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Systemic vascular changes in spontaneous occlusion of the circle of Willis
1Department of Pathology, Keio University School of Medicine, Tokyo, Japan.
Insights
Moyamoya disease involves systemic factors causing vascular changes in both brain and body vessels. This study found significant thickening in pulmonary, renal, and pancreatic arteries, suggesting a widespread condition.
Area of Science:
- Neurology
- Vascular Biology
- Pathology
Background:
- Moyamoya disease, also known as spontaneous occlusion of the circle of Willis, is characterized by cerebrovascular changes.
- The precise etiologic factors, particularly the involvement of systemic vessels, remain incompletely understood.
Purpose of the Study:
- To investigate systemic etiologic factors contributing to vascular changes in moyamoya disease.
- To determine the involvement of extracranial vessels in addition to intracranial vessels.
Main Methods:
- Histopathologic examination of extracranial vessels from 13 moyamoya disease patients.
- Morphometric analysis of pulmonary, renal, and pancreatic arteries.
Main Results:
- Extracranial vessels showed advanced intimal fibrous thickening, similar to intracranial vessels.
- Pulmonary arteries exhibited characteristic intimal fibrous nodular thickening in some patients.
- Significant intimal thickening was observed in pulmonary, renal, and pancreatic arteries compared to controls.
Conclusions:
- Moyamoya disease likely involves systemic etiologic factors alongside focal factors.
- These systemic factors contribute to vascular changes in both intracranial and extracranial vessels.
- Findings suggest a broader systemic vascular involvement in moyamoya disease.
Background And Purpose:
We examined the presence of systemic etiologic factors causing vascular changes in so-called "spontaneous occlusion of the circle of Willis" (cerebrovascular moyamoya disease) to determine whether extracranial, as well as intracranial, vessels are involved in this disease.
Methods:
Histopathologic examination and morphometric analysis of the extracranial vessels were performed in 13 patients with this disease.
Results:
The histopathologic findings of the extracranial vessels were as follows: 1) advanced intimal fibrous thickening similar to that of the intracranial vessels; and 2) characteristic intimal fibrous nodular thickening, which may indicate organization of mural thrombi, at the proximal portions of the pulmonary arteries in three of 13 patients. Morphometric analysis revealed significant intimal thickening of the pulmonary arteries (p less than 0.05), renal arteries (p less than 0.05), and pancreatic arteries (p less than 0.01) in patients with this disease as compared with age- and sex-matched control patients.
Conclusions:
On the basis of these findings, it is highly likely that this disease has systemic etiologic factors, as well as focal etiologic factors, that work to create vascular change in both the intracranial and extracranial vessels.