The Progression of Pathophysiology of Moyamoya Disease

Abstract

Moyamoya disease (MMD) is a chronic steno-occlusive cerebrovascular disease that often leads to hemorrhagic and ischemic strokes; however, its etiology remains elusive. Surgical revascularization by either direct or indirect bypass techniques to restore cerebral hypoperfusion is the treatment of choice to date. This review aims to provide an overview of the current advances in the pathophysiology of MMD, including the genetic, angiogenic, and inflammatory factors related to disease progression. These factors may cause MMD-related vascular stenosis and aberrant angiogenesis in complex manners. With a better understanding of the pathophysiology of MMD, nonsurgical approaches that target the pathogenesis of MMD may be able to halt or slow the progression of this disease.

Circulating immune cell landscape and T‐cell abnormalities in patients with moyamoya disease

“…MMD is now increasingly acknowledged as a condition primarily influenced by chronic inflammation, as indicated by previous research studies 9,11,30–33 . Circulating immune cells are believed to contribute to MMD and its progression.…”

“…MMD is now increasingly acknowledged as a condition primarily influenced by chronic inflammation, as indicated by previous research studies. 9 , 11 , 30 , 31 , 32 , 33 Circulating immune cells are believed to contribute to MMD and its progression. These immune cells are believed to play a role in MMD by infiltrating blood vessel walls, triggering the release of proinflammatory molecules.…”

High-resolution magnetic resonance vessel wall imaging provides new insights into Moyamoya disease

“…At present, surgical revascularization is the main treatment for MMD patients ( Bao and Duan, 2023 ), however, surgical treatment may lead to perioperative complications such as ischemia or hydrocephalus ( Fujimura et al, 2011 ). With a better understanding of the pathophysiology of MMD, non-surgical approaches targeting the pathogenesis of MMD can be used to prevent or slow the progression of the disease ( Chen T. et al, 2023 ). In a 2018 national study in Japan, it was noted that the mechanism by which an antiplatelet agent (APT) is used to treat MMD is unknown, but it was summarized that aspirin, cilostazol, and clopidogrel are used in the course of MMD treatment, with aspirin being the most commonly used ( Oki et al, 2018 ).…”

Liquid chromatography coupled to mass spectrometry metabolomic analysis of cerebrospinal fluid revealed the metabolic characteristics of moyamoya disease

“…Although the epidemiology, clinical features, and treatment of MMD have been extensively studied, the etiology and pathogenesis remain largely unknown. It is believed that MMD is caused by an intricate mechanism involved in inflammatory, angiogenic, and vasculogenic pathway abnormalities in genetically susceptible individuals ( 3 ). Lack of a comprehensive understanding of the causes and mechanisms behind MMD has hindered the development of effective early prevention and intervention strategies for the condition.…”