Abstract

Background and aims: Moyamoya disease refers to a chronic progressive steno-occlusive disease at the distal portion of the internal carotid arteries (ICAs) and compensatory abnormal collateral vessel formation. However, the incidence of progression and its’ related factors in adult moyamoya disease has not been well known. Our study was to investigate the prevalence of progression of steno-occlusion of intracranial vessels and clinical outcome in the patients with adult moyamoya disease. Methods: From Jan 2001 to Jun 2021, we included the patients who was diagnosed with symptomatic or asymptomatic moyamoya disease from moyamoya registries of 4 hospitals in Korea. We excluded patients who did not have any follow-up angiography more than 1 year after baseline angiography [digital subtraction angiography (DSA) or magnetic resonance angiography (MRA)]. We adopted Suzuki’s angiographic staging and MRA score for the analysis. Results: A total of 293 moyamoya patients were included, and mean follow-up period was 92.7 ±52.8 months. Among them, progression of vessel stenosis in DSA and/or MRA was found in 110 patients (37.5%). Recurrent stroke occurred in 39/183 (21.3%) in non-progression group and 40/110 (36.4%) in progression group. Clinical characteristics were not significantly different between two groups. However, previous ischemic stroke history is independently associated with angiographic progression (odds ratio, 3.384 [95% CI, 1.521-7.531]) in multivariable logistic regression analysis. Conclusions: Occlusive arterial status worsened in one-third of the patients with adult moyamoya disease during approximately 8 years of follow-up. Careful neurological and radiological follow-up would be essential to prevent additional stroke occurrence, especially in patients with a history of cerebral infarction.

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