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Moyamoya disease in a primarily white, midwestern US population: increased prevalence of autoimmune disease
Regina S Bower1, Grant W Mallory, Macaulay Nwojo
1Department of Neurosurgery, Mayo Clinic College of Medicine, Rochester, MN, USA.
Background And Purpose:
Moyamoya disease is an uncommon, cerebrovascular occlusive disease of unknown pathogenesis. Previously described Moyamoya cohorts include predominantly Asian populations or ethnically diverse North American cohorts. To gain further insight into the pathogenesis of moyamoya, we examined clinical characteristics of a primarily white, Midwestern US population
Methods:
Retrospective analysis of patients with angiographically confirmed moyamoya disease evaluated at our institution was performed. Prevalence of comorbidities, cerebrovascular risk factors, and autoimmune diseases were compared with the general population.
Results:
Ninety-four patients with moyamoya were evaluated; 72.3% were female. Ethnic composition was primarily white (85%). A significantly higher prevalence of autoimmune disease was seen, particularly type 1 diabetes mellitus (8.5% versus 0.4% in the general population) and thyroid disease (17.0% versus 8.0% in the institutional general patient population). Hyperlipidemia was also increased (27.7% versus 16.3% in the general population).
Conclusions:
This study of a unique, primarily white, Midwestern population of moyamoya patients demonstrates a significantly higher prevalence of autoimmune disease than in the general population. This supports a possible autoimmune component to the pathogenesis of moyamoya disease.
Insights
This study found a higher prevalence of autoimmune diseases, including type 1 diabetes and thyroid disease, in a Midwestern moyamoya disease patient cohort. These findings suggest a potential autoimmune link in moyamoya pathogenesis.
Area of Science:
- Neurology
- Vascular Neurology
- Autoimmune Diseases
Background:
- Moyamoya disease is a rare cerebrovascular occlusive condition with unknown pathogenesis.
- Existing research on moyamoya disease cohorts is predominantly from Asian or ethnically diverse North American populations.
Purpose of the Study:
- To investigate the clinical characteristics of moyamoya disease in a primarily white, Midwestern US population.
- To identify potential insights into the pathogenesis of moyamoya disease.
Main Methods:
- Retrospective analysis of patients with angiographically confirmed moyamoya disease.
- Comparison of comorbidity, cerebrovascular risk factor, and autoimmune disease prevalence with the general population.
Main Results:
- The study evaluated 94 moyamoya patients, predominantly female (72.3%) and white (85%).
- A significantly higher prevalence of autoimmune diseases, including type 1 diabetes mellitus (8.5% vs. 0.4%) and thyroid disease (17.0% vs. 8.0%), was observed compared to the general population.
- Increased prevalence of hyperlipidemia (27.7% vs. 16.3%) was also noted.
Conclusions:
- This study highlights a significantly higher prevalence of autoimmune disease in a unique, primarily white, Midwestern moyamoya patient population.
- The findings support a potential autoimmune component in the pathogenesis of moyamoya disease.
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