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High-resolution MRI vessel wall enhancement in moyamoya disease: risk factors and clinical outcomes

  • Magnetic Resonance
  • Published:
European Radiology Aims and scope Submit manuscript

Abstract

Objectives

Intracranial vessel wall enhancement (VWE) on high-resolution magnetic resonance imaging (HRMRI) is associated with the progression and poor prognosis of moyamoya disease (MMD). This study assessed potential risk factors for VWE in MMD.

Methods

We evaluated MMD patients using HRMRI and traditional angiography examinations. The participants were divided into VWE and non-VWE groups based on HRMRI. Logistic regression was performed to compare the risk factors for VWE in MMD. The incidence of cerebrovascular events of the different subgroups according to risk factors was compared using Kaplan–Meier survival and Cox regression.

Results

We included 283 MMD patients, 84 of whom had VWE on HRMRI. The VWE group had higher modified Rankin Scale scores at admission (p = 0.014) and a higher incidence of ischaemia and haemorrhage (p = 0.002) than did the non-VWE group. Risk factors for VWE included the ring finger protein 213 (RNF213) p.R4810K variant (odds ratio [OR] 2.01, 95% confidence interval [CI] 1.08–3.76, p = 0.028), hyperhomocysteinaemia (HHcy) (OR 5.08, 95% CI 2.34–11.05, p < 0.001), and smoking history (OR 3.49, 95% CI 1.08–11.31, p = 0.037). During the follow-up of 63.9 ± 13.2 months (median 65 months), 18 recurrent stroke events occurred. Cox regression showed that VWE and the RNF213 p.R4810K variant were risk factors for stroke.

Conclusion

The RNF213 p.R4810K variant is strongly associated with VWE and poor prognosis in MMD. HHcy and smoking are independent risk factors for VWE.

Clinical relevance statement

Vessel wall enhancement in moyamoya disease is closely associated with poor prognosis, especially related to the ring finger protein 213 p.R4810K variant, hyperhomocysteinaemia, and smoking, providing crucial risk assessment information for the clinic.

Key Points

• The baseline presence of vessel wall enhancement is significantly associated with poor prognosis in moyamoya disease.

• The ring finger protein 213 p.R4810K variant is strongly associated with vessel wall enhancement and poor prognosis in moyamoya disease.

• Hyperhomocysteinaemia and smoking are independent risk factors for vessel wall enhancement in moyamoya disease.

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Abbreviations

DSA:

Digital subtraction angiography

Hcy:

Homocysteinaemia

HHcy:

Hyperhomocysteinaemia

HRMRI:

High-resolution magnetic resonance imaging

ICA:

Internal carotid artery

MMD:

Moyamoya disease

MMS:

Moyamoya syndrome

MRI:

Magnetic resonance imaging

mRS:

Modified Rankin Scale

PCA:

Posterior cerebral artery

RNF213:

Ring finger protein 213

SNPs:

Single nucleotide polymorphisms

VWE:

Vessel wall enhancement

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Funding

This study was supported by grants from the National Natural Science Foundation of China (grant numbers 82171280 and 82172021). Science and Technology Commission Project (2019-JCJQ-ZD-195–00).

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Authors

Corresponding authors

Correspondence to Jianming Cai or Lian Duan.

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Guarantor

The scientific guarantor of this publication is Lian Duan.

Conflict of interest

The authors of this manuscript declare no relationships with any companies, whose products or services may be related to the subject matter of the article.

Statistics and biometry

Fangbin Hao kindly provided statistical advice for this manuscript.

Informed consent

Written informed consent was obtained from all subjects or their representatives in this study.

Ethical approval

Institutional Review Board approval was obtained (Research Ethics Committee of the Fifth Medical Center of the PLA General Hospital (reference number, 20150622)).

Study subjects or cohorts overlap

No study participants have been previously reported.

The manuscript has a pre-print: https://doi.org/10.1101/2023.05.20.23290282.

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Hao, F., Han, C., Lu, M. et al. High-resolution MRI vessel wall enhancement in moyamoya disease: risk factors and clinical outcomes. Eur Radiol (2024). https://doi.org/10.1007/s00330-023-10535-0

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  • DOI: https://doi.org/10.1007/s00330-023-10535-0

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