Clinical and radiological course of asymptomatic and hemodynamically stable moyamoya disease: a systematic review and
Yang Chen1,2,3,4, Zixuan Zhou2,3,4,5, Bingyang Qin1,2,3,4
1Department of Neurosurgery, First Hospital of Shanxi Medical University, Taiyuan, China.
Background:
Moyamoya disease (MMD) is an idiopathic, chronic intracranial vascular stenosis and occlusion disease. However, there is currently a lack of comprehensive analysis on the clinical and radiological course of asymptomatic MMD (AMMD) and hemodynamically stable MMD (HSMMD).
Data Source:
We conducted a comprehensive literature search using major bibliographic indexing databases, including Embase, Medline, PubMed, Web of Science, and Cochrane Library.
Methods:
This systematic review was conducted based on the PRISMA guidelines. The quality of the included studies was accessed using the Methodological Index for Non-Randomized Studies (MINORS). Effect sizes were pooled with a random-effects model. Heterogeneity between studies was estimated via the I2 test. Publication bias was assessed with Egger's test. The registration code is CRD42023444432.
Result:
A total of seven AMMD studies were included in a meta-analysis, involving 393 patients and 649 hemispheres. Three HSMMD studies were all from the same institution. The pooled rate for clinical progression, hemorrhagic stroke, ischemic stroke, transient ischemic attack (TIA), and radiological progress of conservative group was 10% (95% CI 4.9-15.1%), 3.8% (95% CI 0.4-7.2%), 0.7% (95% CI 0-2.3%), 3.6% (95% CI 0.6-6.6%), and 15.6% (95% CI 10.2-22.1%), respectively. The pooled rate for stroke, TIA, and radiological progress of the surgical group was 3.7% (95% CI 0-10.8%), 0.2% (95% CI 0-3.0%), and 4.8% (95% CI 0-10.5%), respectively. Revascularization did not show a protective effect on TIA and radiological progression for AMMD.
Conclusion:
AMMD and HSMMD present a concerning risk of clinical and radiological progression over a follow-up period of more than 2 years. Further high-quality studies are needed to optimize treatment strategies.
Systematic Review Registration:
https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=444432, CRD42023444432.
Insights
Asymptomatic and hemodynamically stable Moyamoya disease (MMD) patients face significant risks of clinical and radiological progression. Revascularization offers no clear benefit for transient ischemic attack or radiological progression in asymptomatic MMD.
Area of Science:
- Neurology
- Vascular Neurology
- Neuroscience
Background:
- Moyamoya disease (MMD) is a chronic intracranial vascular stenosis and occlusion condition.
- Comprehensive analysis of asymptomatic MMD (AMMD) and hemodynamically stable MMD (HSMMD) clinical and radiological courses is lacking.
Purpose of the Study:
- To systematically review and analyze the clinical and radiological progression of asymptomatic Moyamoya disease (AMMD) and hemodynamically stable Moyamoya disease (HSMMD).
- To evaluate the effectiveness of revascularization in AMMD.
Main Methods:
- Systematic review and meta-analysis adhering to PRISMA guidelines.
- Literature search across Embase, Medline, PubMed, Web of Science, and Cochrane Library.
- Quality assessment using Methodological Index for Non-Randomized Studies (MINORS) and random-effects modeling for pooled effect sizes.
Main Results:
- Seven AMMD studies (393 patients) and three HSMMD studies were included.
- Conservative management in AMMD showed pooled rates of 10% for clinical progression, 3.8% for hemorrhagic stroke, 0.7% for ischemic stroke, 3.6% for TIA, and 15.6% for radiological progression.
- Surgical management in HSMMD showed pooled rates of 3.7% for stroke, 0.2% for TIA, and 4.8% for radiological progression. Revascularization did not demonstrate a protective effect on TIA or radiological progression in AMMD.
Conclusions:
- AMMD and HSMMD carry a significant risk of clinical and radiological progression over two years.
- Current evidence suggests revascularization may not offer protective benefits for TIA and radiological progression in AMMD.
- Further high-quality research is essential to refine treatment strategies for these MMD subtypes.
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