mean age: 12.6 ± 4.0). Subjects underwent a multimodal evaluation of cerebral vascular status, including intracranial arterial MR Angiography (MRA), MRI perfusion with dynamic susceptibility contrast (DSC) technique, and 99mTc-hexamethylpropyleneamine oxime (HMPAO) SPECT.

Results

In 8 out 9 patients (88.8%, 6/8 symptomatic), time-to-peak maps were correlated with the involved cerebral hemisphere, while in 6 out 9 patients (66.6%, 5/6 symptomatic), mean transit time (MTT) maps showed correspondence with the affected cerebrovascular territories. Cerebral blood flow (CBF) calculated using DSC perfusion failed to detect the hypoperfused regions instead identified by SPECT-CBF in all patients, while MTT maps overlapped with SPECT-CBF data in all cases and time-to-peak maps in 60.0%.

Conclusions

Although SPECT imaging still represents the gold standard for CBF assessment, our results suggest that data obtained using DSC perfusion technique, and in particular MTT maps, might be a very useful and noninvasive tool for evaluating hemodynamic status in MMS-NF1 patients.

中文翻译：

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多模式评估1型Moyamoya综合征神经纤维瘤病患者的脑血管储备。

目的

Moyamoya综合征（MMS）是一种罕见的颅内动脉血管病变，可发生于1型神经纤维瘤病（NF1）疾病中，代表脑血管储备（CVR）受损的原因，可能导致缺血性中风。在这里，我们评估了用于评估MMS患者CVR的非侵入性成像技术，描述了受MMS-NF1影响的患者的临床和影像学发现。

方法

遵循严格的纳入和排除标准，在此回顾性观察研究中，我们评估了9例连续MMS-NF1患者的影像学数据（M / F = 5/4，平均年龄：12.6±4.0）。受试者接受了脑血管状态的多模式评估，包括颅内动脉MR血管造影（MRA），具有动态磁化率对比（DSC）技术的MRI灌注和99mTc-六甲基丙烯胺肟（HMPAO）SPECT。

结果

9名患者中有8名（88.8％，6/8有症状），峰峰值时间与受累脑半球相关，而9名患者中有6名（66.6％，5/6有症状），平均通过时间（MTT） ）地图显示与受影响的脑血管区域相对应。使用DSC灌注计算的脑血流量（CBF）未能在所有患者中检测到SPECT-CBF识别出的灌注不足区域，而在所有情况下MTT图与SPECT-CBF数据重叠，而峰时图则重叠了60.0％。

结论

尽管SPECT成像仍然代表着CBF评估的金标准，但我们的结果表明，使用DSC灌注技术（尤其是MTT图）获得的数据可能是评估MMS-NF1患者血液动力学状态的非常有用且无创的工具。