Abstract
Background and purposeMoyamoya 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.Methods32 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.ResultsNo 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.ConclusionsA 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.
Highlights
Moyamoya disease (MMD) and moyamoya syndrome (MMS), referred as the moyamoya angiopathy (MMA), is a chronic progressive steno-occlusive angiopathy at the distal portions of internal carotid arteries (ICAs) and their proximal branches, with typical collateral artery formation, the moyamoya vessels
We evaluated the presence of ivy sign, cerebral microbleeds (CMBs), pattern of ischemic lesions, and presence of white matter lesions (WMLs), and occurrence of new silent or overt ischemic or hemorrhagic lesions since previous imaging
In line with few clinical events, new ischemic or hemorrhagic lesions were found in one patient only, and all the unilateral cases remained unilateral
Summary
Moyamoya disease (MMD) and moyamoya syndrome (MMS), referred as the moyamoya angiopathy (MMA), is a chronic progressive steno-occlusive angiopathy at the distal portions of internal carotid arteries (ICAs) and their proximal branches, with typical collateral artery formation, the moyamoya vessels. We accomplished follow-up imaging with MRI and MRA in 32 Finnish MMA patients to evaluate silent/ asymptomatic progression of the angiopathy, the occurrence of ivy sign, CMBs and WMLs, silent or overt infarcts and intracerebral hemorrhages, and to grade the disease stage. 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. 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
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