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Pathology of so-called "spontaneous occlusion of the circle of Willis"
Insights
Moyamoya disease involves abnormal cerebral vascular networks due to internal carotid artery obstruction. Spontaneous occlusion of the circle of Willis, a subset of Moyamoya syndrome, affects children with unknown causes, requiring further research.
Area of Science:
- Neurology
- Vascular Medicine
- Pediatrics
Background:
- Moyamoya disease presents as abnormal cerebral vascular networks, often linked to internal carotid artery obstruction.
- Collateral circulation visualized on angiograms indicates chronic blockage of major cerebral arteries.
- A subset of cases, particularly in young children with unknown etiology, involves bilateral spontaneous occlusion of the circle of Willis.
Purpose of the Study:
- To define and categorize cases of spontaneous occlusion of the circle of Willis.
- To differentiate these cases from other causes of Moyamoya syndrome.
- To highlight the need for further research into the etiology of this specific condition.
Main Methods:
- Cerebral angiogram analysis to identify abnormal vascular networks.
- Exclusion of cases with known underlying etiological factors.
- Clinical case review focusing on age of onset and bilateral internal carotid artery involvement.
Main Results:
- Abnormal cerebral vascular networks are recognized as collaterals from chronic internal carotid artery obstruction.
- A significant number of cases with bilateral, spontaneous internal carotid artery occlusion in early childhood remain of unknown etiology.
- These idiopathic cases are classified as spontaneous occlusion of the circle of Willis, a component of Moyamoya syndrome.
Conclusions:
- Spontaneous occlusion of the circle of Willis represents a distinct entity within the spectrum of Moyamoya syndrome.
- The unknown etiology in these pediatric cases necessitates further investigation.
- Understanding the precise cause of spontaneous occlusion of the circle of Willis is crucial for potential therapeutic strategies.
Abstract:
Abnormal cerebral vascular networks (Moyamoya blood vessels) are now recognized on cerebral angiograms as collaterals caused by chronic obstruction of the internal carotid arteries due to various conditions. After exclusion of cases in which the etiology is clear, there still remain a considerable number of cases characterized by bilateral involvement of the terminal portions of the internal carotid arteries, and by onset at early childhood due to unknown etiology. These cases should be called "spontaneous occlusion of the circle of Willis." This entity is a part of the "Moyamoya" syndrome. Further investigation is necessary to elucidate the exact cause of this entity.