Vessel Wall Changes on Serial High-Resolution MRI and the Use of Cilostazol in Patients With Adult-Onset Moyamoya Disease

Abstract

Background and Purpose The natural course of adult-onset moyamoya disease (MMD) is unknown, and there is no medical treatment that halts its progression. We hypothesized that progressive shrinkage of large intracranial arteries occurs in adult-onset MMD, and that cilostazol inhibits this process. Methods Serial high-resolution magnetic resonance imaging (HR-MRI) was performed on 66 patients with MMD: 30 patients received cilostazol, 21 received other antiplatelets, and 15 received no antiplatelets or had poor compliance to them. Serial HR-MRI was performed (interval between MRI scans: 29.67±18.02 months, mean±SD), and changes in outer diameter, luminal stenosis, and vascular enhancement were measured. Factors affecting HR-MRI changes were evaluated, including vascular risk factors and the ring finger protein 213 gene variant. Results The progression of stenosis to occlusion, recurrent ischemic stroke, and the development of new stenotic segments were observed in seven, seven, and three patients, respectively. Serial HR-MRI indicated that the degree of stenosis increased with negative remodeling (outer diameter shrinkage). Patients who received cilostazol presented significantly larger outer diameters and lower degrees of stenosis compared with other groups ( p =0.005 and p =0.031, respectively). After adjusting for clinical and genetic factors, only cilostazol use was independently associated with negative remodeling (odds ratio=0.29, 95% confidence interval=0.10–0.84, p =0.023). While vascular enhancement was observed in most patients (61 patients), the progression of enhancement or the occurrence of new vascular enhancement was rarely observed on follow-up HR-MRI (6 and 1 patients, respectively). Conclusions Adult-onset MMD induces progressive shrinkage of large intracranial arteries, which cilostazol treatment may prevent. Further randomized clinical trials are warranted. Trial registration ClinicalTrials.gov identifier NCT02074111 .

Emerging Concept of Intracranial Arterial Diseases: The Role of High Resolution Vessel Wall MRI

“…On HR-VWI, MMD is characterized by strong enhancement in the distal ICA and extension into the proximal M1 or A1 [ 56 ]. In general, it exhibits concentric enhancement.…”

“…This finding provides insights into deciding whether to discontinue antiplatelet therapy, especially in young patients, who are more susceptible to intracranial dissections. In MMD, a study showed that the progression of negative remodeling on follow-up HR-VWI was slower when cilostazol was administered than when other antiplatelet agents were used [ 56 ]. Another study showed that rapid progression of arterial stenosis increased the risk of recurrent stroke in patients with MMD [ 67 ].…”

“…For RCVS, a 12-week follow-up would be reasonable in line with the diagnostic criteria [ 70 ]. MMD can be monitored for approximately 24 months to assess progression unless otherwise indicated [ 56 ]. For vasculitis, imaging should be performed when critical treatment decisions are pending and when a clinical–radiological correlation is needed.…”

High-resolution magnetic resonance vessel wall imaging provides new insights into Moyamoya disease

“…For 3.0 T MR head imaging, there are several receiving coils, including 8, 16, and 32-channel head coils, or 16, 20, 24, and 64-channel high-resolution coils for imaging the head and neck. Presently, the 32-channel high-resolution coil is commonly used ( Kim et al, 2022 ; Liang et al, 2023 ). Studies have shown that, compared with 8-the channel head coil, the 32-channel head coil can increase SNR up to 3.4 times in the cortex and 1.4 times in the corpus callosum ( Wiggins et al, 2006 ); Compared with the 12-channel head coil, the 32-channel head coil increased the SNR in the middle of the brain by about 1.5 times ( Wiggins et al, 2006 ).…”

“…Subsequent studies using HR-VWI were able to demonstrate the specific mechanism played by cilostazol in patients with MMD. It was demonstrated that cilostazol resulted in a significant increase in the outer diameter of the diseased vessel, a significant reduction in the degree of stenosis, and positive remodeling ( Kim et al, 2022 ). This is also the only study that used HR-VWI to analyze medical interventions for MMD.…”