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Neurology

September 11, 2026 · via Europe PMC

Natural Course and Clinical Outcomes of Patients With Hemorrhagic Moyamoya Disease: A Large Cohort Study.

Chen C, Gao G, Wang H, +9 more, Duan L.

Journal ArticleAbstract only — full text under license

AI summary — for doctors, generated from the abstract only

In a large Chinese hemorrhagic moyamoya cohort, indirect revascularization (EDAS) was associated with less rebleeding and better outcomes than conservative management.

What was studied

A retrospective single-center cohort of 1,050 patients with hemorrhagic moyamoya disease (2002-2022), comparing 927 EDAS-treated with 123 conservatively managed patients using overlap weighting.

Key findings

  • Anterior choroidal artery dilation was associated with rebleeding in natural history (HR 8.64)
  • Female sex, AChA dilation (OR 3.41) and bilateral lesions (OR 5.45) were associated with poor outcomes
  • EDAS associated with lower rebleeding (HR 0.49, 95% CI 0.30-0.81)
  • Some comparisons not fully captured in the abstract

Clinical relevance

Suggests EDAS may benefit hemorrhagic moyamoya, but retrospective single-center data with imbalanced groups require prospective validation.

Who should care

Neurology

Limitations

Retrospective design, small and older natural history group, and potential selection bias despite weighting.

Indirect revascularization may reduce rebleeding in hemorrhagic moyamoya.

This is an AI-generated summary of the published abstract, not a clinical recommendation. It may not reflect the full text or subsequent updates — verify against the original source before acting on it.

Abstract

Background and objectives

Given the natural history of hemorrhagic moyamoya disease (hMMD) is unclear and research on long-term outcomes following indirect revascularization is limited, this study sought to assess its natural course and the efficacy of indirect revascularization.

Methods

We conducted a retrospective analysis of patients with hMMD admitted to the Chinese PLA General Hospital from April 2002 to March 2022. Clinical characteristics, radiologic findings, rebleeding events, and prognostic outcomes were evaluated. Logistic and Cox regression models identified risk factors for poor outcomes and rebleeding in the natural history. After overlap weighting to balance the encephaloduroarteriosynangiosis (EDAS) and conservative groups, weighted regression models were used to compare outcomes.

Results

This study enrolled 1,050 patients with hMMD. The mean age at first bleeding was 37.0 ± 12.3 years, and 577 (55.0%) were female. Among them, 123 were in the natural history group and 927 in the EDAS group. The natural history group had a significantly older mean age (43.7 ± 12.1 vs 36.1 ± 12.1 years, p p = 0.204). In the natural history course, anterior choroidal artery (AChA) dilation (hazard ratio [HR] 8.64, 95% CI 3.03-24.67, p p = 0.017), AChA dilation (OR 3.41, 95% CI 1.32-8.82, p = 0.012), and bilateral lesions (OR 5.45, 95% CI 1.45-20.52, p = 0.012) were associated with poor outcomes in patients with hMMD. After overlap weighting, treatment with EDAS was associated with a lower incidence of rebleeding events (HR 0.49, 95% CI 0.30-0.81, p = 0.006) and improved outcomes (OR 0.85, 95% CI 0.79-0.93, p < 0.001).

Discussion

In conclusion, hMMD occurs mainly in adult patients. In the natural history of hMMD, AChA dilation was associated with rebleeding, while female sex, AChA dilation, and bilateral lesions were associated with poor outcomes. This study suggests that EDAS is associated with a reduced incidence of rebleeding and improved outcomes, indicating that EDAS may be an effective treatment for hMMD. However, given the retrospective design, prospective validation is needed.

DOI

10.1212/wnl.0000000000218493

PMID

42727042

Research metadata (titles, abstracts, authors, publication dates) is sourced from Europe PMC, which aggregates content from PubMed/MEDLINE, PubMed Central, and other providers under the U.S. National Library of Medicine (NLM/NCBI). NLM does not claim copyright on abstracts; the original journal publishers or authors may hold rights to the full articles. AI-generated summaries are derived only from the abstract, never the full text, and are always labeled as AI-generated.

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