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Moyamoya disease and syndrome: a review
Zeferino Demartini1,2,3, Bernardo Ca Teixeira1,2, Gelson Luis Koppe2,3
1Complexo Hospital de Clínicas - Universidade Federal do Paraná (UFPR), Curitiba, PR, Brazil.
Insights
Moyamoya disease is a rare cerebrovascular condition causing progressive artery narrowing. Surgical revascularization offers a promising treatment to prevent strokes and improve patient quality of life.
Area of Science:
- Neurology
- Vascular Biology
- Medical Imaging
Background:
- Moyamoya disease is a chronic, non-inflammatory cerebrovascular condition.
- Characterized by progressive stenosis of intracranial carotid arteries and branches.
- Leads to ischemic or hemorrhagic strokes with significant morbidity and mortality.
Purpose of the Study:
- To review recent advances in understanding Moyamoya disease.
- To cover epidemiology, etiology, clinical presentation, and diagnostic imaging.
- To discuss current and emerging treatment strategies, including surgical revascularization.
Main Methods:
- Literature review of recent studies on Moyamoya disease.
- Analysis of epidemiological data, etiological factors, and clinical manifestations.
- Evaluation of diagnostic imaging techniques and therapeutic interventions.
Main Results:
- Moyamoya disease involves endothelial hyperplasia and fibrosis.
- Cerebral angiography reveals characteristic "moyamoya" collateral vessels.
- Surgical revascularization shows potential in preventing ischemic events.
Conclusions:
- Moyamoya disease requires comprehensive understanding for effective management.
- Advances in imaging enhance diagnostic accuracy.
- Surgical revascularization is a key strategy for improving patient outcomes and quality of life.
Abstract:
Moyamoya disease is a chronic occlusive cerebrovascular disease that is non-inflammatory and non-atherosclerotic. It is characterized by endothelial hyperplasia and fibrosis of the intracranial portion of the carotid artery and its proximal branches, leading to progressive stenosis and occlusion, often clinically manifesting as ischemic or hemorrhagic stroke with high rates of morbidity and mortality. On cerebral angiography, the formation of collateral vessels has the appearance of a puff of smoke (moyamoya in Japanese), which became more conspicuous with the refinement of modern imaging techniques. When there is associated disease, it is known as moyamoya syndrome. Treatments are currently limited, although surgical revascularization may prevent ischemic events and preserve quality of life. In this review, we summarize recent advances in moyamoya disease, covering aspects of epidemiology, etiology, presentation, imaging, and treatment strategies.
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