J Stroke.  2016 Jan;18(1):12-20. 10.5853/jos.2015.01760.

The Pathophysiology of Moyamoya Disease: An Update

• Bang OY
• Fujimura M
• Kim SK

• KMID: 2223631
• DOI: http://doi.org/10.5853/jos.2015.01760

Abstract

Moyamoya disease (MMD) is a unique cerebrovascular disease characterized by the progressive stenosis of large intracranial arteries and a hazy network of basal collaterals called moyamoya vessels. Because the etiology of MMD is unknown, its diagnosis is based on characteristic angiographic findings. Re-vascularization techniques (e.g., bypass surgery) are used to restore perfusion, and are the primary treatment for MMD. There is no specific treatment to prevent MMD progression. This review summarizes the recent advances in MMD pathophysiology, including the genetic and circulating factors related to disease development. Genetic and environmental factors may play important roles in the development of the vascular stenosis and aberrant angiogenesis in complex ways. These factors include the related changes in circulating endothelial/smooth muscle progenitor cells, cytokines related to vascular remodeling and angiogenesis, and endothelium, such as caveolin which is a plasma membrane protein. With a better understanding of MMD pathophysiology, nonsurgical approaches targeting MMD pathogenesis may be available to stop or slow the progression of this disease. The possible strategies include targeting growth factors, retinoic acid, caveolin-1, and stem cells.

Keyword

Angiogenesis; Caveolin; Endothelial progenitor cells; Growth factors; Moyamoya disease

MeSH Terms

Arteries
Caveolin 1
Cell Membrane
Constriction, Pathologic
Cytokines
Diagnosis
Endothelium
Intercellular Signaling Peptides and Proteins
Moyamoya Disease*
Perfusion
Stem Cells
Tretinoin
Caveolin 1
Cytokines
Intercellular Signaling Peptides and Proteins
Tretinoin