Moyamoya angiopathy: radiological follow-up findings in Finnish patients

Moyamoya angiopathy: radiological follow-up findings in Finnish patients
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DOI:
10.1007/s00415-020-09837-w
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发表时间:
2020-04-22
影响因子:
6
通讯作者:
Tatlisumak, Turgut
Tatlisumak, Turgut
中科院分区:
医学2区
文献类型:
--
作者:
Savolainen, Marika;Pekkola, Johanna;Tatlisumak, Turgut

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背景和目的烟雾病血管病(MMA)是一种慢性进行性疾病,但随着时间的推移观察到的影像学变化在欧洲人群中尚未得到表征。我们通过芬兰 MMA 登记处的磁共振成像和血管造影(MRI 和 MRA)分析了 MMA 的进展情况。基于 MRA 结果的分期用于评估疾病的进展。方法对32例MMA患者进行MRI和MRA评估,并与既往影像学检查进行比较。随访影像学检查是在 MMA 诊断后 103(范围 6-380)个月以及上次影像学检查后 64(范围 6-270)个月进行的。我们根据之前描述的 MRA 分级标准对疾病阶段进行分级。结果 与最新可用的先前图像相比,在后续图像中未发现急性病变,包括无症状缺血性中风。一名患者自上次影像检查后出现无症状脑出血。在随访图像中,22% 的患者观察到 Ivy 征。在后续影像学检查中,6% (n = 2) 的患者出现微出血,9% (n = 3) 的患者出现白质病变。根据后续图像评估 MRA 等级,分别为 3 分和 2.5 分(左、右)。百分之五十六 (n = 18) 在随访图像中存在陈旧性缺血性病变。大多数(71%)陈旧性缺血性病变是大的前循环梗塞。结论 MRI/MRA 发现 MMA 相关变化进展缓慢,这与我们之前的报告一致,表明芬兰人群中该疾病的病程相当良性。
Background and purpose Moyamoya angiopathy (MMA) is a chronic progressive disorder, but imaging changes observed over time are not yet characterized in European populations. We analyzed the progression of MMA with magnetic resonance imaging and angiography (MRI and MRA) in our Finnish MMA registry. Stage classification based on MRA findings was used to evaluate the progress of the disease. Methods 32 patients with MMA were evaluated with MRI and MRA and compared to previous imaging. The follow-up imaging was done 103 (range 6-380) months after the MMA diagnosis, and 64 (range 6-270) months after the previous imaging. We graded the disease stage according to the previously described MRA grading scale. Results No acute lesions, including silent ischemic strokes were found in the follow-up image compared to latest available previous image. One patient had an asymptomatic intracerebral hemorrhage since the last imaging. Ivy sign was observed in 22% of the patients in the follow-up image. Six percent (n = 2) had microhemorrhages and 9% (n = 3) white matter lesions in the follow-up imaging. The MRA grade was evaluated from the follow-up images and it was 3 and 2.5 points (right and left, respectively). Fifty-six percent (n = 18) had old ischemic lesions in the follow-up image. Majority (71%) of the old ischemic lesions were large anterior circulation infarcts. Conclusions A slow progression of MMA-related changes on MRI/MRA was found, being in line with our previous reports suggesting a rather benign course of the disease in the Finnish population.