Moyamoya angiopathy: radiological follow-up findings in Finnish patients

• Published in: Journal of neurology Vol. 267; no. 8; pp. 2301 - 2306
• Main Authors: Savolainen, Marika, Pekkola, Johanna, Mustanoja, Satu, Tyni, Tiina, Hernesniemi, Juha, Kivipelto, Leena, Tatlisumak, Turgut
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01.08.2020; Springer Nature B.V
• Subjects: Angiography; asymptomatic cerebral microbleeds; Cerebral microbleeds; clinical-features; disease; Follow-up; Hemorrhage; Ischemia; Ivy sign; Lesions; locations; longitudinal course; Magnetic resonance imaging; Medicine; Medicine & Public Health; Moyamoya angiopathy; MRI; Neurology; Neuroradiology; Neurosciences; Neurosciences & Neurology; Neurovetenskaper; Original Communication; progression; Substantia alba; White matter lesions; White matter lesions; Cerebral microbleeds; Ivy sign; MRI; Moyamoya angiopathy; Follow-up
• DOI: 10.1007/s00415-020-09837-w
• ISSN: 0340-5354

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.