Additional value of intracranial vessel wall MRI as an adjunct to MRA in further differentiation of Moyamoya vasculopathy in Malaysia

Authors

  • Professor Dr Kartini Rahmat Department of Biomedical Imaging, Faculty of Medicine, Universiti Malaya, 50603, Kuala Lumpur, Malaysia
  • Dr Lim Yi Ting Department of Biomedical Imaging, Faculty of Medicine, Universiti Malaya, 50603, Kuala Lumpur, Malaysia
  • Dr Farah Diana Tarmizi Thayaparan Department of Biomedical Imaging, Faculty of Medicine, Universiti Malaya, 50603, Kuala Lumpur, Malaysia
  • Dr Nadia Fareeda Mohd Gowdh Department of Biomedical Imaging, Faculty of Medicine, Universiti Malaya, 50603, Kuala Lumpur, Malaysia
  • Associate Professor Dr Jeannie Wong Hsiu Ding Department of Biomedical Imaging, Faculty of Medicine, Universiti Malaya, 50603, Kuala Lumpur, Malaysia
  • Professor Dr Norlisah Ramli Department of Biomedical Imaging, Faculty of Medicine, Universiti Malaya, 50603, Kuala Lumpur, Malaysia
  • Professor Dr Tan Kay Sin Division of Neurology, Department of Medicine, Faculty of Medicine, Universiti Malaya, Kuala Lumpur, Malaysia
  • Associate Professor Dr Khairul Azmi Abd Kadir Department of Biomedical Imaging, Faculty of Medicine, Universiti Malaya

DOI:

https://doi.org/10.54029/2025nyf

Keywords:

Moyamoya disease, Moyamoya syndrome, Vessel wall imaging, luminal imaging, diagnostic accuracy

Abstract

Background & Objective: Moyamoya vasculopathy (MMV) is characterised by the progressive occlusion of the distal internal cerebral arteries (ICA) and its terminal branches, classified into Moyamoya disease (MMD) and Moyamoya syndrome (MMS). Differentiating between MMD and MMS is crucial for appropriate treatment planning. While luminal imaging aids in diagnosis, distinguishing features can sometimes be subtle. This study evaluates the additional diagnostic value of vessel wall magnetic resonance imaging (MRI) in differentiating MMD from MMS.

Methods: Patients with clinical and imaging suspicious features of MMV underwent MRI, including additional vessel wall imaging (VWI). Two neuroradiologists initially reviewed the luminal imaging for features of MMV, and a presumed diagnosis was made in consensus. These luminal images were then correlated with clinical data to classify cases as MMD or MMS, serving as the gold standard diagnosis in this study. Subsequently, the VWI and luminal images were reviewed together, blinded to the initial luminal diagnoses and clinical data, to assess diagnostic accuracy by comparing both methods. The imaging parameters analysed included vessel wall thickening, grading, pattern of wall enhancement, and degree of collateral vessel formation.

Results: Eighteen patients were analysed, comprising 12 with MMD and 6 with MMS. 108 vessels were evaluated, showing improved diagnostic accuracy from 55.6% to 77.8% when combining luminal and VWI. Specificity increased from 16% to 83%, with the positive predictive value rising to 90% and the negative predictive value to 62%. Our study found that MMD and MMS predominantly manifested concentric wall thickening, with no significant difference between the two groups. However, a significant difference was observed in the grading of wall enhancement, as most of the lesions in MMD showed no enhancement, whereas MMS lesions mostly exhibited moderate enhancement.

Conclusion: VWI demonstrates promise as a valuable modality for distinguishing between MMD and MMS when integrated with conventional imaging techniques.

Published

2025-04-01

Issue

Vol. 30 No. 1 (2025)

Section

Original Article