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烟雾病:芬兰患者的影像学随访结果。

Moyamoya angiopathy: radiological follow-up findings in Finnish patients.

机构信息

Department of Neurology, South Karelia Central Hospital, Valto Käkelän katu 1, 53130, Lappeenranta, Finland.

Clinical Neurosciences, Neurology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.

出版信息

J Neurol. 2020 Aug;267(8):2301-2306. doi: 10.1007/s00415-020-09837-w. Epub 2020 Apr 22.

Abstract

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.

摘要

背景与目的

烟雾病(Moyamoya angiopathy,MMA)是一种慢性进行性疾病,但目前尚未对欧洲人群的随时间推移而观察到的影像学变化进行特征描述。我们在芬兰的 MMA 登记处通过磁共振成像(magnetic resonance imaging,MRI)和血管造影(angiography,MRA)分析了 MMA 的进展。基于 MRA 结果的分期分类用于评估疾病的进展。

方法

32 例 MMA 患者接受了 MRI 和 MRA 评估,并与之前的影像学结果进行了比较。随访影像学检查在 MMA 诊断后 103 个月(范围 6-380 个月),上次影像学检查后 64 个月(范围 6-270 个月)进行。我们根据之前描述的 MRA 分级量表对疾病阶段进行分级。

结果

与最近一次可用的上一次影像学检查相比,在随访图像中未发现急性病变,包括无症状性缺血性卒中。1 例患者在最后一次影像学检查后发生无症状性颅内出血。在随访图像中,22%的患者观察到 Ivy 征。6%(n=2)的患者在随访图像中出现微出血,9%(n=3)出现脑白质病变。根据随访图像评估了 MRA 分级,右侧和左侧分别为 3 分和 2.5 分。56%(n=18)的患者在随访图像中出现陈旧性缺血性病变。大多数(71%)陈旧性缺血性病变为大的前循环梗死。

结论

MRI/MRA 上 MMA 相关变化的进展缓慢,与我们之前的报告一致,表明芬兰人群中该疾病的病程相对良性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c1b8/7358936/7eca3aec6bc1/415_2020_9837_Fig1_HTML.jpg

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