Stroke Events and Risk Factors in Older Patients with Moyamoya Disease
Yudai Hirano1, Satoru Miyawaki1, Hideaki Imai2
1Department of Neurosurgery, Faculty of Medicine, The University of Tokyo, Tokyo, Japan.
Background:
We aimed to comprehensively analyze the epidemiology, natural history, stroke events and their risk factors, and the RNF213 p.Arg4810Lys variant in older patients with moyamoya disease (MMD).
Methods:
We enrolled patients with MMD followed-up at our hospital between 2000 and 2023. Those who developed MMD at age ≥60 years or were diagnosed at a younger age and followed-up after age 60 years were included. Baseline characteristics, onset type, radiologic features, and RNF213 p.Arg4810Lys variant status were investigated.
Results:
Among 56 patients with 100 affected hemispheres, 62 were asymptomatic, 26 experienced ischemic onset, and 12 had hemorrhagic onset. A higher incidence of anterior choroidal artery (AchA) dilatation and lower proportion of favorable modified Rankin scale scores were detected in hemorrhagic onset, whereas greater prevalence of bypass surgery in ischemic onset. Of 76 asymptomatic hemispheres at the age of 60 years, subsequent stroke events occurred in 10 hemispheres, comprising 8 hemorrhages and 2 ischemias. Risk factors for de novo hemorrhage in asymptomatic hemispheres included AchA dilatation and choroidal anastomosis. Comparison of the RNF213 p.Arg4810Lys variant status showed no significant differences in baseline characteristics, onset types, or imaging findings, except for a higher percentage of patients in the GA group with a family history of MMD.
Conclusions:
Hemorrhagic events were the most prevalent and prognostically deteriorating factors in older patients with MMD aged ≥60 years. AchA dilatation and choroidal anastomosis were predictors of de novo hemorrhage in asymptomatic nonsurgical hemispheres in older patients with MMD.
Insights
Hemorrhagic events are the main concern for older adults with moyamoya disease (MMD). Anterior choroidal artery dilatation and choroidal anastomosis predict new hemorrhages in asymptomatic cases.
Area of Science:
- Neurology
- Vascular Neurology
- Epidemiology
Background:
- Moyamoya disease (MMD) is a rare cerebrovascular disorder.
- Understanding MMD in older adults (≥60 years) requires specific epidemiological and clinical analysis.
Purpose of the Study:
- To analyze epidemiology, natural history, stroke events, risk factors, and the RNF213 p.Arg4810Lys variant in older MMD patients.
- To identify predictors of stroke events in this demographic.
Main Methods:
- Retrospective analysis of MMD patients (≥60 years) from 2000-2023.
- Investigation of baseline characteristics, onset type, radiologic features, and RNF213 variant status.
- Follow-up of asymptomatic hemispheres to identify subsequent stroke events.
Main Results:
- Among 56 patients, 62 hemispheres were asymptomatic, 26 had ischemic onset, and 12 had hemorrhagic onset.
- Hemorrhagic onset associated with anterior choroidal artery (AchA) dilatation and worse outcomes.
- In asymptomatic hemispheres, 10 experienced subsequent strokes (8 hemorrhagic, 2 ischemic), predicted by AchA dilatation and choroidal anastomosis.
Conclusions:
- Hemorrhagic events are the most prevalent and prognostically significant in older MMD patients (≥60 years).
- AchA dilatation and choroidal anastomosis predict de novo hemorrhage in asymptomatic, nonsurgical hemispheres in older MMD patients.
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