Pathophysiology of Vascular Stenosis and Remodeling in Moyamoya Disease

Moyamoya disease (MMD) and moyamoya syndrome (MMS) are progressive vascular pathologies unique to the cerebrovasculature that are important causes of stroke in both children and adults. The natural history of MMD is characterized by primary progressive stenosis of the supraclinoid internal carotid a...

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Autores principales:	Brandon M. Fox, Kirsten B. Dorschel, Michael T. Lawton, John E. Wanebo
Formato:	article
Lenguaje:	EN
Publicado:	Frontiers Media S.A. 2021
Materias:	angiopathy cerebrovascular moyamoya stenosis stroke Neurology. Diseases of the nervous system RC346-429
Acceso en línea:	https://doaj.org/article/642bfd1796474753b1d005dab2a0ced0
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spelling	oai:doaj.org-article:642bfd1796474753b1d005dab2a0ced02021-12-01T08:41:19ZPathophysiology of Vascular Stenosis and Remodeling in Moyamoya Disease1664-229510.3389/fneur.2021.661578https://doaj.org/article/642bfd1796474753b1d005dab2a0ced02021-09-01T00:00:00Zhttps://www.frontiersin.org/articles/10.3389/fneur.2021.661578/fullhttps://doaj.org/toc/1664-2295Moyamoya disease (MMD) and moyamoya syndrome (MMS) are progressive vascular pathologies unique to the cerebrovasculature that are important causes of stroke in both children and adults. The natural history of MMD is characterized by primary progressive stenosis of the supraclinoid internal carotid artery, followed by the formation of fragile collateral vascular networks. In MMS, stenosis and collateralization occur in patients with an associated disease or condition. The pathological features of the stenosis associated with MMD include neointimal hyperplasia, disruption of the internal elastic lamina, and medial attenuation, which ultimately lead to progressive decreases in both luminal and external arterial diameter. Several molecular pathways have been implicated in the pathophysiology of stenosis in MMD with functions in cellular proliferation and migration, extracellular matrix remodeling, apoptosis, and vascular inflammation. Importantly, several of these molecular pathways overlap with those known to contribute to diseases of systemic arterial stenosis, such as atherosclerosis and fibromuscular dysplasia (FMD). Despite these possible shared mechanisms of stenosis, the contrast of MMD with other stenotic pathologies highlights the central questions underlying its pathogenesis. These questions include why the stenosis that is associated with MMD occurs in such a specific and limited anatomic location and what process initiates this stenosis. Further investigation of these questions is critical to developing an understanding of MMD that may lead to disease-modifying medical therapies. This review may be of interest to scientists, neurosurgeons, and neurologists involved in both moyamoya research and treatment and provides a review of pathophysiologic processes relevant to diseases of arterial stenosis on a broader scale.Brandon M. FoxKirsten B. DorschelMichael T. LawtonJohn E. WaneboFrontiers Media S.A.articleangiopathycerebrovascularmoyamoyastenosisstrokeNeurology. Diseases of the nervous systemRC346-429ENFrontiers in Neurology, Vol 12 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	angiopathy cerebrovascular moyamoya stenosis stroke Neurology. Diseases of the nervous system RC346-429
spellingShingle	angiopathy cerebrovascular moyamoya stenosis stroke Neurology. Diseases of the nervous system RC346-429 Brandon M. Fox Kirsten B. Dorschel Michael T. Lawton John E. Wanebo Pathophysiology of Vascular Stenosis and Remodeling in Moyamoya Disease
description	Moyamoya disease (MMD) and moyamoya syndrome (MMS) are progressive vascular pathologies unique to the cerebrovasculature that are important causes of stroke in both children and adults. The natural history of MMD is characterized by primary progressive stenosis of the supraclinoid internal carotid artery, followed by the formation of fragile collateral vascular networks. In MMS, stenosis and collateralization occur in patients with an associated disease or condition. The pathological features of the stenosis associated with MMD include neointimal hyperplasia, disruption of the internal elastic lamina, and medial attenuation, which ultimately lead to progressive decreases in both luminal and external arterial diameter. Several molecular pathways have been implicated in the pathophysiology of stenosis in MMD with functions in cellular proliferation and migration, extracellular matrix remodeling, apoptosis, and vascular inflammation. Importantly, several of these molecular pathways overlap with those known to contribute to diseases of systemic arterial stenosis, such as atherosclerosis and fibromuscular dysplasia (FMD). Despite these possible shared mechanisms of stenosis, the contrast of MMD with other stenotic pathologies highlights the central questions underlying its pathogenesis. These questions include why the stenosis that is associated with MMD occurs in such a specific and limited anatomic location and what process initiates this stenosis. Further investigation of these questions is critical to developing an understanding of MMD that may lead to disease-modifying medical therapies. This review may be of interest to scientists, neurosurgeons, and neurologists involved in both moyamoya research and treatment and provides a review of pathophysiologic processes relevant to diseases of arterial stenosis on a broader scale.
format	article
author	Brandon M. Fox Kirsten B. Dorschel Michael T. Lawton John E. Wanebo
author_facet	Brandon M. Fox Kirsten B. Dorschel Michael T. Lawton John E. Wanebo
author_sort	Brandon M. Fox
title	Pathophysiology of Vascular Stenosis and Remodeling in Moyamoya Disease
title_short	Pathophysiology of Vascular Stenosis and Remodeling in Moyamoya Disease
title_full	Pathophysiology of Vascular Stenosis and Remodeling in Moyamoya Disease
title_fullStr	Pathophysiology of Vascular Stenosis and Remodeling in Moyamoya Disease
title_full_unstemmed	Pathophysiology of Vascular Stenosis and Remodeling in Moyamoya Disease
title_sort	pathophysiology of vascular stenosis and remodeling in moyamoya disease
publisher	Frontiers Media S.A.
publishDate	2021
url	https://doaj.org/article/642bfd1796474753b1d005dab2a0ced0
work_keys_str_mv	AT brandonmfox pathophysiologyofvascularstenosisandremodelinginmoyamoyadisease AT kirstenbdorschel pathophysiologyofvascularstenosisandremodelinginmoyamoyadisease AT michaeltlawton pathophysiologyofvascularstenosisandremodelinginmoyamoyadisease AT johnewanebo pathophysiologyofvascularstenosisandremodelinginmoyamoyadisease
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Pathophysiology of Vascular Stenosis and Remodeling in Moyamoya Disease

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